High Dose Intravenous Methylprednisone in the Treatment of Severe Acute Respiratory Syndrome

Abstract

The case of a 72-year-old woman with probable severe acute respiratory syndrome is reported. While on treatment with ribavirin and antibiotics (for community-acquired pneumonia), the patient continued to have progressive clinical deterioration and chest radiographic evidence of respiratory deterioration. Pulse dose intravenous corticosteroids were used in an unsuccessful attempt to treat the inflammatory component of this respiratory illness.

Full text

ACUTE RESPIRATORY DISTRESS SYNDROME RELATED TO
ANTI-THYMOCYTE GLOBULIN (RABBIT) THERAPY FOR RE-
CURRENT ACUTE LUNG ALLOGRAFT REJECTION
Luisa F. Bazan, MD*; Jackie Edgar, RN; Karen Rampp, RN; Kevin M.
Chan, MD, FCCP. Henry Ford Hospital, Detroit, MI
INTRODUCTION: Anti-thymocyte globulin (rabbit) (thymoglobulin)
can be effective in reversing steroid-resistant rejection of solid transplanted
organs. This is a rare case report of ARDS related to thymoglobulin.
CASE PRESENTATION: A 66 year-old male with a history of
bilateral lung transplantation for emphysema developed steroid-resistant
acute rejection. Thymoglobulin therapy was instituted. Fever, chills and
malaise occurred after each infusion and thrombocytopenia and anemia
later developed. Oral itraconazole was started for a bronchoalveolar lavage
(BAL) culture positive to aspergillus 2 weeks before thymoglobulin. After
the fourth dose of thymoglobulin, he developed worsening dyspnea and
hypoxia. Chest radiograph (CXR) showed bilateral diffuse infiltrates that
did not respond to diuresis. He progressed to acute respiratory failure and
was intubated. There was no hemodynamic instability. PaO2/FiO2 ratio of
143, central venous pressure (CVP) of 7 mmHg and CXR were consistent
with a picture of ARDS. All cultures were negative including BAL.
Computed tomography of the chest showed bilateral ground glass opac-
ities with slight peripheral sparing consistent with edema and inflamma-
tory pneumonitis. He received broad spectrum antibiotics, antifungal
therapy and high dose corticosteroids. He was extubated four days after
the last thymoglobulin infusion despite a positive fluid balance of 8.5
liters.
DISCUSSION: Only two cases of ARDS related to thymoglobulin
have been described. The transfusion-related ARDS mechanism of
leukoagglutinating and lymphocytotoxic antibodies in the transfused
serum has been attributed to this complication. However, the theoret-
ical basis for acute lung injury with thymoglobulin is unclear. The low
CVP and recovery of the patient despite non-response to diuresis
makes congestive heart failure an unlikely cause of his respiratory
failure which seems better explained by an abnormal alveolar-capillary
membrane permeability.
CONCLUSION: Clinicians should be aware that the potential for
acute lung injury exist in patients receving thymoglobulin.
REFERENCES:
1. Walton G et al. Antithymocyte globulin-induced adult respiratory
distress syndrome. Arch Int Med 1998; 158: 1380.
2. Dean N et al. Adult respiratory distress syndrome related to
antilymphocyte globulin therapy. Chest 1987; 91: 619-20.
DISCLOSURE: L.F. Bazan, None.
INTERSTITIAL PNEUMONITIS DUE TO PEGYLATED INTER-
FERON ALFA-2B AND RIBAVIRIN
Ravi R. Nemivant, MD*; Rasheed Hammadeh, MD; Nikunj Shah, MD;
Jaime Villanueva, MD. Loyola University Medical Center, Maywood, IL
INTRODUCTION: Infection with hepatitis C is effectively treated
with interferon and ribavirin. We present the case of a patient who
developed interstitial pneumonitis during treatment with pegylated inter-
feron alpha-2b and ribavirin.
CASE PRESENTATION: A 72 year-old male with hepatitis C and
a viral load greater than 500,000 IU/mL began treatment with the
combination of ribavirin 400 mg twice daily and pegylated interferon
alpha-2b (PEG-Intron) 1.5 mcg/kg subcutaneously on a weekly basis.
After four months of treatment, he developed a diffuse, pruritic rash
concurrent with eosinophilia (1420/mm3, 38% total WBC). He was
diagnosed with exfoliative erythroderma and treated with topical
steroids. Ribavirin was discontinued. The erythroderma and eosino-
philia resolved. Four days later he developed dyspnea and hypoxia. A
CT scan of the chest revealed dense consolidations in both upper lobes
(see figure). Interferon was discontinued. Bronchoscopy revealed
moderate mucus production from all bronchial segments. Bronchoal-
veolar lavage failed to grow bacteria, acid-fast organisms, fungus, virus
or Pneumocystis carinii. Transbronchial biopsy revealed focal fibrosis,
eosinophilic proteinaceous exudates and reactive type II pneumocytes.
The patient required intubation and mechanical ventilation. High-dose
steroids were started. His infiltrates improved on chest roentgenogra-
phy. Five days after starting mechanical ventillation, he was success-
fully extubated. Two weeks later he was discharged from the hospital.
Four weeks after discharge, the patient was seen in the outpatient
clinic. His dyspnea had completely resolved.
DISCUSSION: Treatment of hepatitis C infection with non-pegylated
interferon alpha-2b and ribavirin is becoming common practice. The
addition of polyethylene glycol (PEG) to interferon alpha allows for a
longer half-life and sustained therapeutic drug levels thus enabling
once-weekly injections.
This is the first case report documenting the development of
interstitial pneumonitis after initial therapy with a combination of
pegylated interferon alpha-2b and ribavirin for hepatitis C. The
mechanism by which pneumonitis develops is unknown but is believed
to be secondary to an allergic-immunologic process (1). In our case, the
development of skin rash, peripheral eosinophilia and eosinophilic
exudates on lung biopsy support this hypothesis. In addition, this
theory is further supported by the quick resolution after drug cessation
and steroid therapy.
The only other incident of interstitial pneumonitis associated with
pegylated interferon occurred in a patient who had already developed
respiratory symptoms from previous treatment with a combination of
non-pegylated interferon alpha and ribavirin (3). The combination of
non-pegylated interferon alpha and ribavirin has been associated with
the development of interstitial pneumonitis (1-4). There are no cases
reported in the medical literature of interstitial pneumonitis from
ribavirin alone.
CONCLUSION: Interstitial pneumonitis can develop during initial
treatment of hepatitis C with pegylated interferon alpha-2b and ribavirin.
Interstitial pneumonitis from pegylated interferon alpha-2b and ribavirin
can resolve after steroid therapy and drug cessation. Interstitial pneumo-
nitis must be considered in the diagnostic differential in patients who
develop pulmonary complaints while being treated with pegylated inter-
feron alpha-2b and ribavirin.
REFERENCES:
1. Ishizaki, T., et al. Pneumonitis during interferon and/or herbal drug
therapy in patients with chronic active hepatitis. European Respira-
tory Journal. 1996; 9(12) : 2691-6.
2. Karim, A., et al. Interstitial pneumonitis in a patient treated with
alpha-interferon and ribavirin for hepatitis C infection. American
Journal of the Medical Sciences. 2001; 322(4): 233-5.
3. Kumar, KS., et al. Significant pulmonary toxicity associated with
interferon and ribavirin therapy for hepatitis C. American Journal of
Gastroenterology. 2002; 97(9): 2432-40.
4. Rothfuss, KS. et al. Interstitial pneumonitis during combination
therapy with interferon-alpha and ribavirin in a patient with chronic
Hepatitis C. Zeitschrift fur Gastroenterologie. 2002; 40(9): 807-10.
DISCLOSURE: R.R. Nemivant, None.
Monday, October 27, 2003
Drug-Induced Lung Disease
4:15 PM - 5:45 PM
242S CHEST 2003—Case Reports

LIFE-THREATENING DIFFUSE ALVEOLAR HEMORRAGE
RELATED TO CRACK COCAINE USE: SPONTANEOUS RESO-
LUTION WITHOUT STEROIDS
Juan E. Morales, MD*; Daniel Iltchev, MD; James N. Allen, MD. Ohio
State University Medical Center, Columbus, OH
INTRODUCTION: Smoking of crack cocaine has been associated
with a broad spectrum of pulmonary manifestations. We report a case of
diffuse alveolar hemorrhage, a rare and potentially life threatening
complication of cocaine smoking.
CASE PRESENTATION: A 49-year-old woman presented to our ER
with 1 week-history of cough, dyspnea and hemoptysis. On the day of
admission she had expectorated about 500cc of bright red blood. Her
medical history was significant for hypertension. On examination she was
tachypneic with a room air saturation of 85%. She had diffuse crackles.
The chest-X-ray showed bilateral alveolar infiltrates. She was admitted to
the ICU where an urgent bronchoscopy revealed diffuse alveolar hemor-
rhage. Angiography of the bronchial arteries showed no local lesions and
chest CT showed no masses. Toxicology screen was positive for cocaine
and later she was found to have crack cocaine in her possession. Blood and
respiratory cultures were negative. Initial treatment consisted mainly of
supportive therapy with oxygen supplementation and fluids. By the second
day the bleeding had resolved spontaneously and she was sent to the
medicine floor.
DISCUSSION: Cocaine use has been related to a number of pulmo-
nary complications from asymptomatic decreased diffusing capacity to
acute lung injury with respiratory failure. Although 6% to 27% of cocaine
users report having episodes of blood-streaked sputum, few cases of
massive hemoptysis or life threatening alveolar hemorrhage have been
reported. Possible mechanisms include direct toxic epithelial cell damage,
vasoconstriction of the pulmonary circulation, cocaine-induced clotting
abnormalities resulting in thromboembolism, and cocaine-related pulmo-
nary hypertension. Previously, a beneficial effect of steroids has been
suggested, however, no control trials have been published. Neither
steroids nor other anti-inflammatory therapies were used in this patient,
who improved with supportive care only.
CONCLUSSION: Diffuse alveolar hemorrhage is a serious complica-
tion of crack cocaine smoking. Its frequency is likely underreported. The
value of adding steroids to the standard supportive treatment is not
known.
DISCLOSURE: J.E. Morales, None.
A CASE OF INFLIXIMAB AS A PROMOTER OF METASTATIC
MELANOMA
Michael N. Solomon, MD*; Julie-Anne Thompson, MD; Armand J. Wolff,
MD; Eric D. Anderson, MD. Georgetown University Medical Center,
Washington, DC
INTRODUCTION: Infliximab is a chimeric mouse-human monoclo-
nal IgG antibody directed against soluble and cellular tumor necrosis
factor-alpha (TNF-alpha) and is approved for use in the United States for
treatment of refractory Crohn’s Disease (CD) and fistulizing CD. Inflix-
imab is an immunomodulatory agent with immunosuppressive properties.
We report on an individual with a distant history of malignant melanoma
that developed widespread melanoma metastases after receiving several
infusions of infliximab.
CASE REPORT: A 68-year-old hypertensive man presented with new
onset dyspnea and diffuse pulmonary nodules. Thirty-two years previ-
ously, a melanoma had been radically excised from his right ankle. He also
had a 15-year history of CD and a five-year history of IgA nephropathy.
For the CD, he had received azathioprine for one year and had chronically
received prednisone. During the past year, he had received five 500 mg
infusions of infliximab; the most recent infusion had been two months
prior. For the IgA nephropathy, he had received mycophenolate mofetil.
The patient was hospitalized because of shortness of breath, dry cough
and fatigue for three days. He had lost 15 pounds over the prior month.
He denied symptoms of chills, fevers, night sweats, hemoptysis, and chest
pain. A chest radiograph and CT scan of the thorax revealed diffuse
moderate-sized pulmonary nodules.
He had had a negative PPD one year ago. Medications included
prednisone and mycophenolate mofetil. He was afebrile and hemodynam-
ically stable with a saturation of 94% on 6 L/min of oxygen. He had no
adenopathy, the lung fields were clear, and there was no hepatospleno-
megaly or clubbing/cyanosis of his digits.
Transbronchial biopsies were performed, but no diagnosis was
made. Worsening hypoxemia necessitated mechanical ventilation.
Wedge resection was performed and the pathology was consistent with
metastatic melanoma. The patient was extubated and he expired
shortly thereafter.
DISCUSSION: To date, in all chronically immunosuppressed popula-
tions, an increased risk for the development of malignancies has not been
found. However, in epidemiologic studies of immunosuppressed renal
and cardiothoracic transplant populations, an increased incidence of
cutaneous neoplasms has been documented compared with age-matched
controls.
TNF-alpha is a complex cytokine with immunomodulatory properties.
In vitro studies have demonstrated that TNF-alpha augmented the
activated-macrophage destruction of murine melanoma and carcinoma
cells. In randomized, human studies, TNF-alpha has been added to
melphalan and low dose interferon-gamma therapy, and a selective
coagulation and necrosis of tumor microvasculature has resulted. Such
evidence supports the idea that TNF-alpha has anti-tumor capability.
However, other studies also have demonstrated that TNF-alpha has tumor
promoter activity. When TNF-alpha was given to rats with fibrosarcomas,
it promoted metastases.
It is unclear what relationship exists between antibodies to TNF-alpha,
such as infliximab, and the development of neoplasms. While evidence
suggests that immunosuppression and life-threatening infections are
associated with its use, infliximab therapy has not been associated with the
development of malignancies. Longer-term population-wide surveillance
will be required to identify such a link.
Because of the possible tumor promoting effects of infliximab, we
believe it important to report on our patient. We find it curious that he
presented with metastatic melanoma 32 years after apparently curative
resection of his tumor. While exposure to other immunosuppressives
such as prednisone, azathioprine and mycophenolate mofetil cannot be
discounted as contributing to the development of metastatic mela-
noma, the proximate cause seems likely to be treatment with inflix-
imab.
CONCLUSION: Infliximab has many properties, one of which is
immunosuppression. There may be a relationship between infliximab
infusions and the development or promotion of metastatic cancer. Patients
who are to receive infliximab and who have a history of cancer require
close observation for the development of metastatic disease.
DISCLOSURE: M.N. Solomon, None.
INTERSTITIAL LUNG DISEASE ASSOCIATED WITH PROXI-
MAL MUSCLE WEAKNESS AND SKIN RASH DEVELOPING
AFTER PRAVOSTATIN USE
Shoaib Alam, MD*; Robert L. Vender, MD; Kathleen M. Chaisson, MD;
Shazia Riaz, MBBS. Penn State University-Milton S. Hershey Medical
Center, Hershey, PA
INTRODUCTION: Polymyositis and dermatomyositis(PM/DM) are
uncommon causes of interstitial lung disease(ILD). However, the preva-
lence of ILD in patients with PM/DM is reported to be as high as 32%.
Pravostatin associated inflammatory myopathy is a well known entity but
its association with full blown classical PM/DM along with ILD is not a
well recognized phenomenon.
CASE PRESENTATION: A 66-year -old caucasian male was referred
to the pulmonary clinic for the evaluation of progressive dyspnea on
exertion over ten months and worsening interstitial infiltrates on chest
radiograph. He was recently started on Pravostatin for his hypercholes-
terolemia. He was completely asymptomatic before the drug was started.
After 2-3 weeks on Pravostatin, he started having generalized muscle
aches and proximal muscle weakness. Due to this, the drug was discon-
tinued. He took Pravostatin for a total of 25 days. Despite the discontin-
uation of drug the proximal muscle weakness continued to get worse.
Later on, he started developing worsening dyspnea on exertion. Patient
also noticed a maculo-papular erythematous skin rash involving lower half
of the legs and dorsum of both hands up to the wrists along with puffiness
of the knuckles. A similar rash was also present on the upper chest , both
anteriorly and posteriorly.
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Suspicion of dermatomyositis was raised secondary to his profound
proximal muscle weakness and typical skin rash. Neurology consult was
obtained and a thoracic paraspinal muscle biopsy was performed which
was consistent with inflammatory myopathy. No granulomatous inflam-
mation, perifascicular atrophy, rimmed vacuoles or vasculitis was seen.
Electron microscopy showed abnormally high number of ragged red
fibers. No intramitochondreal crystal inclusions, membrane-bound
glycogen or intracytoplasmic filamentous inclusions were seen. EMG
study showed fibrillation potentials and changes consistent with my-
opathy. Nerve conduction study, cervical spine and lumber MRI were
non-revealing. CPK:133, TSH: 7.54, Free T4: 0.61, ANA: 40, ANCA:
non-detectable, Anti-acetylcholine antibody: negative, Jo-1 antibody:
0, Lyme antibody titre: negative, bacterial, AFB and fungal cultures of
bronchoalveolar lavage: negative, transbroncheal biopsy: no pathologic
diagnosis.
A diagnosis of dermatomyositis and PM/DM related ILD was made.
The patient was initially treated with Prednisone 60 mg per day and later
Azathioprine 50 mg, twice daily with Prednisone 10 mg per day. Marked
improvement in muscle strength was observed and rate of decline of lung
functions was also decreased.
DISCUSSION: Based on profound muscle weakness, classical skin
rash and extensive interstitial infiltrates on the chest CT scan, along with
the results of muscle biopsy and EMG, the diagnostic certainty was quite
high. The precipitous evolution of full blown classical DM in association
with new onset severe dyspnea and exercise induce hypoxemia with
extensive interstitial infiltrates on CT scan, in a completely asymptomatic
patient, within a few weeks of Pravostatin use suggests the possibility of an
adverse reaction to Pravostatin as the basis for the development of this
unusual presentation.
CONCLUSION: The development of dermatomyositis and PM/DM
associated interstitial lung disease can be seen in association with Pra-
vostatin use. Immunosuppression resulted in significant improvement in
muscle strength and decreased rate of decline of lung functions.
REFERENCES:
1. Curr Rheumatol Rep 2002 Oct;4(5):409-14. Ascherman DP. Pul-
monary complications of inflammatory myopathy.
2. Chest 1999 Mar;115(3):886-9. Liebhaber MI, Wright RS, Gelberg
HJ, Dyer Z, Kupperman JL Polymyalgia, hypersensitivity pneumo-
nitis and other reactions in patients receiving HMG-CoA reductase
inhibitors: a report of ten cases
3. Semin Arthritis Rheum 2003 Apr;32(5):273-84Schnabel A, Reuter
M, Biederer J, Richter C, Gross WL. Interstitial lung disease in
polymyositis and dermatomyositis: clinical course and response to
treatment.
DISCLOSURE: S. Alam, None.
GEMCITABINE PULMONARY TOXICITY
Ognjen Gajic, MD*. Mayo Clinic, Rochester, MN
INTRODUCTION: Gemcitabine is a newer pyrimidine analog that
has been increasingly used in the chemotherapy of solid tumors.
During the initial studies it appeared to have a favorable side-effect
profile with a dose-limited marrow suppression being the most
important toxicity[1]. While mild dyspnea was common(⬃10%), seri-
ous pulmonary toxicity was thought to be unusual. With a more
frequent use of Gemcitabine in clinical practice there have been
reports of severe acute lung injury (ALI) temporally related to a
Gemcitabine use[2,3]. Here we describe a case of a 77-year-old woman
who presented with severe ALI during treatment with Gemcitabine for
metastatic breast carcinoma.
CASE PRESENTATION: A 77-year-old woman presented with
progressive dyspnea after the 3
rd
cycle of Gemcitabine treatment in the
setting of metastatic breast carcinoma. Three days earlier she was
admitted to a local hospital with fever and dyspnea. She was given
levofloxacin for E.coli bacteriuria. Over the next two days her condition
deteriorated and she was transferred to a tertiary center. Her past
history was significant for mastectomy, local radiation and tamoxifen
1yr earlier. On admission to our hospital, she was in moderate
respiratory distress using accessory respiratory muscles. Her vital signs
were as follows: T 102F, BP 130/65, HR 110, RR 32. She was
maintaining adequate oxygen saturation on 100% FiO
2
via mask. A few
inspiratory crackles were present at both bases. Heart sounds were
unremarkable and there was no jugular venous distension or peripheral
edema. After a brief trial of noninvasive positive pressure ventilation
endotracheal intubation was performed and mechanical ventilation
started. Arterial blood gas measurement revealed significant hypox-
emia with PaO
2
/FiO
2
ratio of 109. Portable CXR (Figure 1) demon-
strated bilateral infiltrates without significant increase in cardiac size
or vascular pedicle width consistent with non-cardiogenic pulmonary
edema. Computerized tomography of the chest demonstrated upper
lobe predominance of alveolar and course interstitial infiltrates (Figure
2). Urgent bronchoscopy was performed to rule out infectious etiology.
Bronchoalveolar lavage fluid, blood and urine culture did not reveal
specific causative organism to suggest infectious etiology. The findings
on transbronchial biopsy were consistent with diffuse alveolar damage
with prominent hyaline membranes and type 2 pneumocyte prolifer-
ation (Figure 3). The patient was treated with protective ventilatory
strategy and immediately started on systemic corticosteroid therapy for
probable drug-induced acute lung injury. Over the next several days
her condition rapidly improved. She was weaned from mechanical
ventilation and discharged home after a total of two weeks in the
hospital (Figure 4).
DISCUSSION: We presented a case of ALI in a setting of gemcitabine
chemotherapy. Clinical course, radiologic and pathologic characteristics,
rapid response to corticosteroids and the absence of other potential causes
are suggestive of drug-induced ALI. Clinical and pathologic characteris-
tics are similar to a lung injury caused by an older pyrimidine analog,
cytarabine, which is known to cause ALI in up to 30% of patients treated
with high dose[1]. The exact frequency of gemcitabine associated ALI is
not known. Associated clinical factors are previous radiation or chemo-
therapy and older age[1]. Lung biopsy features diffuse alveolar damage
with type2 cell proliferation[4]. Radiologic findings consist of asymmetric
bilateral ground glass infiltrates[5]. Clinical course is variable with rapid
response to systemic corticosteroid treatment in many patients(Figure
4)[5,6].
CONCLUSION: Gemcitabine toxicity should be strongly considered
in differential diagnosis of acute lung injury in the immunocompromised
patient. Rapid diagnostic work up(including bronchoscopy) is necessary to
exclude infectious etiology enabling early corticosteroid treatment which
appears to be highly effective.
REFERENCES:
1. Gupta N et al.Am J Clin Oncol 2002;25:96
2. Linskens RK et al.Neth J Med 2000;56:232
3. Stern JB et al.Rev Mal Respir 2002;19:253
4. Marruchella A et al.Eur Resp J 1998;11:504
5. Boiselle PM et al.J Comput Assist Tomogr 2000;24:977
6. Vander Els N et al.Chest 1998;114:1779
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DISCLOSURE: O. Gajic, None.
NON-ALLERGIC GRANULOMATOSIS WITH EOSINOPHILIA: A
NEW ENTITY IN EOSINOPHILIC LUNG DISEASE
Usha G. Nayak, MBBS*; Hyder Jaffrey, Chad Stone, Pathology; Alan D.
Betensley, Henry Ford Hospital; Fortune Alabi, Henry Ford Hospital.
Henry Ford Hospital, Detroit, MI
INTRODUCTION: Eosinophilic lung diseases encompass a wide
variety of pulmonary disorders. We describe a case of eosinophilic lung
disease which does not fall into any of the categories that have been
previously reported in the literature.
CASE PRESENTATION: A 43 year old female presented with acute
dyspnea, wheezing and chest tightness. She denied cigarette smoking and
drug use and had no history of previous illnesses including HIV or tubercu-
losis. She had never traveled outside of Michigan, she had no pets, and she
denied any occupational exposures or toxic inhalation. Exam was remarkable
only for bilateral wheezing. Peripheral blood eosinophil count was 1280/mm
3
.
Serum creatinine was normal and there was no hematuria or proteinuria.
Serologies for HIV, antinuclear antibody, anti-neutrophil cytoplasmic anti-
body, and rheumatoid factor were negative. Stool cultures were negative for
ova and parasites. Chest X-ray and CT scan both revealed bilateral diffuse
micronodules as shown below. Bronchoalveolar lavage excluded infection.
Kveim test was negative. Open lung biopsy was performed and histopathology
revealed necrotizing granulomas with tissue eosinophilia as shown below.
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Drug-Induced Lung Disease, continued Granulomatous Diseases
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DISCUSSION: The combination of peripheral eosinophilia, tissue
eosinophilia, and necrotizing granuloma would be consistent with allergic
granulomatosis and angiitis. However, the absence of atopy and lack of
vasculitis makes this diagnosis unlikely. The literature describes isolated
cases of eosinophilic lung disease secondary to medications, infections,
and sarcoidosis, but our patient had an extensive workup including
bronchoscopy and Kveim test that excluded these etiologies. Eosinophilia
in Wegener’s granulomatosis has been described, but the negative ANCA
coupled with the absence of vasculitis makes this diagnosis unlikely.
CONCLUSION: By excluding previously reported etiologies of eosin-
ophilic lung disease, we conclude that this case may represent a new
entity, which we describe as non-allergic granulomatosis with eosinophilia.
REFERENCES:
1. James et al. Eosinophilic lung disease, Am J Respir crit Care Med,
1994:150;1423-38.
2. Potter MB et al. Eosinophilia in WG. Chest. 1999:116; 1480-1483.
3. Katzenstein A, et al. Am J Clin Pathol 2000 114;767-772 .
DISCLOSURE: U.G. Nayak, None.
PULMONARY SARCOIDOSIS AS A COMPLICATION OF INTER-
FERON TREATMENT OF CHRONIC HEPATITIS C
Sean Devine, MD*; Jeffrey Liu; Rohit Ahuja, MD; Gonzalo Gianella,
MD; Daniel Salerno, MD; Ganesan Murali, MD; Kenneth Rothstein,
MD; Michael Lippmann, MD. Albert Einstein Medical Center & Thomas
Jefferson University Hospital, Philadelphia, PA
INTRODUCTION: Two patients with chronic hepatitis C (CHepC)
receiving interferon (IFN) and ribavirin (RIB) present with progressive
dyspnea and pulmonary infiltrates.
CASE PRESENTATIONS: Case(1) A 43-yr old woman with CHepC
had a non-productive cough and dyspnea for 6 weeks. There was a remote
history of suspected sarcoidosis. She had completed 10 months of
intra-muscular injection of Pegylated IFN␣-2b every week and RIB 600
mg by mouth twice a day. She was afebrile and anicteric with stable vital
signs. Inspiratory crackles were heard at lung bases. Chest xray revealed
bilateral infiltrates and chest CT scan revealed diffuse interstitial infil-
trates without hilar adenopathy.(Fig:1) Mild restrictive pattern with
severe reduction of diffusing capacity was noted on pulmonary function
tests. Angiotensin converting enzyme (ACE) level:1400 mmol/L. Cob-
blestoning of bronchial mucosa was noted on bronchoscopy (Fig:2) and
transbronchial lung biopsy(TBB) revealed non-caseating granuloma.(Fig:
3). Case (2): A 51 yr-old male smoker with CHepC had dry cough,
malaise for 2 months. Prior to the onset of symptoms, he had received
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Granulomatous Diseases, continued
246S CHEST 2003—Case Reports

IFN and RIB for 4 months. His vital signs were stable. Inspiratory
bibasilar crackles were heard. He had a normal ACE level and negative
autoimmune workup. A chest xray revealed bilateral interstitial pattern.
CT Chest confirmed diffuse reticulonodular disease.TBB revealed non-
caseating granulomas. IFN and RIB were discontinued in both patients.
Three months later, their cough has improved significantly.
DISCUSSION: T helper (Th-1) cells play a major role in the patho-
genesis of sarcoidosis. IFN ␣ is known to activate macrophages and
T-Cells. This leads to release of IFN-2 and IFN-␥ that induce CD4⫹ cells
to differentiate into Th-1 effector cells. Whereas this immune cascade
occurs naturally over the course of an infection, exogenous IFN may
potentially contribute to the Th-1 response in sarcoidosis.
CONCLUSION: IFN for hepatitis C is being reported to be associated
with either a flare up of underlying indolent sarcoidosis and de novo
pulmonary sarcoidosis. This also support an immunomodulatory hypoth-
esis behind the development of sarcoidosis.
DISCLOSURE: S. Devine, None.
SARCOID LIKE FOREIGN BODY GRANULOMATOSIS
Ghazala T. Farooqui, MBBS*; Judy King, MD; Ronald Allison, MD.
University of South Alabama, Mobile, AL
INTRODUCTION: Inorganic dust exposure produces variety of re-
sponses the complexity of which is not yet fully understood. Sarcoid like
illness is well documented with beryllium and aluminum. Recently, an
association is also observed between viterous fiber exposure and sarcoid
like granulomatosis.
(1,3)
Our case is an example of such type of illness
secondary to man made mineral fiber (MMMF).
CASE PRESENTATION: 39 year old black male was working as a
factory insulation installer for fifteen years.He had six months history
of progressive exertional dyspnea, fatigue and occasional mild cough
productive of white colored sputum. He denied fever, chills, night
sweats, chest pain, hemoptysis, peripheral edema or orthopnea. Chest
radiographs revealed diffuse alveolar opacities, bilateral apical cavitary
lesions, bilateral granulomata, nodules and multiple calcified medias-
tinal lymph nodes. Transbronchial biopsy specimen showed non
caseating foriegn body type granulomas. The foriegn bodies were
polarized and scanning electron microscopy (SEM) identified them as
silica, titanium, aluminum, chromium and nickel . In adddition peaks
of magnesium, sulfur, potassium, calcium, phosphorus, copper and
iron were also present.
DISCUSSION: Production of artificial fiber has become a major
industry since World War II .Fiber glass is used in acoustic and thermal
insulation and has been implicated as a potential cause of fibrotic and
granulomatous lung diseases.
Several mechanism have been proposed for lung injury which include
nonspecific accumulation of macrophages in alveoli with occasional
granulomatous reaction
(4)
, production of hydroxyl radical resulting in
DNA damage
(5)
, T-cell alveolitis and particle induced NF-kB stimulation.
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Drent et al. have demontrated presence of activated NF-kB in sarcoidosis
as well.
Three types of parenchymal responses have been identified with
occupational dust exposure.These include interstitial fibrosis, nodular
fibrosis and macule formation with emphysema.
Stetter et al. studied lungs from 33 subjects who had no history of
occupational exposure. All analysed lungs contained aluminum, silica, iron
and titanium and some of them also had potassium, zinc, sulfur, magne-
sium and chromium.
(6)
Induction of disease by a foriegn particle depends on cumulative
exposure, clearance an dissolution, latency, genetic susceptibility, biologic
plausibility and interaction with host factors. Single occupational agent
may cause many diseases because of differences in susceptibility and
metabolism of individual or several agents may produce only one type of
illness.
An effective approach is to obtain a comprehensive lifetime occupa-
tional history. Material Safety Data Sheets will certainly be an asset to
determine the chemical composition of commercial materials.
Treatment is aimed to suppress inflammation in an attempt to halt
disease process. Systemic corticosteroids may provide benefit in releiving
symptoms
(2)
but relapses are reported with steroid tapering. Long term
efficacy of steroid therapy is unknown. Serial monitoring of pulmonary
function testing can give an objective assessment of patient’s disease
activity.
Future harmful exposure should be avoided and to prevent further
occurrences employer should be notified with patient’s permission.
CONCLUSION: In conclusion, widespread deposition of foriegn
particles found in MMMF have contributed to the disease process in this
patient. Data pertaining to epidemiology, presentation, prognosis and
treatment is inadequate. Further studies are needed to focus these issues
in future.
REFERENCES:
1) Drent et al. association of MMMF exposure and sarcoidlike granu-
loma. Resp. Medicine 2000;94:815-820
2) Beckett WS. occupational respiratory diseases. NEJM 2000;342:406-
413
3) Takahashi et al. Pulmonary firosis in a carpenter with long-lasting
exposure to fiberglass. AJ Indus Med 1996;30(5):596-600
4) Jackel M et al. Lung damaging effects of glasswool-fibers. An
experimental study. Eur Resp J 1999;14:212s
5) De Vust et al. Sarcoidlike granulomatosisinduced by aluminum dust.
Am Rev Resp Dis 1987;135:493-497
Monday, October 27, 2003
Granulomatous Diseases, continued
248S CHEST 2003—Case Reports

6) Stetter et al. Particulate concentration in urban lungs.1989 Hemi-
sphere Press, New York. 133-146
DISCLOSURE: G.T. Farooqui, None.
COEXISTENT SARCOIDOSIS AND HIV INFECTION: AN IM-
MUNOLOGICAL PARADOX
Francisco A. Almeida, MD*; Jeffrey Sager, MD; Glenn Eiger, MD,
FACP, FCCP. Albert Einstein Medical Center, Philadelphia, PA
INTRODUCTION: The CD4 lymphocyte plays a pivotal role in both
sarcoid and HIV infection. Sarcoidosis is CD4 mediated and symptomatic
HIV is CD4 depleted. Concurrent HIV infection and sarcoidosis has
rarely been reported. The management of a patient with both conditions
represents a diagnostic and therapeutic challenge. We describe a patient
in whom these two conditions coexist and review the literature.
CASE PRESENTATION: A 41-year-old African American female was
diagnosed with biopsy proven cutaneous sarcoidosis in 1992. She did not
have pulmonary symptoms but the chest radiograph revealed mild
bilateral interstitial infiltrates. During 1996 she was started on highly
active anti-retroviral therapy (HAART) for newly diagnosed HIV disease
with poor medical compliance (CD4/CD8 228/804, ratio 0.28). In 2000
she presented with a 4-week history of exertional dyspnea, dry cough and
orthopnea. Physical examination revealed small submandibular lymph
nodes and clear lungs on auscultation. Chest radiograph revealed wors-
ening bilateral infiltrates. Serum CD4/CD8 ratio was 0.83 (435/524). The
angotensin-1-converting enzyme was slightly elevated at 56U/L. The
broncho-alveolar lavage (BAL) fluid had a CD4/CD8 ratio of 1.4.
Transbronchial biopsies showed non-caseating granulomas. All cultures
were negative. The patient was treated with corticosteroids with marked
clinical and radiographic improvement.
DISCUSSION: To our knowledge, only 3 cases of sarcoidosis and HIV
infection with CD4/CD8 ratio more than 1.0 in the BAL have been reported.
This patient was found to have a relatively elevated CD4/CD8 ratio in the
BAL compared to her serum, suggesting that active sarcoid rather than HIV
disease was the etiology for her pulmonary disease. This raises the possibility
of “immune reconstitution syndrome” which may have worsened her sarcoid-
osis as a result of treatment of HIV disease with HAART. The well-defined
non-caseating granulomas on lung biopsy suggest sarcoidosis as CD4 lym-
phocyte depletion may attenuate granuloma formation.
CONCLUSION: Sarcoidosis should be considered in the differential
diagnosis of HIV patients with pulmonary symptoms while on HAART. In
patients with coexisting sarcoidosis and HIV, the CD4/CD8 ratio in the
BAL fluid may be diagnostically helpful.
REFERENCES:
1. Coots LE, Lazarus AA. Sarcoidosis diagnosed in a patient with
known HIV infection. Chest 1989;96:201-2.
2. Gowda KS, Mayers I, Shafran SD. Concomitant sarcoidosis and HIV
infection. Can Med Assoc J 1990;142(2):136-7.
3. Lowery WS, Whitlock WL, Dietrich RA, Fine JM. Sarcoidosis
complicated by HIV infection: three cases reports and review of the
literature. Am Rev Respir Dis 1990;142:887-9.
4. Newman TG, Minkowitz S, Hanna A, Sikand R, Fuleihan Farid.
Coexisting sarcoidosis and HIV infection: a comparison of bronchoal-
veolar and peripheral blood lymphocytes. Chest 1992;102:1899-1901.
5. Naccache JM, Antoine M, Wislez M, Fleury-Feith J, Oksenhendler
E et al. Sarcoid-like pulmonary disorder in human immunodefi-
ciency virus-infected patients receiving antiretroviral therapy. Am J
Respir Crit Care Med 1999;159:2009-13.
6. Gomez V, Smith P, Burack J, Daley R, Rosa U. Sarcoidosis after
antiretroviral therapy in a patient with acquired immunodeficiency
syndrome. Clin Inf Dis 2000;31:1278-80.
7. Lenner R, Bregman Z, Teirstein A, DePalo L. Recurrent pulmonary
sarcoidosis in HIV-infected patients receiving highly active antiret-
roviral therapy. Chest 2001;119:978-81.
8. Viani RM. Sarcoidosis and interstitial nephritis in a child with
acquired immunodeficiency syndrome: implications of immune
reconstitution syndrome with an indinavir-based regimen. Pediatri
Infect Dis J 2002;21:435-8.
DISCLOSURE: F.A. Almeida, None.
MAGNETIC RESONANCE IMAGING RESOLUTION OF VERTE-
BRAL SARCOIDOSIS WITH HYDROXYCHLOROQUINE AND
PREDNISONE
Shaheen U. Islam, MD, MPH*; Anthony C. Campagna, MD, FCCP;
Richard Baker, MD; Paul Dellaripa, MD; Om P. Sharma, MD, FCCP.
Lahey Clinic, Burlington, MA
INTRODUCTION: Sarcoidosis of bone is infrequent and vertebral
involvement is rare. We present a unique case of vertebral sarcoidosis that
responded to hydroxychloroquine and low dose steroids. This is most
likely the first reported case of vertebral sarcoidosis treated successfully
with hydroxychloroquine and prednisone.
CASE PRESENTATION: A 58-year-old Caucasian female developed
severe back pain. Physical examination was unremarkable. A bone scan
showed multiple focal uptakes in the lower thoracic and upper lumbar (L)
vertebrae. Magnetic resonance imaging (MRI) of her spine revealed
multiple areas of hypo-intensity on T1 weighted images within the lumbar
vertebral bodies. A chest computed tomogram (CT) revealed two small
(⬍7mm) lung nodules. Her Gallium-67 imaging scan did not show any
focal uptake. Her serum angiotensin converting enzyme (39u/l) and serum
calcium (10.7mg/dl) were normal. A CT guided biopsy of the L1 pedicle
revealed non-caseating granulomas with no tumor cells. Acid fast and
fungal stains and cultures were negative. Hydroxychloroquine 200mg
twice a day and Prednisone 20mg daily were started. Serial MRI showed
significant improvement of the lumbar and thoracic vertebral sarcoid
lesions over the following two years. Prednsione was tapered after four
months and was discontinued at 16 months. Hydroxychloroquine was
continued for two years.
DISCUSSION: Osseous sarcoid is reported in 3% to 13% of cases
with multi-system sarcoidosis (1). Small bones of the hand and the feet
are most commonly affected and vertebral involvement is very rare (1,
2). In such cases diagnosis is fairly straightforward. The diagnosis may
remain obscure when bone involvement occurs in the absence of other
manifestations of sarcoidosis. On presentation, our patient did not have
active pulmonary sarcoidosis. She was reluctant to start high dose
steroids, so a combination of hydroxychloroquine with prednisone was
given. Hydroxychloroquine may inhibit processing of the putative
antigen by macrophages and the production of TNF-alpha and IL-6,
thereby modifying the initial steps in the pathogenesis of the disease
process (6,7,8). It reduces hyperglycemia and is helpful in patients with
steroid induced hyperglycemia (6). Hydroxychloroquine alone or in
combination with prednisone is effective in neurosarcoidosis, hyper-
calcemia and skin lesions of sarcoid (7,8). To our knowledge, this is the
first reported case in English literature of vertebral sarcoidosis suc-
cessfully treated with combination of hydroxychloroquine and low dose
steroids.
CONCLUSION: We present a case of MRI resolution of vertebral
sarcoidosis with hydroxychloroquine and low dose prednisone.
Less aggressive regimen may be effective in treatment of vertebral
sarcoid.
REFERENCES:
1. Wilcox A, Bhardawaj P, Sharma OP. Bone sarcoidosis. Curr Opin
Rheum 2000;12:321-330.
2. Chatham WW. Sarcoidosis. In Kelly’s textbook of Rheumatology, 6
th
Ed. 2001, WB Saunders Company, Philadelphia.
3. Fox RI. Mechanism of action of hydroxychloroquine as an anti-
rheumatic drug. Semin Arthritis Rheum 1993;23(suppl 1):82-91.
4. Zhu X, Ertel A et al. Chloroquine inhibits tumor necrosis factor-
alpha mRNA transcription. Immunology 1993;80:122-26.
5. Baltzan M, Mehta S, et al. Randomized trial of prolonged chloro-
quine therapy in advanced pulmonary sarcoidosis. Am J Respir Crit
Care Med 1999;160:192-197.
6. Quatraro A, Consoli G, et al. Hydroxychloroquine in decompen-
sated, treatment-refractory non-insulin dependent diabetes melli-
tus. Ann Intern Med 1990;112:678-81.
7. Sharma OP. Neurosarcoidosis A personal perspective based on the
study of 37 patients. Chest 1997;112:220-28.8. Adams JS, Diz MM,
Sharma OP. Effective reduction in the serum 1,25-dihydroxyvita-
min-D and calcium concentration in sarcoidosis associated with
hypercalcemia with short course chloroquine therapy. Ann Intern
Med 1989;111:437-38.
DISCLOSURE: S.U. Islam, None.
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UNUSUAL APPEARANCE OF NEUROSARCOIDOSIS ON MAG-
NETIC RESONANCE AND POSITRON EMISSION TOMO-
GRAPHIC IMAGES
Muhammad Raees, MD*; David Lilien, MD; Gerry S. San Pedro, MD.
Louisiana State University Health Sciences Campus at Shreveport,
Shreveport, LA
INTRODUCTION: The diagnosis of neurosarcoidosis is very chal-
lenging especially in the absence of other symptoms. Computed
tomography (CT) and magnetic resonance imaging (MRI) of the brain
are essential in the assessment of central nervous system (CNS)
involvement, with MRI now the modality of choice due to the
superiority of the images. Ring-enhancing lesions have not been
reported in neurosarcoidosis, so our biopsy-proven case represents the
first such presentation. Lymph nodes and other organs involved in
sarcoidosis typically exhibit intense uptake of Fluorine-18-labelled
2-deoxy-2-fluoro-D-glucose (FDG) on positron emission tomographic
(PET) imaging. Thus, whole body FDG PET has been used to assess
the extent of pulmonary and extra-pulmonary sarcoidosis. To date, no
report on FDG PET in neurosarcoidosis has been published. Our
report, then, is also the first report of FDG-PET imaging in biopsy-
proven intracranial sarcoidosis.
CASE PRESENTATION: A 22-year-old man presented with 9
months of progressive memory disturbances, unsteady gait, vomiting,
and urinary incontinence. He had no other symptoms. He was
somnolent but arousable to verbal stimuli, oriented to person and
situation but not to time or location, and had short and long-term
memory loss. Cerebellar signs included a stumbling shuffling gait,
inability to walk a straight line, inability to perform heel-shin testing,
and abnormal rapid alternating movements. The remainder of his
physical examination was normal. Biochemical, haematological, micro-
biological and immunological testing remained unrevealing. Cerebro-
spinal fluid (CSF) was clear, with normal glucose and total protein,
total leukocytes 11 cells/mm
3
(90% lymphocytes), and negative smears
for cryptococcus and acid fast bacilli. CSF cultures were negative.
Chest radiograph showed enlarged hilar shadows; chest CT confirmed
mediastinal lymphadenopathy without interstitial disease. MRI of the
brain showed multifocal disease with ependymal enhancement, skull base
meningeal inflammation, a ring-enhancing lesion in the left caudate
nucleus (Figure 1) and obstructing hydrocephalus.
Brain and whole body PET scans were obtained, searching for an
underlying neoplasm. PET images of the brain showed intense FDG
uptake in the brain lesions, correlating with the MRI findings (Figure 2).
Body images showed extensive FDG uptake in multiple node chains; the
intrathoracic nodal involvement had a “Christmas tree” configuration,
typical for sarcoidosis.
A stereotactic biopsy of the ring enhancing lesion yielded non-caseating
granulomas. No malignant cells, acid-fast bacilli, bacteria, Toxoplasma, or
fungal organisms were found. All cultures of the biopsy remained
negative.
The patient started on prednisone, 60 mg daily; a ventriculoperitoneal
shunt was implanted for residual hydrocephalus. He rapidly regained full
alertness and orientation, full urinary continence and could again walk
properly with minimal gait disturbance. On a follow-up visit 6 months
later, he exhibited continuing improvement.
DISCUSSION: Neurosarcoidosis remains a diagnostic challenge.
Radiologic imaging studies, particularly the MRI, are essential in the
evaluation of suspect patients; however, the MRI findings are not
specific for sarcoidosis. Reported manifestations of neurosarcoidosis
on MRI include nonenhancing periventricular white matter lesions;
meningeal enhancement; hydrocephalus; cranial nerve involvement;
enhancing brain parenchymal lesions; dural mass lesions; and spinal
cord involvement. Thus far, ring-enhancing lesions have not been
reported. Such a finding usually raises a differential diagnosis list of
infectious processes, primary neoplasms, metastases, demyelinating
diseases, and contusions.
Our patient had involvement of lymph nodes on CT and PET scanning
quite typical of sarcoidosis, assisting in the diagnosis but not excluding a
separate etiology for the CNS involvement. The diffuse involvement of
the brain demonstrated on MRI was also typical for sarcoidosis, but the
ring-enhancing lesion was quite unusual. Stereotactic biopsy with negative
stains and cultures of that lesion provided strong evidence that this lesion
represented the granuloma of sarcoidosis.
CONCLUSION: We claim, then, the first report of the ring-enhancing
lesion and FDG PET in neurosarcoidosis.
DISCLOSURE: M. Raees, None.
Monday, October 27, 2003
Granulomatous Diseases, continued
250S CHEST 2003—Case Reports

PULMONARY EMBOLI FROM MASSIVE THROMBOSIS OF
THE PERIPROSTATIC VENOUS PLEXUS MIMICKING ACUTE
RESPIRATORY DISTRESS SYNDROME
Prakash V. Reddy, MD*; David Solomon, MD FCCP. University Of
South Florida, Tampa, FL
INTRODUCTION: A 25 year old male presented with a flu like
syndrome rapidly progressing to acute respiratory distress syndrome and
ultimately resulting in death. On autopsy examination, death was due to
pulmonary emboli ( PE) originating from thrombosis of the periprostatic
venous plexus. This is the first reported case of PE in a young patient as
a result of thrombosis from the prostatic venous plexus. This case
substantiates and emphasizes the importance of identifying PE in a timely
fashion.
CASE PRESENTATION: The patient was a 25 year old male who
presented to the emergency room with complaints of difficulty in
breathing, fever, chills and a cough. His initial chest x-ray revealed a right
lower lobe infiltrate. Following a diagnosis of pneumonia, the patient was
discharged home on erythromycin.
Two days later, he was admitted to the hospital with symptoms of
persistent shortness of breath. A repeat chest X-ray showed bilateral
infiltrates. Treatment with antibiotics was continued for a possible
diagnosis of atypical pneumonia.
Initial arterial blood gases showed a pH 7.51, pCO2 31 mm Hg, pO2 51
mm Hg, HCO3 24.6 and Oxygen saturation of 85 %.
Cultures for bacteria, acid fast organisms, fungi and viruses were
collected. His HIV antibody titer was negative, WBC was 8.7 K/ UL, LDH
428 IU/ L. Repeated attempts failed to demonstrate an infectious etiology.
The patient was treated presumptively for pneumocystis carinii pneumo-
nia with Trimethoprim/ Sulfamethoxazole and continued on erythromy-
cin. He developed progressive hypoxia with respiratory failure resulting in
endotracheal intubation and mechanical ventilation. Despite aggressive
respiratory treatment he continued to deteriorate and expired ten days
later.
An autopsy was performed which demonstrated bilateral massive
pulmonary emboli with extensive infarctions. The source of the clots was
found to be massive thrombosis of the periprostatic venous plexus. The
prostate gland itself was unremarkable.
DISCUSSION: PE commonly originates from thrombi located in the
deep veins of the lower extremities. This represents the first report of
multiple emboli secondary to massive thrombosis of the periprostatic
plexus in a previously healthy 25 year old male. Common testing for deep
venous thrombosis would not have detected the originating thrombi. This
may explain why there is no English language medical literature describ-
ing periprostatic vein thrombi with a normal prostate gland and no history
of examination or trauma to the prostate. Based on the age and previous
good health of this young man pulmonary thromboembolic disease was
not suspected.
CONCLUSION: This report emphasizes that PE should be considered
in a young patient with progressive hypoxia and respiratory failure without
a known infectious etiology.
DISCLOSURE: P.V. Reddy, None.
RESPIRATORY FAILURE DUE TO METASTATIC PULMONARY
CALCIFICATIONS IN ACUTE RENAL FAILURE DIAGNOSED
WITH COMPUTERIZED TOMOGRAPHY AND BRONCHOAL-
VEOLAR LAVAGE (BAL), WHICH RESOLVED WITH RESOLU-
TION OF RENAL FAILURE AND CONSERVATIVE MANAGE-
MENT
Hakop H. Hrachian, MD*, William D. Marino, MD, FCCP, Julie A.
Daly-Dorrian, RN, Siobhain T. McHale, RN, Anna W. Komorowski, MD,
Stanley Oiseth, MD. Our Lady of Mercy Medical Center, Bronx, NY
INTRODUCTION: We present a case demonstrating (1) the possi-
bility of diagnosing metastatic calcification with respiratory failure occur-
ring in acute renal failure using CT densitometry and bronchoalveolar
lavage, and (2) its relatively rapid resolution with hydration after the
return of renal function.
CASE PRESENTATION: A 30 year old male was admitted with a
cocaine/narcotic mixed overdose and diffuse burns. His laboratory studies
revealed rhabdomyolysis with CPK levels in excess of 400, 000 IU, renal
failure with a creatinine level of 6.0 mg/dl and a phosphate level of 7.3
mg/dl. He was removed from dialysis on hospital day 51. Subsequent to
this he developed acute hypercalcemia with the attendant development of
diffuse interstitial pulmonary infiltrates and acute hypoxemic respiratory
failure.
A gallium scan of the lungs showed diffuse uptake throughout both lung
fields. Fiberoptic broncoscopy yielded BAL fluid containing cal-
cium particles. The CT of the chest showed that the pixel densities of the
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Acute Lung Injury/ARDS
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 251S
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interstitial infiltrates were consistent with calcification, and this was
confirmed by nuclide bone scanning which demonstrated calcification in
both lung fields. With further improvement of renal function and
hydration, the patient’s infiltrates and oxygenation progressively im-
proved, until on hospital day 135 he was removed from mechanical
ventilation.
DISCUSSION: Metastatic calcification of the lungs is a cause of
interstitial lung disease in acute renal failure which can be diagnosed
without lung biopsy utilizing minimally invasive and non-invasive meth-
ods. It can resolve spontaneously with improvement in renal function and
conservative management.
CONCLUSION: In the setting of acute renal failure, especially with
rhabdomyolysis, respiratory failure and acute interstitial lung disease can
be evaluated with CT densitometry, nuclide scanning and BAL. If
metastatic calcification of the lungs is identified, prolonged conservative
therapy should be provided.
DISCLOSURE: H.H. Hrachian, None.
DIFFUSE ALVEOLAR HEMORRHAGE AFTER ALLOGENEIC
HEMATOPOIETIC STEM CELL TRANSPLANTATION: TREAT-
MENT WITH RECOMBINANT FACTOR VIIA
Louis P. Voigt, MD*; Stephen M. Pastores, MD, FCCP; Esperanza
Papadopoulos, MD; Neil A. Halpern, MD, FCCP. Memorial Sloan
Kettering Cancer Center, New York, NY
INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a serious
complication that occurs in ⬍ 20% of patients after hematopoietic stem
cell transplantation (HSCT), with a mortality rate of 64%-100%.
1
Recom-
binant activated factor VIIa (rFVIIa) is an approved agent for the
treatment of bleeding episodes in patients with hemophilia when inhibi-
tors to factor VIII or IX are present.
2
We report a case of DAH after
allogeneic HSCT that was treated with rFVIIa in addition to standard
therapy.
CASE PRESENTATION: A 48-year-old male with non-Hodgkin’s
lymphoma (NHL) who received an allogeneic HSCT 14 months earlier
was admitted to the ICU after developing massive hemoptysis associated
with respiratory failure requiring mechanical ventilation. He had pre-
sented to the hospital 10 days earlier with mild hemoptysis. His platelet
count was 23,000/mm
3
and a chest x-ray (CXR) showed clear lung fields.
Chest CT revealed bilateral patchy alveolar opacities and small pleural
effusions. Fiberoptic bronchoscopy demonstrated old blood in the airways
but no active bleeding. Cultures and cytologic exam of bronchial washings
were negative for bacteria, AFB, fungi, and malignant cells. Despite
treatment with corticosteroids, antibiotics, and transfusions to keep the
platelet count ⬎50,000/mm
3
, his hemoptysis persisted. In the ICU, he was
mechanically ventilated but was afebrile and hemodynamically stable. The
physical examination was remarkable for bilateral basal rales. Laboratory
data were significant only for a hemoglobin level of 8.7 g/dL, and a platelet
count of 153,000/mm
3
. The CXR after intubation revealed bilateral patchy
alveolar opacities. Six hours into the ICU admission, rFVIIa was admin-
istered using a dose of 90 ␮g/kg IV every two hours for persistent massive
hemoptysis. The hemoptysis subsided rapidly after only two doses of
rFVIIa. No adverse effects of rFVIIa were observed. Fiberoptic bron-
choscopy 24 hours later showed old blood clots overlying the main carina
and both major bronchi with no active bleeding. After 1 week, the patient
was extubated with resolution of the alveolar infiltrates on CXR. He was
transferred to the ward and discharged home three weeks later. Following
treatment with rFVIIa, the patient exhibited no further hemoptysis.
DISCUSSION: The high morbidity and mortality rates of DAH after
HSCT
1
demand that novel therapeutic approaches be considered for this
serious condition. Recent advances in our understanding of hemostasis
and development of novel hemostatic agents have resulted in the safe and
successful application of these agents in clinical practice.
2
rFVIIa has been
shown to be safe and effective for life-threatening bleeds in patients with
hemophilia, congenital, and acquired thrombocytopathies. rFVIIa pro-
motes hemostasis primarily by enhancing thrombin generation on acti-
vated platelets independently of tissue factor.
2
rFVIIa should be used with
caution in patients with an increased risk for thrombotic events. The
administration of rFVIIa in our patient highlights the difficulties that
clinicians face when choosing to administer a new and expensive agent
(⬎$6,000 per dose) outside of the recommended indication. The dosage
regimen used in our patient was developed from hemophilia patients with
inhibitors, and not from experience in transplant patients. Therefore,
further study is required to determine whether rFVIIa is indeed beneficial
in this population, and if so, what the adequate dosage and frequency of
administration would be.
CONCLUSION: This case illustrates the successful use of rFVIIa in an
allogeneic HSCT patient with DAH. We propose that rFVIIa be consid-
ered in any patient who develops DAH after HSCT, in addition to
standard therapy, particularly when hemoptysis is massive and/or recur-
rent.
REFERENCES:
1. Afessa B et al: Diffuse alveolar hemorrhage in hematopoietic stem
cell transplant recipients. Am J Respir Crit Care Med 2002;166:641-
645
2. Hedner U: Recombinant factor VIIa (NovoSeven) as a hemostatic
agent. Dis Mon 2003;49(1):39-48
DISCLOSURE: L.P. Voigt, None.
ADULT RESPIRATORY DISTRESS SYNDROME (ARDS) AS THE
PRESENTING MANIFESTATION OF MILIARY TUBERCULO-
SIS (MLTB)
Lakshmipriya Kasirajan, MD*; K Gupta, MD; P Krishnan, MD. Coney
Island hospital, Brooklyn, NY
INTRODUCTION: ARDS as a complication during the course of
MLTB though rare has been reported.It usually occurs several weeks after
the onset of symptoms of TB.We describe a patient in whom ARDS was
the presenting manifestation of MLTB.Familiarity with this rare presen-
tation is necessary in order to consider tuberculosis in the setting of ARDS
even in the abscence of prior symptoms suggestive of tuberculosis
especially as MLTB is one of the few treatable causes of ARDS.
CASE PRESENTATION: A 42 year old Asian male presented with
fever,cough and increasing dyspnea of 3 days duration.Prior to this he was
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Acute Lung Injury/ARDS, continued
252S CHEST 2003—Case Reports

well and had immigrated to the United States 1 month ago.He was febrile
and found to be in severe respiratory distress with bilateral rales heard on
lung auscultation.The chest x-ray revealed bilateral diffuse air space
disease.The patient was severly hypoxic and on assist mode ventilation
with 500cc tidal volume and rate of 12/min,PEEP 7.5 cm of H
2
O the
plateau pressure was 38 cm of H
2
O and ABG on fio
2
0.6 revealed
PH7.48,PCO
2
24
,
PO
2
56 .WBC was 2300 with 18% lymphocytes,hemo-
globin 11g/dl and platelets 100K.Sodium was126,bicarbonate 18 and
anion gap 14 while renal function was normal.There was no evidence of
congestive heart failure. Bronchoalveolar lavage done on day 2 grew
mycobacterium tuberculosis on day 10 when parenteral rifampin,quin-
olone,capreomycin and enteral ethambutol,pyrazinamide, isoniazid via
nasogastric tube was started along with methylprednisolone at 1mg/
kg.HIV test was negative.
In 10 days fever decreased,oxygenation and ARDS improved but the
subsequent course was complicated by DIC, bilateral pneumothoraces
and ventilator assosiated pneumonia leading to the patient’s demise 7
days later.Autopsy revealed poorly formed granuloma with AFB
scattered through out both lungs,liver,spleen and adrenal glands.Hya-
line membrane formation and areas of pyogenic consolidation were
noted in both lungs.
DISCUSSION: Though uncommon ARDS has been described with
MLTB and to a lesser extent with TB pneumonia.Upto 1995,71 cases have
been described world wide.In Canada 13 cases of ARDS due to TB have
been reported,7 due to MLTB of a total of 37 cases(18.9%) and 6 of a total
of 722(.08%)due to TB pneumonia.
Mortality was 68% in MLTB and ARDS and 46% in TB pneumonia and
ARDS. Mean duration of symptoms was 2 – 4 weeks unlike 3 days in our
patient.Pathogenesis of ARDS in TB include cell wall lipoarabinonimo-
mine causing machrophage activation and tumor necrosis factor (TNF)
and interlukin lB production leading to alveolar capillary injury. Prognosis
is poor in spite of treatment.Therapeutic delay and difficulty in drug
administration in the critically ill may be one reason.Diagnostic clues
include pancytopenia, DIC and hyponatremia.Fiberoptic bronchoscopy-
,bone marrow examination and liver biopsy have the highest diagnostic
yield.
CONCLUSION: ARDS due to TB is uncommon and therefore may
lead to delay in diagnosis.ARDS is usually not an early complication of the
disease and thereby provides time for timely diagnosis if it is recognised
that TB can lead to ARDS.The case we describe draws attention to the
fact that ARDS may be the presenting manifestation of TB disease.Fa-
miliarity with this fact will allow for timely recognition and treatment of
TB in patients with ARDS.
REFERENCES:
1. Penner, C, Tuberculosis as a Primary Cause of Respiratory Failure
requiring Mechanical Ventilation, Am J Respir Crit Care Med Vol
151, pp 867-872, 1995.
2. Maartens, G, Miliary Tuberculosis and Outcome in 109 Treated
Adults. The American Journal of Medicine, September 1990, Vol-
ume 89, p 291.
DISCLOSURE: L. Kasirajan, None.
SEVERE VASCULAR LEAK SYNDROME FROM DENILEUKIN
DIFTITOX PRESENTING AS ACUTE RESPIRATORY DISTRESS
SYNDROME (ARDS)
Vosudesh Pai, MD*; Pratima Pandey, MD; Bruno DiGiovine, MD,
FCCP; Michael Iannuzzi, MD, FCCP. Henry Ford Hospital, Detroit, MI
INTRODUCTION: Denileukin diftitox (ONTAK) is a recombinant
cytokine-targeted fusion protein that delivers the catalytic domain of
diphtheria toxin to lymphoma cells expressing the interleukin-2 receptor
(IL-2R).Vascular leak syndrome(VLS) is a complication of ONTAK
therapy and is usually a self-limited illness
(1)
.The following is the first case
report of severe VLS presenting as ARDS.
CASE PRESENTATION: A 53-year-old healthy male with mycosis
fungoides received 5 daily infusions of ONTAK. Two days later he
developed intractable nausea, fever(41
o
C) and dyspnea progressing
over 48 hours to acute respiratory failure requiring intubation. His
PaO2/FiO2 ratio of 50, pulmonary capillary wedge pressure of 15
mmHg and chest-x-ray were consistent with a picture of ARDS. His cardiac
index was 4.4L/M
2
, serum albumin 2.6gms/dL, white blood cell count 44000
and all cultures were negative with no source of infection identified. His mean
arterial pressure dropped to 40mmHg and he received intravenous crystal-
loids, antibiotics, vasopressors, recombinant drotrecogin alfa and mechanical
ventilation. Within 48 hours his hypoxemia and shock resolved completely
leading to extubation despite a positive fluid balance of almost 10 litres.
DISCUSSION: Upto 27% of patients receiving denileukin diftitox
can develop VLS,usually a self-limited illness defined as the occurence
of at least 2 of the following: edema, hypoalbuminemia(⬍ 2.8gms/dL)
and/or hypotension occuring during days 1-14 of the treatment
(1).
The
low wedge pressure, normal cardiac index and recovery despite
non-response to diuretics and positive fluid balance makes congestive
heart failure an unlikely cause of this patient’s respiratory failure-
.Although fulfilling conventional criteria for ARDS
(2)
given the rapidity
of recovery it is more likely that severe VLS was the underlying
pathophysiology.
CONCLUSION: In patients receiving denileukin diftitox(ONTAK),
vascular leak syndrome(VLS) should be considered in the differential
diagnosis of ARDS.
REFERENCES:
1. Olsen E et al. Denileukin for the treatment of Cutaneous T-cell
Lymphoma. Journal of Clinical Oncology. 19(2):376-88, 2001 Jan
15.
2. Bernard GR et al.The American-European Consensus Conference
on ARDS. Am J Respir Crit Care Med 1994; 149:818.
DISCLOSURE: V. Pai, None.
EXTRA CORPOREAL MEMBRANE OXYGENATION FOR FUL-
MINANT HYPOXEMIC RESPIRATORY FAILURE AND PULMO-
NARY HEMORRHAGE DUE TO PERINUCLEAR ANTINEUTRO-
PHIL CYTOPLASMIC ANTIBODIES POSITIVE VASCULITIS IN
A CHILD
Hemant S. Agarwal, MD*; Venkatramanan Shankar, MD, FCCP; Mary B.
Taylor, MD; Marek J. Grzeszczak, MD; Harold Lovvorn, MD; Tracy E.
Hunley, MD; Kathy Jabs, MD. Vanderbilt University Medical Center,
Nashville, TN
INTRODUCTION: Diffuse pulmonary hemorrhage in children is rare
and a potentially life threatening situation. We present one such case
secondary to Perinuclear Antineutrophil Cytoplasmic Antibodies
(pANCA) positive systemic vasculitis requiring Extra Corporeal Mem-
brane Oxygenation (ECMO)
CASE PRESENTATION: A sixteen-year old Caucasian boy presented
to the emergency department with acute history of cough, hemoptysis and
severe respiratory distress. In the past six months, he had episodes of
cough and occasional hemoptysis; rhinorrhea and ankle swelling with
erythematous skin rashes which were treated symptomatically. Urine
examination revealed mild proteinuria and a renal ultrasound was re-
ported as normal.
Patient was emergently intubated endotracheally and mechanically
ventilated with high settings with FiO2 of 1.0 and PEEP of 16. Chest x-ray
revealed diffuse bilateral pulmonary infiltrates (Figure 1). Bronchial
secretions were sent to test for unusual infections. Urine examination
showed proteinuria and hematuria. Serologic investigations for systemic
vasculitis were sent.
Broad-spectrum anti-microbials and high dose methylprednisolone
therapy were started. Patient rapidly deteriorated with progressive
hypoxemia and pulmonary air-leaks. A brief trial of high frequency
ventilation was ineffective. Patient was emergently placed on veno-
venous ECMO. pANCA levels were positive the next day . Patient was
started on a regimen of plasmapheresis, pulse methylprednisolone and
cyclophosphamide. Patient showed dramatic improvement in 72 hour-
s.He was weaned off ECMO on the sixth day and extubated the next
day (Figure 2). Renal biopsy showed pauci-immune necrotizing cres-
centic glomerulonephritis.
Patient was discharged home on maintenance steroid therapy.
DISCUSSION: pANCA postive systemic vasculitis with diffuse alveo-
lar haemorrhage and fulminant hypoxemic respiratory failure as a pre-
senting feature is quite uncommon in children. Aggressive supportive care
including ECMO can be life-saving (1) while the autoimmune vasculitis is
being treated with pulse dose steroids, cyclophosphamide and plasma-
pheresis.
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CONCLUSION: ECMO is an effective and safe supportive manage-
ment strategy for fulminant acute hypoxemic respiratory failure secondary
to pANCA positive systemic vasculitis even in the presence of pulmonary
hemorrhage.
REFERENCES:
1) Kolovos NS et al. Extracorporeal life support for pulmonary haem-
orrhage in children: a case series. Crit Care Med 2002; 30(3):577-80
DISCLOSURE: H.S. Agarwal, None.
Lung Disease in Immunocompromised
Patients
4:15 PM - 5:45 PM
PULMONARY CRYPTOCOCCOSIS AFTER INITIATION OF
ANTI-TUMOR NECROSIS FACTOR-ALPHA THERAPY
Chadi A. Hage, MD*; Karen L. Wood, MD; George Sarosi, MD; Kenneth
S. Knox, MD. Indiana University, School of Medicine, Indianapolis, IN
INTRODUCTION: Pulmonologists often participate in the care of
immunocompromised patients as the majority of infections in this population
involve the lungs. The infectious complications associated with the use of
traditional cytotoxic agents are well known. The immunosuppressive effect of
targeted anti-cytokine therapy, however, is now being defined. Infliximab, a
chimeric monoclonal antibody against Tumor Necrosis Factor-alpha (TNF-
␣), has revolutionized the care of patients with rheumatoid arthritis. Inter-
estingly, the spectrum of infectious disease associated with the use of
anti-TNF-␣ therapy is similar to patients with AIDS and include tuberculosis,
histoplasmosis, and cryptococcosis. This suggests that anti-TNF-␣ therapy
produces a downstream defect in the T helper-1 arm of immunity.
We report a case of pulmonary cryptococcosis after the initiation of
infliximab.
CASE PRESENTATION: A 61-year-old man with a 6 year-history of
advanced rheumatoid arthritis was admitted to the hospital for shortness
of breath and anemia. His medications included prednisone 10mg daily,
methotrexate 25mg weekly, and leflunomide 25mg daily. He was started
on infliximab and received three doses at 3mg/kg ideal body weight, the
last dose being administered three weeks prior to presentation.
He denied fever, chills, night sweats, chest pain and weight loss. He
lives in a trailer home surrounded by large trees. Physical examination
revealed a temperature, 99.8°F; respiratory rate, 20 breaths/min; blood
pressure, 125/61 mmHg; pulse, 85 beats/min; room air oxygen saturation,
96%. Lungs were clear to auscultation. He had upper extremity synovitis
and rheumatoid skin nodules.
Admission bloodwork was remarkable for a normal cell count and
differential, hemoglobin 7.1. The biochemistry profile and liver enzymes
were normal. Chest radiograph showed a new round opacity with adjacent
airspace disease in the right lower lobe (Fig 1). Subsequent testing
included a non-reactive tuberculin skin test, negative fungal serologies,
negative serum cryptococcal antigen and a negative HIV screen. A
CT-guided fine needle aspiration of the parenchymal lesion was per-
formed. Direct smear showed “few fungal elements” and mixed inflam-
matory cells. Seventeen days later, fungal cultures grew Cryptococcus
neoformans. Infliximab therapy was discontinued. The patient was treated
with amphotericin-B, followed by fluconazole maintenance therapy, with
significant improvement of his respiratory symptoms.
DISCUSSION: The lung is the site of primary infection with Crypto-
coccus neoformans. Containment and effective control of this infection
requires intact host defenses. Immunosuppression can lead to dissemina-
tion to the central nervous system and reticuloendothelial system with
significant morbidity and mortality.
TNF-␣ plays a major role in the generation of cell-mediated immunity
to cryptococcal infection. Moreover, TNF-␣ is essential in maintaining a
Th-1 immune response as it induces the production of IL-12 and IL-18,
with subsequent production of fungicidal IFN-␥.
TNF-␣ blockade has become a popular anti-inflammatory strategy in
the treatment of rheumatoid arthritis and Crohn’s disease. Despite
reported safety of these agents, infectious complications have been linked
to the use of infliximab and etanercept, the two anti-TNF-␣ agents
currently available in the United States. Since their approval, the FDA’s
Adverse Event Reporting System has reported four cases of cryptococco-
sis related to the use of etanercept but none with infliximab.
CONCLUSION: We describe a patient with pulmonary cryptococcosis
that developed shortly after initiation of infliximab for severe rheumatoid
arthritis. The diagnosis was made early and appropriate therapy was initiated
before evidence of dissemination. The infection occurred early, within 2
months of initiating therapy, suggesting that infectious complications can
occur quickly. New pulmonary infiltrates in a patient receiving anti-TNF-␣
Monday, October 27, 2003
Acute Lung Injury/ARDS, continued
254S CHEST 2003—Case Reports

therapy should be aggressively pursued, as infections are common and delay
in diagnosis is likely to be associated with disseminated disease and significant
morbidity.
This case should alert clinicians to the increased incidence of pulmo-
nary mycoses in patients receiving anti-TNF-␣ therapy.
DISCLOSURE: C.A. Hage, None.
TSUKAMURELLA INFECTION IN AN IMMUNOCOMPRO-
MISED PATIENT
Juan P. Gomez, MD*. University of Miami, Miami, FL
INTRODUCTION: Opportunistic infections in immunosuppressed
patients are common. However, as the life expectancy of patients with
AIDS increases, the number of new or less common pathogens diagnosed
has increased significantly. This is the case of a patient with AIDS who
developed a pulmonary infection with Tsukamurella sp.
CASE PRESENTATION: Patient is a 55 year old male with AIDS
(CD4 count 116), on antiretroviral therapy, who presented with two weeks
of fever, malaise and cough productive of white sputum.
Past history significant for cutaneous T-cell lymphoma, no allergies, and
120 pack-year tobacco use.
Physical exam: normal vital signs, weight 122lbs. Head and neck examina-
tion is normal. Chest exam significant for decreased breath sounds bilaterally.
Abdominal and extremities examination unremarkable. Skin examination
shows lesions compatible with cutaneous T-cell lymphoma.
Chest CT scan shows “shotty” adenopathy in the aorto-pulmonary
window and left hilum. There is a thick wall, irregular, right upper lobe
cavitary lesion (Fig. 1). Similar lesions are seen with peripheral parenchy-
mal opacities in the left upper lobe, left lower lobe, and middle lobe.
Mild anemia (Hemoglobin 11.9) and MCV 108. Normal chemistry and
liver function tests. Viral load 1,361 copies.
Sputum examination 3 times for AFB was negative; gram stain showed
normal respiratory flora.
Bronchoscopy was normal. Bronchoalveolar lavage (BAL) showed normal
respiratory flora. Transbronchial biopsies of the right upper lobe showed
bronchial wall and lung parenchyma with mild chronic inflammation.
Upon follow up, cultures grew Tsukamurella species. Repeat cultures
were sent in sputum and Tsukamurella grew again.
Patient was placed on Ciprofloxacin and Rifabutin (based on previously
reported sensitivities). He had remission of symptoms and improvement
of radiographic findings (Fig. 2). Sensitivities could not be confirmed in
the laboratory, since the organism could not be re-grown.
DISCUSSION: Initially, differential diagnosis included infections,
such as Tuberculosis, Nocardiosis, Rhodococcus Equi, Actinomycosis,
pulmonary mycoses, Sporotrichosis and parasitic. Non infectious possibil-
ities included Wegener’s Granulomatosis, Lymphoma, Carcinoma, Rheu-
matoid Lung Disease, Sarcoidosis, and Langerhan’s Cell Histiocytosis.
Tsukamurella, Nocardia, Gordona and Rhodococcus are mycolic acid-
containing bacteria classified as Nocardioform Actinomycetes. Infections
due to these organisms have been well reported, except for those due to
Tsukamurella sp. T. paurometabola, T. pulmonis, inchonensis, T. wratisla-
viensis, and T. tyrosinosolvens have been described.
These organisms were named after Tsukamura, a Japanese microbiol-
ogist who in 1971 described infection by Gordona aurantiaca in sputum of
patients with chronic lung disease. In 1988, this organism was reclassified
as Tsukamurella paurometabola. Infection has not been reported on
immunosuppressed patients with HIV, most are related to malignancy.
There are10 reported cases of Tsukamurella infections, only one in the
lung. That case was due to Tsukamurella inchonensis, isolated from lung
tissue of a patient with a necrotic tumor. These organisms are usually
considered a contaminant, until they are isolated repeatedly.
The natural habitat is unknown. Tsukamurella paurometabolum has
been isolated from sputum, a mycetoma, and as pseudo-infection due to
laboratory contamination.
Repeated isolation and no evidence of contamination are most helpful
in making the diagnosis, as well as lack of other pathogens, and good
response to therapy. It is difficult to determine antimicrobial susceptibil-
ities, since these are slow-growing organisms and do not give a smooth
suspension of cells.
Treatment is often based on previously reported susceptibilities. These
organisms have been sensitive to Amikacin, Clarithromycin, Ciprofloxacin,
Imipenem, and Sulfamethoxazole.
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CONCLUSIONS: Tsukamurella is an uncommon pulmonary pathogen
in immunosuppressed patients. Repeated isolation is important for diag-
nosis. Treatment is based on previously reported sensitivities and in the
response to it. This organism should be included in the differential
diagnosis of lung infections in immunosuppressed patients.
REFERENCES:
-Clin Infect Dis 1992;14:1015-22
-Clin Infect Dis 1997;24:1267-8
-Clin Infect Dis 2002;35:e72-7
DISCLOSURE: J.P. Gomez, None.
BORDETELLA BRONCHISEPTICA INFECTION IN AN IMMU-
NOSUPPRESSED PATIENT
Satish C. Chada, MBBS*; Jennifer A. LaRosa, MD; Michael V. Cutaia,
MD; Ravindra M. Mehta, MD. SUNY Downstate, Brooklyn, NY
INTRODUCTION: Infection with Bordetella bronchiseptica, a pleo-
morphic gram-negative coccobacillus is rare in humans. About 35 cases
have been reported, predominantly in hiv or immunosuppressed patients
with exposure to dogs. We report a case of this infection in an immuno-
suppressed individual with exposure to cats.
CASE PRESENTATION: 55-year black male presented with 5 days of
fever, chills and productive cough. Past medical history included diabetes,
end stage renal disease and renal transplant (1994) on predisnone and
cylcosporine, and treatment for tuberculosis (1971). Other relevant details
included a significant smoking history and two pet cats. Physical exam was
unremarkable. Chest xray showed a new right upper lobe infiltrate. Due
to the acute presentation, he was treated for community acquired
pneumonia with ampicillin/sulbactam and azithromycin.
He returned 2 months later with persistent cough and blood-tinged
sputum. Chest imaging showed a persistent right upper lobe infiltrate,
with partial scarring. Three sputum samples were negative for acid-fast
bacilli. Bronchoscopy was unremarkable, but bal cultures of the affected
segment were positive for Bordetella bronchiseptica, which was sensitive
to quinolones/aminogycosides, and resistant to ampicillin/cephalosporins.
He recieved ciprofloxacin for 4 weeks, with complete resolution of
symptoms and infiltrate/scarring on chest imaging
DISCUSSION: Most cases of Bordetella bronchiseptica infection are
associated with respiratory infections in hiv and immunosuppressed
individuals, though it may also occur in the immunocompetent host. The
organism is isolated from sputum cultures, nasal swab, or BAL fluid.
Treatment is based on results of susceptibility testing, Bactrim or
aminoglycosides are empirical options. Contact with animals is important.
In our patient, the infection was likely acquired from a cat, and of note
Bordetella bronchiseptica culture-positive cats may be clinically normal.
The exact duration of treatment is not clear. This case also emphasizes the
importance of bal in immunocompromised patients in isolating unusual
organisms, especially in the setting of failure of empirical therapy.
CONCLUSION: Bordetella bronchiseptica infection is a treatable
cause of infiltrates in immunosuppressed patients, especially with a history
of animal contact.
DISCLOSURE: S.C. Chada, None.
MYCOBACTERIUM TUBERCULOSIS INFECTION OF THE
MOUTH AND LOWER GASTROINTESTINAL TRACT COMPLI-
CATING LUNG TRANSPLANTATION
James J. Carswell, MD*; Mark K. Robbins, MD. University of Virginia,
Charlottesville, VA
INTRODUCTION: Despite the immunosuppression required after-
ward, tuberculosis (TB) uncommonly complicates lung transplantation.
Medical literature contains only thirteen reports, and our experience is
similarly limited. We describe our first TB infection of 196 transplants, an
unusual case of Mycobacterium tuberculosis infecting the mouth and
lower gastrointestinal tract.
CASE PRESENTATION: A 62 year-old Caucasian female underwent
left lung transplantation for severe emphysema. After successful treat-
ment of acute rejection and cytomegalovirus infection, she was maintained
on prednisone, tacrolimus, azathioprine, acyclovir, and trimethoprim/
sulfamethoxazole. Her condition improved, with increasing exercise tol-
erance, but she was hospitalized 16 months later with vomiting and
dehydration. An Alternaria skin lesion was found and treated with
itraconazole. Vomiting continued over the next month, and she was
admitted to our hospital with dehydration, fever, and a new painful mouth
lesion. Examination revealed an ulcerated erythematous 5-millimeter hard
palate lesion. Lungs were clear with diminished right breath sounds, and
cardiovascular, abdominal, and extremity examination were normal. Eval-
uation revealed acute renal failure, tacrolimus level of 23.7ng/mL (normal
5-17), and white blood cell (WBC) count 4.8. Chest radiograph showed no
infiltrates or scarring.
Pain and fever persisted, and she developed melena. Biopsy of mouth
and colonic lesions both revealed acid-fast bacilli with the appearance of
atypical mycobacteria. She was started on clarithromycin, rifabutin, and
ethambutol, and her symptoms improved. Two weeks later cultures grew
Mycobacterium tuberculosis, and isoniazid was added to her regimen,
leading to resolution of the mouth lesions.
DISCUSSION: Tuberculosis has been reported uncommonly among
lung transplant recipients. This most likely is a reflection of two facts: a)
lung transplantation is relatively rare; and b) post-transplant survival time
is comparatively short. This case adds to thirteen previously reported.
Nearly all reported cases have been pulmonary in origin and likely due
to re-activation of latent TB. This patient’s enhanced immunosuppression,
evidenced by a high tacrolimus level and low WBC count, suggest her
infection was due to re-activation as well, though no evidence of prior
pulmonary TB was found. Most likely she expectorated and then swal-
lowed mycobacteria, leading to oro-gastrointestinal infection.
Complications post-transplantation pose a particular diagnostic chal-
lenge. Immunosuppression induces susceptibility to many organisms not
usually pathogenic, and infections present in unusual ways. Physicians
must maintain a high index of suspicion and a low threshold for additional
testing to establish a diagnosis and develop the proper treatment plan. In
this case, the key to the diagnosis was biopsy and culture of lesions
identified by history and examination.
Therapy for suspected infectious complications following transplanta-
tion should be aggressive. Typically anti-infective therapy is broad initially,
with culture results as a guide when available. In this case full anti-
tuberculous therapy was withheld at first, as suspicion for atypical
mycobacteria was very high. While symptoms began to improve on limited
therapy, addition of isoniazid was required for full recovery.
CONCLUSION: As more lung transplants are performed and survival
time lengthens, TB will likely become established as an important
pathogen. It can present in unusual ways and requires an aggressive
approach for diagnosis and therapy. If treated properly, TB infection, even
in the immunosuppressed population, can be overcome.
Monday, October 27, 2003
Lung Disease in Immunocompromised
Patients, continued
256S CHEST 2003—Case Reports

REFERENCES:
Kesten S and Chaparro C. Mycobacterial infections in lung
transplant recipients. Chest 1999;115:741-45.
Malouf MA and Glanville AR. The spectrum of mycobacterial
infection after lung transplantation. Am J Respir Crit Care Med
1999;160:1611-16.
Miller RA et al. Mycobacterium tuberculosis in lung transplant
recipients. Am J Respir Crit Care Med 1995;152:374-6.
Schulman LL et al. Pulmonary tuberculosis after lung
transplantation. Chest 1997:111:1459-62.
Singh N and Paterson DL. Mycobacterium tuberculosis infection in
solid-organ transplant recipients: impact and implications for
management. Clin Infect Dis 1998;27:1266-77.
DISCLOSURE: J.J. Carswell, None.
ENDOBRONCHIAL ASPERGILLUS IN AN IMMUNOCOMPRO-
MISED HOST WITH RESPIRATORY FAILURE AND NEW ON-
SET WHEEZING
Cristina A. Reichner, MD*; Charles A. Read, MD FCCP; Eric D.
Anderson, MD FCCP. Georgetown University Medical Center, Washing-
ton, DC
INTRODUCTION: The new onset of wheezing in an immunocom-
promised can herald serious endobronchial pathology.
CASE PRESENTATION: We report a case of a 39 year old female
with human immunodeficiency virus (HIV) well controlled on highly
active antiretroviral therapy (CD4 596 and viral load ⬍ 50 copies/mL) and
no prior pulmonary disease who was admitted to the intensive care unit
with fever of unknown origin, lactic acidosis and thrombotic thrombocy-
topenic purpura. She was intubated, started on broad spectrum antibiot-
ics, placed on continuous venous-venous filtration, plasmapheresed and
given methylprednisolone 60mg IV Q6h. A bone marrow biopsy revealed
B cell lymphoma. She was given vincristine, prednisone and procarbazine
(CHOP) chemotherapy and became neutropenic. On the third day after
CHOP, she remained febrile and was empirically started on liposomal
amphotericin. On the fifth day, she developed diffuse bilateral wheezing
and elevated peak pressures unresponsive to albuterol nebulizers or
diuresis. Her chest X-ray showed new right upper and lower lobe collapse
(Figure 1). A bronchoscopy revealed plaque-like growths coating the
airways diffusely (Figure 2 at the level of the carina and figure 3 of the
right bronchus intermedius) and obstructing the right upper lobe bron-
chus (Figure 4). Bronchial brushings and biopsies were positive for
septate hyphae consistent with Aspergillus. The patient was switched to
voriconazole, caspofungin and aerosolized amphotericin but her condition
continued to deteriorate with hypercapnia and hypotension. Care was
withdrawn.
DISCUSSION: Tracheobronchitis is an uncommon manifestation of
Aspergillus with infection limited entirely or predominantly to the tra-
cheobronchial tree. The patient population at risk for this form of
Aspergillus includes primarily patients with HIV, neutropenia and lung
transplant recipients. Early in the course of infection, it can go undiag-
nosed as it often occurs in the setting of a normal chest X-ray. Clinical
manifestations include wheezing, cough, hypoxia and hemoptysis. Diag-
nosis is established by bronchoscopy with biopsy or brushings. The
treatment includes systemic amphotericin B. There have been some case
reports of aerosolized amphotericin B with variable clinical response. The
outcome is poor. Patients can progress to transmural necrosis of the
airways, tracheal perforation or invasive pulmonary Aspergillosis.
CONCLUSION: In immunocompromised patients with respiratory
failure, the differential diagnosis of new onset wheezing not responsive to
conventional treatments should include endobronchial fungal infections
and should prompt early investigation by bronchoscopy.
REFERENCES:
1. Marr KA, Patterson T, Denning D. Aspergillosis: Pathogenesis,
clinical manifestations, and therapy. Infect Dis Clin Am 2002; 16:
875-894.
Monday, October 27, 2003
Lung Disease in Immunocompromised
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2. Boots RJ, Paterson DL, Allworth AM et al. Successful treatment of
post-influenza pseudomembranous necrotizing bronchial aspergillo-
sis with liposomal amphotericin, inhaled amphotericin B, gamma
interferon and GM-CSF. Thorax 1999; 54: 1047-1049.
3. Tait RC, O’Driscoll BR, Denning DW. Unilateral wheeze caused by
pseudomembranous aspergillus tracheobronchitis in the immuno-
compromised host. Thorax 1993; 48: 1285-1287.
4. Routsi C. Aspergillus bronchitis causing atelectasis and acute respi-
ratory failure in an immunocompromised patient. Infection 2001;
29: 243-244.
DISCLOSURE: C.A. Reichner, None.
HYPOGAMMAGLOBULINEMIA FOLLOWING LUNG TRANS-
PLANTATION IS ASSOCIATED WITH SEVERE RECURRENT
INFECTIONS
Jessie S. Wilt, MD*; Selim M. Arcasoy, MD; Joshua Sonett, MD; Steven
Kawut, MD. Columbia University, New York, NY
INTRODUCTION: The leading cause of death in the first year
post-lung transplantation is infection. Efforts to reduce mortality from
infectious complications include prophylaxis against the most common
opportunistic pathogens. Herein we report a case of significant hypoga-
mmaglobulinemia following lung transplantation complicated by frequent
infections and treated successfully with intravenous gammaglobulin.
CASE PRESENTATION: SB was a 52 year-old female with extensive
emphysema. She received a left single lung transplant on March 11, 2002.
Her immunosuppressive regimen consisted of cyclosporine, mycopheno-
late mofetil and prednisone. Prophylactic medications included Bactrim,
IV Ganciclovir for 90 days followed by oral valganciclovir, IV CMV-
hyperimmune globulin and Nystatin oral suspension. Three days post-
discharge she experienced increasing dyspnea, chest pain and fevers. A
bronchoscopy with transbronchial biopsy revealed minimal acute rejec-
tion. She was treated for acute rejection with 1 gram of methylpred-
nisolone for three days, and had resolution of her fever and hypoxemia.
On May 13, 2002, the patient presented with three days of fever and
cough. Blood and sputum cultures were positive for methicillin-resistant
Staphylococcus aureus. The patient completed a two week course of
intravenous vancomycin.
On August 7
th
, bronchoscopy with endobronchial biopsies of a lesion
at the anastomosis revealed Aspergillus infection. Surveillance bron-
choscopy performed September 17, 2002 revealed moderate acute
cellular rejection (Grade A2B3). The patient was treated with 1 gram
of methylprednisolone for three days. One week later, the patient
developed worsening dyspnea, fever and cough. A chest radiograph
revealed opacification of the left lower lobe. Bronchoscopy performed
was positive for MRSA. On October 10th serum IgG was 443 mg/dl. A
sample of preoperative serum was obtained from storage; IgG was 614
mg/dl. The patient was started on intravenous gammaglobulin
(Gamimmune) 20 grams every four weeks. She has not experienced
any subsequent episodes of infection or rejection.
DISCUSSION: Immunoglobulin deficiency in solid organ transplan-
tation is poorly described. In the only article on hypogammaglobulinemia
in lung transplant recipients, Goldfarb et al reported on 67 patients with
post-transplant humoral immune surveillance data. Forty-seven patients
had IgG levels less than 600 mg/dl, and 25 had levels less than 400 mg/dl.
They document a significantly increased incidence of infections in the
“lowest IgG” group (IgG ⬍ 400 mg/dl), with invasive aspergillosis
occurring only in the hypogammaglobulinemic groups (3).
This group published a study of nine cardiac transplant recipients with
severe post-transplant HG (IgG ⬍ 350 mg/dl) who received pre-emptive
immunoglobulin replacement therapy (5), and compared these patients to
eleven patients with severe HG from a prior study. There was a significant
decrease in clinical opportunistic infections. This study is the first to
demonstrate the clinically utility of immunoglobulin replacement therapy
in severe HG following solid organ transplantation.
Our patient did not suffer from clinical CMV infection. This
probably was the result of intensive and prolonged prophylaxis. Her
only opportunistic infection was aspergillosis of the airway and other-
wise presented with staphylococcal disease. Although the targets of
immunosuppression regimens are the T cell dependent pathways,
calcineurin inhibitors, corticosteroids and purine antagonists can all
affect B cell proliferation and immunoglobulin production (6, 7). A
hypothesis can be made that the observed IgG deficiency results from
the indirect action of T cell inhibition. However, given the overlap
between T cell and B cell receptor/intracellular signaling, it would be
likely that the immunosuppressive agents are exerting a direct effect on
B cell function.
CONCLUSION: Hypogammaglobulinemia is a significant factor con-
tributing to the risk of infection following solid organ transplantation.
Further studies are warranted to characterize the risk factors for the
development of HG, and to assess the benefit of immunoglobulin
replacement on infection risk and patient survival.
DISCLOSURE: J.S. Wilt, None.
Interstitial Lung Disease
4:15 PM - 5:45 PM
ETARNERCEPT THERAPY FOR IDIOPATHIC PNEUMONIA SY-
DROME AFTER ALLOGENEIC HEMATOPOIETIC STEM CELL
TRANSPLANTATION IN AN ADULT
Kevin H. Wallace, MD*; Stephen F. Grinton, MD; Han Tun, MD. Mayo
Clinic Jacksonville, Jacksonville, FL
INTRODUCTION: Noninfectious pulmonary complications are var-
ied and include diffuse alveolar hemorrhage, idiopathic pneumonia
syndrome, bronchiolitis obliterans, bronchiolitis obliterans with organizing
pneumonia, diffuse alveolar damage, lymphocytic interstitial pneumonia
and nonclassifiable interstitial pneumonia. The therapeutic mainstay for
these syndromes is the administration of immunosuppressant therapy in
the form of corticosteroids. However, there is variable response to therapy
depending on the syndrome. Bronchiolitis obliterans with organizing
pneumonia responds well to therapy with corticosteroids. On the other
hand, idiopathic pneumonia syndrome is far less responsive. The clinical
response to standard therapy with high dose corticosteroids is limited.
Ultimately, the diagnosis of idiopathic pneumonia is associated with
mortality rates of 50% to 70%.
In a recent case series, etanercept, a dimeric tumor necrosis factor
alpha binding protein, and corticosteroids were administered to 3 consec-
utive pediatric patients as an alternative therapeutic regimen for the
treatment of idiopathic pneumonia syndrome. In each case etanercept was
well tolerated and resulted in significant improvement in pulmonary
dysfunction.
Monday, October 27, 2003
Lung Disease in Immunocompromised
Patients, continued
258S CHEST 2003—Case Reports

CASE PRESENTATION: A 53 year old female underwent allogeneic
bone marrow transplant for M1 acute myelogenous leukemia. Her
hospital course was uneventful and she was discharged after 2 weeks in
the hospital. Five months after discharge from the hospital, the patient
developed dyspnea with exertion, fevers and a non productive cough.
Pulmonary function testing revealed moderate restriction. Computed
tomography of the chest which revealed diffuse patchy ground glass
opacities throughout both lung fields. Bronchoscopy with bronchoalveolar
lavage revealed no evidence of infection with bacterial, viral or fungal
organisms. Examination of transbronchial lung biopsy samples revealed
histopathological findings consistent with idiopathic pneumonia syndrome
after bone marrow transplantation. Etanercept at a dose of 25 mg twice a
week in combination with prednisone at 1 mg/kg was initiated. The patient
experienced symptomatic improvement after 7 days of therapy. Therapy
was continued for 4 weeks. At the completion of therapy, there was a
regression in pulmonary infiltrates and improvement in lung function.
DISCUSSION: Idiopathic pneumonia syndrome is very serious and
life threatening complication of bone marrow transplatation. The National
Institutes of Health defines idiopathic pneumonia syndrome as wide-
spread alveolar injury in the absence of active lower respiratory tract
infection following bone marrow transplantation. The time course to the
development of symptoms is variable but the prognosis is ultimately poor
with reported mortality rates up to 70%.
Several hypotheses exist regarding the pathogenesis of idiopathic
pulmonary syndrome. Radiation therapy induced injury, cytokine media-
tion and occult infection have all been implicated in the pathogenesis of
idiopathic pneumonia syndrome. Increasing evidence is mounting that
points to cytokine mediation, namely tumor necrosis factor alpha, as an
integral contributor to lung injury following bone marrow transplantation.
There are elevated levels of tumor necrosis factor alpha in the serum of
patients that develop lung injury after bone marrow transplantation.
Additionally, neutralization of tumor necrosis factor alpha after bone
marrow transplantation reduced the severity of idiopathic pneumonia
syndrome in an experimental model. These findings in combination with
the marked clinical and radiographic improvement in our patient point to
etanercept, and tumor necrosis factor inhibition, as a central part in the
treatment of idiopathic pneumonia syndrome
CONCLUSION: The combination of etanercept and corticosterid is a
viable therapy for adult patients with clinical presentation consistent
idiopathic pneumonia syndrome. The therapy is well tolerated and was
associated with significant clinical and radiographic improvement. The
poor prognosis previously associated with idiopathic pneumonia syndrome
and the prompt response to therapy in this case highlights the need for
further clinical trials in pediatric and adults with noninfectious lung injury
after allogeneic bone marrow transplantation
DISCLOSURE: K.H. Wallace, None.
SARCOIDOSIS DEVELOPING DE NOVO AFTER ORTHOTOPIC
LIVER TRANSPLANTATION
Sean T. Devine, MD*; Ganesan Murali, MD; Richard Sacks, MD;
Gonzalo Gianella, MD; Glenn Eiger, MD. Albert Einstein Medical
Center, Philadelphia, PA
INTRODUCTION: Liver transplantation is a therapeutic option for
multiple causes of end stage liver disease. As we become more experi-
enced and develop new methods of preventing rejection of the trans-
planted organ, unforeseen effects will be more common. We present a
case of sarcoidosis developing de novo after orthotopic liver transplanta-
tion.
CASE PRESENTATION: A 53 year old white male presented for
further evaluation of shortness of breath of two months duration. His past
medical history was significant for cryptogenic cirrhosis, an orthotopic
liver transplant performed 1
3
⁄
4
years prior to current presentation, and
hypothyroidism. The donor was an otherwise healthy female who died
from a cerebral vascular accident. His dyspnea was accompanied by a
non-productive cough and right-sided chest discomfort that worsened
with inspiration. The patient was limited to walking two blocks. He
reported no fever, weight loss or other systemic symptoms. The patient
was doing very well post-transplant except for an episode of pancreatitis 1
1
⁄
2
years post transplant. Two months later, he developed the shortness of
breath. At presentation, the patient’s medications were tacrolimus, levo-
thyroxine, a multi-vitamin, magnesium, and calcium. The patient had
stopped taking prednisone one year prior to presentation.
The physical exam was remarkable for an afebrile, well appearing male
with bilateral basilar crackles. Significant labs included a room air pulse
oximetry of 85% at rest and an ABG on 28% oxygen of 7.42/42/104/97%.
A CT scan of the chest revealed bilateral basilar infiltrates and minimal
adenopathy. All other labs including acid fast bacilli, viral, fungal, and
routine cultures from a bronchoalveolar lavage were negative. A lung
biopsy obtained by means of a video-assisted thoracoscopy and a conjunc-
tival biopsy revealed abundant non-caseating granulomas. All biopsy
cultures were negative.
DISCUSSION: Sarcoidosis is a multisystem disorder of unknown
cause(s). The diagnosis is established when clinicoradiological findings are
supported by histological evidence of noncaseating epithelioid cell gran-
ulomas. Granulomas of known causes and local sarcoid reactions must be
excluded.[1]
The development of sarcoid in a patient receiving an immunosuppres-
sant drug regimen is an apparent contradiction. IL-2 is an important
mediator in the development of sarcoidosis. Tacrolimus was included in
this patient’s immunosuppressive regimen. Tacrolimus’ mechanism of
action is believed to be the inhibition of T lymphocyte activation and the
subsequent production of IL-2 and several other cytokines such as IL-3,
IL-4, GM-CSF, TNF alpha, and interferon gamma.
To our knowledge, this is only the second case of post-transplant de
novo sarcoidosis reported in the literature.[2] That this can occur seems
immunologically paradoxical; however, it may not be completely unex-
pected. There are numerous cases in the literature reporting the recur-
rence of sarcoidosis post-transplant and the development of sarcoidosis in
patients with B and T cell immunodeficiencies. Tacrolimus and cyclospor-
ine have similar mechanisms of action, and it has been demonstrated that
cyclosporine has not been effective in treating sarcoidosis.[3]
This case highlights the fact that there may be multiple immunologic
mechanisms involved in the pathogenesis of sarcoidosis.
CONCLUSION: We present a case of sarcoidosis that developed de
novo after liver transplantation while receiving immunosuppressive ther-
apy. This provides further evidence that the pathogenesis of sarcoidosis
may involve more than T-cell activity.
REFERENCES:
[1] ATS Hunninghake GW, Costabel U, Ando M, et al. Statement on
sarcoidosis (the joint statement of the American Thoracic Society,
the European Respiratory Society, and the World Asoociation of
Sarcoidosis and Other Granulomatous Disorders). Am J Respir Crit
Care Med 1999; 160:736–755.
[2] Schmidt RJ, Bender FH, Chang WWL, Teba L: Sarcoidosis After
Renal Transplantation Transplantation 1999: 68: 1420-1423
[3] Semenzato G, Agnostini C: Lung transplantation in sarcoidosis:
Lessons learned from immunology. Sarcoidosis Vasculitis and Dif-
fuse Lung Diseases 1999:16: 21-23.
DISCLOSURE: S.T. Devine, None.
TUBEROUS SCLEROSIS AND LYMPHANGIOLEIMYOMATO-
SIS: AN UNUSUAL CAUSE OF RECURRENT PNEUMOTHORAX
IN A MAN
Dean A. Nasser, MD*; Nereida A. Parada, MD, FCCP; Blesilda Quinio-
nes-Ellis, MD, FCCP. Tulane University Health Sciences Center, New
Orleans, LA
INTRODUCTION: Lymphangioleimyomatosis (LAM) is a rare histo-
logic finding seen in tuberous sclerosis (TSC), a multisystem autosomal
dominant disorder seen almost exclusively in women. We report the case
of a man with TSC and LAM presenting with recurrent pneumothorax.
CASE PRESENTATION: The patient is a 29-year-old African-Amer-
ican male smoker with 3 biological children, diabetes, hypertension,
chronic renal insufficiency, spontaneous pneumothorax 6 months ago,
presenting with dyspnea and right shoulder pain after falling. He denies
chest pain and seizure. He is a hospital custodian and denies exposures or
pets.
Exam reveals blood pressure, 170/90,room air oxygen saturation of 97%
and otherwise stable vital signs. He appears comfortable with clear
symmetric breath sounds. He has multiple facial subcentimeter flesh color
papules and nodules. The remainder of his exam is unremarkable.
The abnormal lab is a creatinine of 2.2mg/dl. Chest x-ray shows diffuse
reticular infiltrates and a small right side pneumothorax. A chest CT shows
the right pneumothorax and multiple bilateral thin walled cysts.
After a chest tube and a persistent air leak, the patient had thoracos-
copy, biopsy and pleuradesis.
The biopsy showed LAM and multifocal micronodular pneumoncyte
hyperplasia (MNPH).
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A renal ultrasound showed renal cysts and a brain MRI showed an
archnoid cyst and a subependymal nodule. Pulmonary function testing
showed only an abnormal DLCO, 60% predicted.
DISCUSSION: TSC affects the skin, CNS, kidneys, heart and lungs.
Pulmonary pathology includes MNPH, clear cell micronodules, angio-
myolipoma, localized angiomyolipoma-like infiltrative lesion and LAM.
With a prevalence of 1 per 1.1 million, LAM almost exclusively affects
premenopausal women and in women with TSC its prevalence is 4-34%,
with few recent cases in men. LAM is characterized by a proliferation of
unique smooth muscle cells that can present with pulmonary manifesta-
tions causing progressive respiratory failure resulting in death or trans-
plantation.
CONCLUSION: LAM pathology is exceedingly unusual in men,
presenting therapeutic challenges that cannot be treated by hormonal
modulation.
DISCLOSURE: D.A. Nasser, None.
IDIOPATHIC PULMONARY FIBROSIS WITH ELEVATED B-
TYPE NATRIURETIC PEPTIDE
David M. Safley, MD*; Alan Forker, MD. University of Missouri – Kansas
City, Kansas City, MO
INTRODUCTION: B-type natriuretic peptide (BNP) levels are ele-
vated in congestive heart failure as well as other conditions that increase
wall tension in the cardiac ventricles. A level of 100 pg/ml has been
suggested as diagnostic of congestive heart failure (CHF).
CASE PRESENTATION: LB, a 55 year old African American woman,
was admitted from the emergency department with dyspnea and hypoxia.
Oxygen saturation was 78% on room air and increased to 92% on 4L/min
supplemental oxygen. Her past medical history is significant for emphy-
sema with continued tobacco abuse and coronary artery disease with
recent (2 months ago) 4 vessel bypass surgery. She was re-admitted 1
month later with congestive heart failure (CHF) and left ventricular
ejection fraction (LVEF) was documented to be 45% by transthoracic
echocardiogram (TTE). Physical exam revealed bilateral ronchi with no
S3, S4, or other signs of congestive heart failure. Chest x-ray revealed
diffuse pulmonary infiltrates, BNP was 2450 pg/ml. She was admitted to
a telemetry unit and aggressively diuresed with intravenous furosemide.
Over 5 days her weight dropped to 105.5 from 122.8 pounds at admission,
and serum creatinine increased from 2.1 to 2.5 mg/dl. Repeat BNP was
1830 pg/ml. Nesiritide infusion was begun and continued for 72 hours,
with continued diuresis of approximately 2 liters daily net negative fluid
balance. The patient remained hypoxic, requiring 50 percent oxygen by
face mask to maintain oxygen saturations greater than 90 percent. Repeat
TTE revealed an LVEF of 65%. A third BNP level was 674 pg/ml. Chest
Computed Tomography (CT) was obtained due to persistent pulmonary
infiltrates on chest radiographs. This revealed extensive honeycombing
with severe bullous disease and infiltration of the remaining lung paren-
chyma as well as a discrete mass in the left upper lobe. This result was not
felt to rule out concurrent CHF, so to definitively assess cardiac output,
volume status and left ventricular filling pressure, a right heart catheter-
ization was performed. Pulmonary artery pressure was 22/10 mmHg,
pulmonary capillary wedge pressure was 2 mmHg, and cardiac output was
4.5 liters per minute. Open lung biopsy was then performed, revealing
interstitial fibrosis, bronchial and squamous metaplasia, and extensive
honeycomb lung consistent with idiopathic pulmonary fibrosis. Oral
corticosteroid therapy was begun with poor results.
DISCUSSION: BNP is a very useful test that has been extensively
studied. It can aid in the correct diagnosis of CHF in those patients who
remain undiagnosed after clinical evaluation. Levels of BNP are elevated
in diastolic as well as systolic cardiac dysfunction. Pulmonary causes have
also been shown to be associated with elevated BNP, but to a lesser
degree than decompensated LV failure.
CONCLUSIONS: Laboratory tests serve to confirm clinical diagnoses
made by thoughtful history taking and careful physical examination, or to
help with difficult cases. Laboratory tests such as BNP may be misleading
if not taken in clinical context, delaying correct diagnosis and treatment.
There has been much enthusiasm regarding the diagnostic utilities of
BNP. This is very useful test, but should be used in a specific clinical
context to answer a specific question, not as a replacement for the history
and physical.
Monday, October 27, 2003
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260S CHEST 2003—Case Reports

REFERENCES:
Morrison LK. Utility of a rapid B-natriuretic peptide assay in
differentiating congestive heart failure from lung disease in patients
presenting with dyspnea. J Am Coll Cardiol 2002; 39(2): 202-9.
McCullough PA, et al. B-type natriuretic peptide and clinical
judgment in emergency diagnosis of heart failure: analysis from
Breathing Not Properly (BNP) Multinational Study. Circulation
2002; 106(4): 416-22.
DISCLOSURE: D.M. Safley, None.
HEPATOPULMONARY SYNDROME UNMASKED BY INTERSTI-
TIAL PNEUMONITIS
Karen J. Wesenberg, MD*; Dave Lewinsohn, MD, PhD. Oregon Health
Sciences University, Portland, OR
INTRODUCTION: We present a case of hepatopulmonary syndrome
which was unmasked by interstitial pneumonitis resulting from a drug
exposure.
CASE REPORT: MB is a 43 year old male with Childs Class A
cirrhosis secondary to hepatitis C who presented with increasing dyspnea
on exertion and dry cough over a one month period. He had previously
been well but was now winded with minimal exertion and reported his
fingertips turning blue with one flight of stairs. Six weeks prior he had
begun therapy with ribaviron and alpha interferon. Past medical history
was otherwise noncontributory.
Vitals: BP 130/65, R 18, HR 78, O2 saturations 90% on room air (RA)
at rest, 84% with ambulation. Exam notable for bibasilar inspiratory rales
and clubbing. Chest radiograph revealed interstitial opacities. High
resolution CT chest confirmed subpleural interstitial fibrosis with ground
glass attenuation predominantly in the bases. He was also noted to have
significant orthodeoxia, with RA O2 saturations of 78% while standing and
93% in the supine position. ABG’s revealed a PaO2 of 55 on RA and 266
on 100% oxygen (mmHg). Physiologic shunt estimated at 15-20%.
Delayed positive contrast echocardiogram confirmed intrapulmonary
shunt.Pulmonary angiogram showed no arteriovenous malformations.
Extrapulmonary (brain) uptake of radioisotope was demonstrated to be
8.5% on 99mTcMAA lung scan.
The patient was diagnosed with hepatopulmonary syndrome (HPS), as
well as an interstitial pneumonitis secondary to his Hep C medications.
These were discontinued, a trial of steroids was initiated and the patient
was listed for liver transplantation.
He reported significant symptomatic improvement within weeks. Fol-
low up CT chest showed improvement with resolution of ground glass
attenuation and some mild residual fibrosis. Repeat ABG two months
after presentation now showed a PaO2 of 81 on RA and 428 on 100%
oxygen (mmHg).
DISCUSSION: We present a case of HPS that was unmasked by a
concomitant interstitial pneumonitis. Alpha interferon and ribaviron have
been implicated as a cause of reversible interstitial pneumonitis.
1
HPS is
defined as the clinical triad of chronic liver disease (cirrhotic or non cirrhotic),
arterial hypoxemia (PaO2 ⬍ 70 mmHg or AaO2 gradient ⬎ 20 mmHg)) and
intrapulmonary vascular dilations.
2
Interestingly, the degree of hypoxemia
associated with HPS is not related to the severity of liver disease.
3
Orthotopic
liver transplantation may result in complete resolution of HPS, but carries a
higher surgical mortality.
4
The co-existence of intrinsic lung abnormalities in
addition to HPS has been reported in 20 % to 31% of patients.
3
Given the rapid progression of symptoms, we postulate that he had
clinically silent HPS and the additional insult to his gas exchange by the
interstitial process caused hypoxemic vasoconstriction. This effectively
shunted more blood through his pulmonary vascular dilatations. In
addition, by treating the pneumonitis we were able to demonstrate
improvement in the shunt, although it remains present.
CONCLUSION: Severity of HPS may be increased by concomitant
defects in gas exchange, as seen with interstitial pneumonitis. By treating the
pulmonary process, the degree of shunt from HPS may be lessened, although
not eliminated. Liver transplantation remains the best treatment for HPS.
REFERENCES:
1. Karim A, et al. Interstitial pneumonitis in a patient treated with
alpha-interferon and ribaviron for hepatitis C infection. Am J Med
Sciences 2001;322:233-235.
2. Krowka MJ. Hepatopulmonary syndrom: recent literature (1997-
1999) and implications for liver transplantation. Liver Transpl 2000;
6: S31-S35
3. Krowka MG,et al. Hepatopulmonary Syndrome: A prospective study
of Relationships between severity of liver disease, PaO2 response to
100% oxygen and brain uptake after 99mTcMAA lung scanning.
Chest 2000; 118:615-624
4. Arguedas MR, et al. Prospective evaluation of Outcomes and
predictors of mortality in patients with hepatopulmonary syndrome
undergoing liver transplantation. Hepatology 2003;37:192-197
DISCLOSURE: K.J. Wesenberg, None.
Airway
4:15 PM - 5:45 PM
VASCULAR RING AS THE CAUSE OF UPPER AIRWAY OB-
STRUCTION IN AN ADULT
Thomas P. Stern, MD*; E. Regis McFadden, MD. MetroHealth Medical
Center, Cleveland, OH
INTRODUCTION: Vascular rings are a rare cause of obstructive lung
disease. We report a case of a double aortic arch that was mistaken for
asthma and did not manifest itself until adulthood.
CASE PRESENTATION: A 35-year-old female with history of refrac-
tory asthma treated with systemic corticosteroids for a decade presented
with worsening dyspnea. She did not have childhood asthma but had
multiple hospitalizations for bronchitis and pneumonia. She was diag-
nosed with asthma in her early twenties with spirometry showing severe
obstruction with a bronchodilator response of 19% and 420cc in FEV1.
Her prolonged treatment with steroids led to significant weight gain, sleep
apnea, and pregnancy induced diabetes. She remained dyspnic despite
bonchodilators so a spiral CT was done to evaluate for pulmonary
embolus. The CT showed a double aortic arch resulting in compression of
the trachea and the esophagus. Subsequent spirometry showed blunting
of both the inspiratory and expiratory limb of the flow volume loop. The
patient was referred to cardiothoracic surgery for definitive treatment.
DISCUSSION: This case is unique for a vascular ring because of the
delayed onset of symptoms in adulthood. This case of a vascular ring in an
adult presented with wheezing rather than dysphagia. Delayed diagnosis
of the vascular ring caused significant morbidity in the form of recurrent
dyspnea and side effects from systemic corticosteroids.
CONCLUSIONS: Upper airway obstruction is one of the differential
diagnoses of patients presenting with obstructive airway disease. Vascular
rings frequently manifest during childhood although some people are
incidentally diagnosed on autopsy. The primary presenting symptom of
older children and adults with a vascular ring is dysphagia [1]. Barium has
been the primary method of diagnosis of vascular rings in the past, but
newer imaging modalities such as CT and MRI are also effective.
Definitive treatment is surgical repair.
REFERENCES:
1. Van Son J, Julsrud P, Hagler D, et al. Surgical Treatment of Vascular
Monday, October 27, 2003
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Rings: The Mayo Clinic Experience. Mayo Clin Proc 1993:68;
1056-1063
DISCLOSURE: T.P. Stern, None.
ENDOBRONCHIAL CHONDROMA: A RARE TUMOR CAUSING
COMPLETE LEFT MAINSTEM BRONCHIAL OCCLUSION
Prashant Grover, MBBS*; Richard Zuwallack, MD, FCCP. University of
Connecticut, Farmington, CT
INTRODUCTION: Benign tumors of the tracheobronchial tree are
rare and their incidence ranges from 1-5% of all tumors.These tumors are
usually peripheral and endobronchial involvement is seldom seen. We
describe a case of an endobronchial chondroma which was left untreated
for 4 years due to non-compliance and grew to cause complete left lung
collapse.
CASE PRESENTATION: A 54 year old male was evaluated in the
pulmonary clinic for a positive tuberculin test and persistent cough. Chest
radiograph showed left upper lobe collapse. This prompted a bronchoscopy
which revealed a tumor at the bifurcation of the left mainstem bronchus with
complete occlusion of left upper lobe orifice. Biopsy showed benign cartilag-
inous tissue consistent with a chondroma. The patient was lost to follow up for
4 years when he returned with increasing cough, dyspnea and fevers. Chest
xray and CT showed near total collapse of left lung with post obstructive
pneumonia. A bronchoscopy showed a large tumor protuding from the left
mainstem bronchus with 100% occlusion of the lumen. Specimens at
bronchoscopy were identical to the tissue obtained four years back. V/Q scan
did not show any ventilation or perfusion of the left lung. FEV1 was 1.92L
which was reduced from 2.4L at the time of the initial presentation.The
patient underwent a left pneumonectomy.
DISCUSSIONS: Chondromas are rare benign pulmonary tumors.
They are almost always found in the lung parenchyma and can be seen in
association with Carney’s complex(gastric stromal sarcoma, extra-adrenal
paraganglioma with pulmonary chondroma). Failure to follow up on part
of our patient led to more extensive surgery with greater loss of pulmonary
function. He was not a candidate for laser resection of the tumor as the
lung distal to the obstruction was destroyed.
CONCLUSION: Benign endobronchial tumors although rare should
be treated early aggressively with either laser resection or surgery. If left
alone they have the potential to destroy an entire lung.
REFERENCES:
1. Benign bronchopulmonary tumors: radiologic and pathologic find-
ings. Journal of Computer Assisted Tomography 2002;26(5):784-796
2. Benign tumors of the tracheobronchial tree:endoscopic character-
istics and role of laser resection.Chest 1995;107:1744-51
3. Discovery of the carney complex, a familial lentiginosis-multiple
endocrine neoplasia syndrome: a medical odyssey. The Endocrinol-
ogist 2003;13(1):23-30
DISCLOSURE: P. Grover, None.
PULMONARY CAPILLARY HEMANGIOMATOSIS
Nikhat Salamat, MD*; Victor J. Test, MD. Scott and White, Temple, TX
INTRODUCTION: Pulmonary capillary hemangiomatosis is a locally
aggressive benign vascular neoplasm of lung characterized by cytologically
benign thin walled capillary–sized blood vessels proliferating diffusely
through the pulmonary interstitium in and around airways and pulmonary
vessels.It is a rare disease characterized by pulmonary hypertension and a
slowly progressive clinical course.This case reports describe the clinical
presentation,diagnostic approach and problems encountered in this rare
but clinically important disease.
CASE REPORT: Patient is a 51 year old male who presented with
dyspnea,functional Class III of the NYHA.He was diagnosed with con-
gestive heart failure of unknown aetilogy seven to eight yrs ago and did
reasonably well until recently when his dyspnea worsened and he had two
episodes of pre syncope.He also carried the diagnosis of obstructive sleep
apnea,diabetes mellitus and hypertension.Physical examination revealed
room air saturation of 77% and increasing to 94% with 2 litres of
Monday, October 27, 2003
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262S CHEST 2003—Case Reports

oxygen.He had a loud P2,fixed split,right sided S3 and right ventricular
heave.As part of his work up he had an of ANA 1:160 in a speckled
pattern.Echo showed dilation of RA/RV,moderately-severe TR with ele-
vated RVSP and EF of 55 %.CXR showed prominent hilar region.High
resolution CT showed diffuse ground opacities.Spirometry showed mild
obstructive and restrictive pattern.Right Heart catheterization showed
right atrial pressure of 9,right ventricular pressure of 79/10, pulmonary
artery pressure of 80/28 with mean of 49,wedge pressure was 12.There
was no significant response to intravenous prostacyclin.Because of con-
cern for pulmonary veno-occlusive disease he was not started on flolan
and underwent a surgical biopsy.Biopsy showed hemosiderin laden alve-
olar macrophages,vascular changes consistent with pulmonary hyperten-
sion and foci resembling pulmonary capillary hemangiomatosis.
DISCUSSION: Pulmonary capillary hemangiomatosis is a rare disease
with proliferation of thin walled alveolar capillaries associated with
infiltration of lung parenchyma and invasion of blood vessels and bron-
chial tree. We are aware of only twenty nine cases that have been
reported.It usually presents as pulmonary hypertension and is confused
clinically with primary pulmonary hypertension and pulmonary veno-
occlusive disease.It usually presents between ages of 20-40 years with
dyspnea,hemoptysis and abnormal radiographic findings.The typical clin-
ical course is that of rapid deterioration. Early diagnosis and bilateral lung
transplantation is the only cure . One case of successful treatment has
been reported with interferon alpha-2a.
There have been case reports of PCH- like foci that were incidental
findings at autopsy in which there was no clinical evidence of pulmonary
hypertension.PCH-like foci are more likely to be seen in autopsies if
multiple lung section are reviewed.It is a clinically significant finding only
if the patient has signs and symptoms of pulmonary hypertension.How-
ever,the patient should be followed closely.
The case report in this article describes all the difficulties encounter in
diagnosis and management,late diagnosis,confusion clinically with primary
hypertension, veno-occlusive disease and no good, definite treatment so
far besides transplantation.Our patient was recently started on Bosentan
and we will follow him up clinically and with some objective measurement
to see whether his dyspnea and pulmonary hemodynamics has improved
or not.There has been no reported cases of therapy with this medication
to date that we are aware of.
CONCLUSION: The cause of PCH is unknown and it is a rare
disorder,most cases diagnosed postmortem since diagnosis is delayed.
Successful therapy to date has been described with interferon (1 case
report) and bilateral lung transplant that offers long term survival.Further
studies are needed to settle clear understanding of pathogenesis that will
lead to new therapeutic strategies.
DISCLOSURE: N. Salamat, None.
ESOPHAGEAL DUPLICATION CYST PRESENTING AS
CHRONIC COUGH
Lazaro O. Bravo, MD*; Jeffrey G. Walls, MD; Justin Q. Ly, MD;
Christopher J. Lisanti, MD; Stephen P. Roberts, MD. Wilford Hall
Medical Center, San Antonio, TX
INTRODUCTION: Cough is the most common complaint for
which patients seek medical attention. Cough can be classified as acute
(less than 3 weeks) or chronic (3 to 8 weeks or longer). The most
common causes of chronic cough in nonsmokers include asthma,
gastroesophageal reflux disease (GERD) and postnasal drip syndrome
(PNDS). Lesions that compress the upper airway remain a rare cause
of chronic cough.
CASE PRESENTATION: We report the case of a 50-year-old
housewife, who presented to the pulmonary clinic for evaluation of a
non-productive cough. The cough had been present for approximately
1 year. It was described as slow in onset, worse at night with frequent
awakenings and was accompanied with pleuritic chest pain. No
aggravating factors or relationship to food or body position. The cough
was refractory to prescription and over-the-counter cough suppres-
sants. The patient and her husband were both lifelong non-smokers
and denied any exposure to chemicals, irritants or pets. They also
denied any recent travel. Laboratory data, pulmonary function tests
(PFT) and chest radiographs were unremarkable. The patient had
already been adequately treated for allergic rhinitis and gastroesoph-
ageal reflux disease. Neither resolved her cough. Computed tomogra-
phy (CT) of the chest showed a 1.7 cm low-density mass posterior to
the carina. Bronchoscopy revealed an extrinsic compression without
obstruction proximal and posterior to the carina. Barium swallow
showed minimal extrinsic compression of the esophagus. Magnetic
resonance imaging (MRI) was compatible with an esophageal duplica-
tion cyst (EDC). The patient underwent open thoracotomy with
complete excision of the cyst. Pathology revealed a cyst that was
multilocular and lined with cuboidal to cilliated columnar epithelium.
It had a smooth muscle coat with no evidense of mucous glands,
cartilage or neoplasia. The findings were consistent with an EDC. The
patient’s cough subsequently resolved.
DISCUSSION: This case demonstrates a rare presentation of an EDC
as an etiology of chronic cough. EDCs are congenital anomalies of the
embryonic foregut commonly located in the posterior mediastinum. They
are common in children, may be single or multiple and present as
respiratory or gastrointestinal symptoms. One third of patients remain
asymptomatic throughout childhood. In adults, EDCs are usually found
incidentally. When present, dysphagia is the most common complaint.
Complications include infection, hemorrhage, obstruction and neoplastic
transformation. EDC’s can be detected by numerous modalities including
chest CT, esophageal ultrasound or MRI. Often these are incidentally
detected on chest radiographs or upper gastrointestinal examinations.
Needle aspiration to confirm the benign diagnosis may be performed in
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conjunction with endoscopy or with CT guidance. Pathology usually
reveals a variety of mucosa including squamous, columnar, cuboidal or
pseudostratified. The recommended treatment is complete excision either
with conventional or muscle-sparing posterolateral approach as well as
though newer modalities like video assisted thorascopic surgery (VATS).
Recurrences are rare.
CONCLUSION: Cough is the most common complaint for which
patients seek medical attention. Though rare, extrinsic compression of the
trachea can be a cause of chronic cough. EDC’s mostly present as
dysphagia in adults. Though benign, complete excision is recommended.
DISCLOSURE: L.O. Bravo, Department of Defense.
The views presented in this case do not represent that of the Depart-
ment of Defense
18 YEAR OLD MALE WITH COUGH, FEVER AND PLEURITIC
CHEST PAIN FOR TWO MONTHS
Jameel F. Durrani, MBBS*; Hormoz Ashtyani-Asl, MD, Director, Pul-
monary and Critical Care Medicine; Ciaran Mannion, MD, Assistant
Professor of Pathology. UMDNJ- New Jersey Medical School, Wallington,
NJ
INTRODUCTION: We present the case of a 18 year old male
presenting with cough, fever and left-sided pleuritic chest pain.
CASE PRESENTATION: An 18-year-old otherwise healthy 2 pack
year smoker male presented to Emergency Room with a two-month
history of cough with fever, chills and left-sided pleuritic pain on
coughing. Chest X-ray done as outpatient Showed Left upper lobe
infilterate. PPD was negative and a course of oral antibiotics had failed to
resolve symptoms
In ER, patient had a temperature of 103°F, blood pressure of 116/60,
pulse 120/min. and a respiratory rate of 25/min. and a room air saturation
of 90%. Systemic examination was significant for markedly diminished
breath sounds in the left upper lung fields along with dullness on
percussion in the same areas. Cardiac, neurologic, skin and examination of
the extremities were unremarkable.. Initial ER Chest Xray and CT scan
revealed a dense consolidation in the left upper lung with pleural effusion.
Thoracentesis was done which revealed an exudative pleural effusion
with a pH of 6.7, WBC count of 4700, RBC count 1.9 m/cu.mm, glucose
less than 20, LDH of 12285, protein of 4.9 gm/dl. A chest tube was placed
for drainage of empyema. Bronchoscopy revealed a polypoid 1x1 cm
endobronchial tumor in the left main stem bronchus. Multiple endobron-
chial biopsies were done and sent for histopathological examination. The
histopathological diagnosis of low-grade Mucoepidermoid carcinoma of
salivary gland origin was made.
DISCUSSION: Mucoepidermoid tumors (MET) are rare pulmonary
neoplasms. Miller et al
1
described their incidence as 0.15% of primary
lung cancers. METs were first described in the salivary glands where they
were presumed to arise from the excretory ducts of the salivary glands.
Occurrence of these tumors in the lungs was first described by Smetana
et al
2
Table-1: Criteria for differentiating between high and low grade
mucoepidermoid tumors
Feature LowGradeMET HighGradeMET
Age at
presentation
Ave.age 34.8 years
(usu. ⬍30 Yr. Old.)
Ave. age 44.5 years
(Usu. ⬎30 yr.)
Symptoms Same in both groups including fever, SOB, cough,
hemoptysis and wheezing. Same in both groups
including fever, SOB, cough, hemoptysis and
wheezing.
Location No particular lung lobar or segmental predilection.
Histopathology Combination of
epidermal and mucus
producing cells. Equal
cystic and solid
components, no
pulmonary parenchymal
invasion and absence of
necrosis and mitotic
activity
More areas of solid than
cystic growth, with
invasion of surrounding
pulmonary parenchyma.
Dominant component of
squamoid and
transitional cells.
Presence of mitotic
activity and necrosis.
Staining Both types are positive for keratin, CAM 5.2 and
negative for S-100 protein, actin and chromogranin .
Metastasis Uncommon Common
Treatment Surgery Surgery ⫹/- Chemo and
radiation Rx.
Figure 1: Outpatient chest X-ray.
Figure 2: Chest X-ray and CT scan done in ER, showing dense
consolidation with effusion in left lung.
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264S CHEST 2003—Case Reports

The clinical presentation of MET is not specific and it can present with
symptoms like recurrent infection, new onset of shortness of breath and
wheezing and may be initially misdiagnosed as a case of adult onset
asthma.
The tumors characteristically show a solid growth pattern composed of
epidermoid cells distributed in sheets that obliterate the normal lung
parenchyma. Admixed with this epidermoid cell proliferation are areas
showing cystic structures lined by mucin producing epithelium and often
containing mucinous material in the lumen.
Figure 3: The low-grade mucoepidermoid tumor consists of cytologi-
cally bland tubules, devoid of mitosis, nuclear pleomorphism or necrosis.
H&E Stain. Magnification x 200
Figure 4: Intracytoplasmic mucin is evident in tumor cells on Muci-
carmine stain. Magnification x 400
TREATMENT and PROGNOSIS: Surgery alone is sufficient for
resectable low-grade tumors, whereas adjuvant radiation or chemotherapy
or both are recommended for resectable high grade METs. In difficult
cases pneumonectomy may be the only alternative. High grade METs
should be treated as well differentiated non-small cell or squamous cell
carcinoma, by lobectomy with nodal sampling if surgical resection is not
possible or incomplete.Overall 5-year survival rate for the primary
Mucoepidermoid tumors of the lung is 45%
1
. High grade Mucoepider-
moid tumors has a 100% mortality with a median survival of 5 months.
CONCLUSION: Because of non specific symptoms, diagnosis of
METs is often delayed. High index of suspicion is required for diagnosis
of Mucopepidermoid tumor
REFERENCES:
1: Miller et al. Rare pulmonary neoplasms. Mayo Clinic Proc. 1993;
68:492-498.
2: Smetana et al. Bronchogenic carcinoma, an analysis of 100 autopsy
cases. Milit Surg. 1952; 111:335-351.
3: Pearson FG et al. Experience with primary neoplasms of the trachea
and carina. J thorac cardiovasc Surg 1984; 88:511-516.
DISCLOSURE: J.F. Durrani, None.
BARRETT’S ESOPHAGITIS-RELATED BRONCHOESOPHA-
GEAL FISTULA. THE DIAGNOSTIC VALUE OF PERSISTENT
AIR LEAK IN THE VENTILATED SETTING
Raghan Abouasaleh, MD; Osama Halaweh, MD; Bohdan Pichurko, MD*.
Providence, Southfield, MI
The patient is a 44 year old white female with a history of mental
impairment who presented with shortness of breath and associated right
lower lobe pneumonia noted on the chest roentogram. Parenteral antibi-
otics were instituted. The patient became septic with significant respira-
tory insufficiency. She was therefore placed on mechanical ventilation
through an oral endotracheal tube.
During ventilation there was a constant leak of 150 cc of the tidal
volume with each breath. The endotracheal tube was replaced without
improvement in the consistent loss of the tidal volume. For this reason,
emergency bronchoscopy was performed and identifieda1cmleft sided
bronchoesophageal fistula close to the carina.
Esaphagogastroduodenoscopy confirmed this finding at 30 cm from the
incisor teeth. Biopsies from the esophageal and bronchial sides of the
fistula showed Barrett’s mucosa. No neoplasia was present. Computed
tomography of the chest did not show any other mediastinal abnormalities.
Primary surgical repair was undertaken with longitudinal suturing of the
individual bronchial and esophageal components of the fistula. A small
post-operative leak was identified on the barium esophagogram that was
successfully treated with conservative management consisting of jejunal
feeding tube and proton pump inhibitor.
Barrett’s esophagus is an intestinal metaplastic response of the esoph-
ageal squamous mucosa to chronic gastroesophageal reflux. It can present
initially as tongue-like extensions or scattered islands of columnar epithe-
lium, circumferential involvement of the distal esophagus is seen in
advanced cases. Ulceration is noted in 10% of cases with Barrett’s
esophagitis, and deep wide-mouthed ulcer can penetrate or perforate to
adjacent mediastinal structures. The majority of patients with bronchoe-
sopahgeal fistula present with dysphagia, cough and choking on swallow-
ing, and less commonly wheezing, dyspnea, weight loss, hemoptysis and
epigastric or chest pain. Surgical therapy of benign fistula offers an
excellent chance of cure.
Further, the present report underscores the diagnostic importance of
pursuing associated clinical findings including unexplained loss of tidal
volume in the intubated patient.
DISCLOSURE: B. Pichurko, None.
Cardiovascular
4:15 PM - 5:45 PM
ADRENERGIC SHOCK – AN OVERLOOKED CLINICAL EN-
TITY?
Branislav Schifferdecker, MD*; Dhatri Kodali, M.D.; David H. Spodick,
M.D., D.Sc.; Jayashri Aragam, M.D.. Saint Vincent Hospital at Worcester
Medical Center, Worcester, MA
INTRODUCTION: The cardinal sign of pheochromocytoma - hyper-
tension - may not be present in up to 30% of patients despite high
circulating levels of catecholamines. We report a case of recurrent
hypotension and shock induced by pheochromocytoma.
CASE REPORT: A 38-year-old, previously healthy woman presented
with severe headache and chest discomfort. The physical examination
revealed a drowsy, ill appearing woman. Body temperature of 100.1 °F,
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blood pressure of 100/60 mm Hg, heart rate of 110 beats per minute, mild
neck stiffness, fourth heart sound and rales at lung bases were noted.
Laboratory values included creatine kinase, 563 mg/dL; Troponin I 21
ng/mL and leukocyte count, 36,700 cells/␮L. After treatment of nausea
with metoclopramide patient developed chest pain, hypotension (72/40
mm Hg) and EKG changes (diffuse PR segment depressions and ST
elevations in leads V
2-3
). Echocardiogram showed left ventricular ejection
fraction of 30% with circumferential, basal hypokinesis and preserved
contractility of the apex. Heart catheterization revealed normal coronary
arteries, cardiac index 3.9 L/min/m
2
and systemic arteriolar resistance 660
dynes/sec/cm
5
. The diagnosis of fulminant myopericarditis in the setting
of sepsis was made. Patient responded to the therapy with volume
resuscitation, dopamine and norepinephrine. Extensive testing to identify
infectious pathogen was unrevealing. Patient was discharged in five days
on ␤-blocker and ACE-inhibitor therapy. Follow-up echocardiogram in
four weeks was normal.
Eight months later patient presented with similar symptoms. However,
this time shock was interrupted with several brief episodes of hyperten-
sion despite stable dose of vasopressors. Patient complained of palpitation,
chest discomfort, muscle cramps and generalized weakness. Low grade
fever, leukocytosis, sinus tachycardia, troponin I of 7.9 ng/mL and
echocardiographic findings identical with those at the previous admission
were noted. MRI of the abdomen revealed 36-mm right adrenal mass.
Serum testing confirmed diagnosis of pheochromocytoma (plasma nor-
epinephrine 6766, epinephrine 2296, dopamine 741, and urine metaneph-
rines 8338 nmol/L). She underwent successful surgical resection of the
tumor. Area of central necrosis was noted. Follow-up echocardiogram
showed normal left ventricular function. She has been asymptomatic for 8
months on no medical therapy.
DISCUSSION: Intravascular hypovolemia and decreased myocardial
contractility seem to be the key features of shock in patients with
pheochromocytoma. Adrenergic diuresis, increased capillary permeability,
peripheral ␤-adrenergic stimulation and attenuated vasoconstrictive re-
sponse due to the down-regulation of ␣1 adrenergic receptors result in
hypovolemia. Myocarditis is a well-documented complication of any
hyperadrenergic state. Dilated, apical hypertrophic, obstructive hypertro-
phic and non-obstructive hypertrophic cardiomyopathies have been de-
scribed in patients with pheochromocytoma. This is a first report of a
recently recognized, hyperadrenergic state-associated, apical contractility
preservation in patient with pheochromocytoma. Imaging with CT, MR or
bedside US allows early identification of adrenal mass in patients with
pheochromocytoma-induced shock. Serologic confirmation of pheochro-
mocytoma is necessary since clinically silent adrenal incidentalomas are
common. Emergent removal of pheochromocytoma in the setting of
adrenal shock may be lifesaving.
CONCLUSIONS: Pheochromocytoma is a great imitator. In patients
with hypotension diagnosis of pheochromocytoma should be considered if
they have:
1. Other symptoms suggesting pheochromocytoma
2. Shock of unclear etiology despite thorough evaluation
3. Rapid non-iatrogenic fluctuation of arterial blood pressure
4. Myocardial infarction without obstructive coronary stenosis
5. Cardiomyopathy, especially with regional wall motion abnormalities
that do not match typical coronary artery distributions.
6. Relatively mild abdominal trauma causing severe hemodynamic
instability
7. History of neurofibromatosis, tuberous sclerosis, von Hippel-Lindau
syndrome or MEN 2 syndrome
8. Family history of pheochromocytoma or MEN 2 syndrome.
DISCLOSURE: B. Schifferdecker, None.
SUDDEN CARDIAC DEATH FOLLOWING NON-ST SEGMENT
ELEVATION MYOCARDIAL INFARCTION; A RARE CASE OF
ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA
Lee M. Arcement, MD*; Kathy Hebert, MD; Sahil Bakshi, DO; Roy
Culotto, MD; Prakash Krishnan, MD; Leo Seoane, MD; James McKinnie,
MD; Hector O. Ventura, MD. Ochsner Clinic Foundation, Metairie, LA
INTRODUCTION: Sudden cardiac death (SCD) is a well known
complication of non ST segment elevation myocardial infarction
(NSTEMI). Arrhythmogenic right ventricular dysplasia (ARVD) is an
inherited cardiomyopathy which is frequently associated with ventric-
ular arrhythmias incluidng SCD. We present a case of ARVD present-
ing as SCD in the setting of non ST elevation myocardial infarction.
CASE PRESENTATIONS: A 51 year-old African-American man with
new complaints of chest pain of several hours duration called 911 after an
episode of near-syncope . He had a past medical history of hypertension
and gastritis as well as a family history including myocardial infarction. He
was on nifedipine XR and cimetidine. He had no known drug allergies. He
had a forty pack-year history of tobacco use. En route to the ER, he had
an episode of pulseless ventricular tachycardia (VT) in the ambulance and
was successfully cardioverted with 360 Joules.. In the ER, his vital signs
were BP 150/95, HR 59, RR 18, T 37.3. Physical exam was normal. His
electrocardiogram (EKG) showed sinus bradycardia with voltage criteria
for left ventricular hypertrophy (LVH) and T wave inversion in leads
V1-V3. Routine labs were within normal limits. He was admitted to the
ICU and placed on aspirin, nitropaste, intravenous heparin, beta-blockers
and intravenous lidocaine. Serial cardiac enzymes were consistent with a
NSTEMI. 2D echocardiography revealed four-chamber enlargement with
a left ventricular ejection fraction of 50% without regional wall motion
abnormalities. A signal average EKG was performed and was negative. A
cardiac catheterization (LHC) revealed a 70% distal left anterior descend-
ing artery stenosis with mild LV dysfunction. Medical treatment was
continued including aspirin, beta-blockers and an ACE inhibitor. Over-
night in the ICU, the patient had an episode of non-sustained monomor-
phic VT with documented left bundle branch morphology. An electro-
physiologic study (EPS) was obtained and revealed a right ventricular
outflow tract (RVOT) VT, an uncommon and ablatable cause of VT. Other
focuses of VT were not ellicited with programed stimulation. The patient
subsequently underwent successful radio-frequency ablation of his RVOT
VT but had an episode of sustained monomorphic VT post-procedure
treated with cardioversion. A 2D echocardiogram was repeated to evalu-
ate for right ventricular dysplasia. This was inconclusive. The patient was
sent for a cardiac MRI which revealed right ventricular free wall and
septal fat deposits consistent with right ventricular dysplasia. An automatic
implanted cardioverter defibrillator (AICD) was then placed and the
patient was later discharged home on aspirin, metoprolol, benazepril, and
simvastatin. He was symptom free at his one year visit.
DISCUSSION: Arrhythmogenic right ventricular dysplasia is an inher-
ited cardiomyopathy with a genetic abnormality on chromosome 14.
There is fatty infiltration of the right ventricular free and/or septal walls.
There is associated ventricular tachycardia and SCD. ARVD presents
more frequently in children and young adults. EKG findings include
ventricular tachycardia with left bundle branch morphology, right axis
deviation and T wave inversion over the precordial leads. Epsilon waves
can also be seen in the right precordial leads. Most patients show an
abnormal right ventricle by echocardiography, computed tomography,
magnetic resonance imaging or right ventricular angiography. MRI is the
non-invasive test of choice. Endomyocardial biopsy is definitive but
usually not performed. There is no definitive medical treatment of the
associated arrhythmias. AICD’s are now indicated after an episode of
SCD or VT associated with ARVD. Cardiac transplantation is the only
definitive treatment currently.
CONCLUSION: The case above displays the diagnostic challenges of
SCD. Despite potential multiple mechanisms of VT including ischemia
and RVOT VT, a third mechanism was ultimately uncovered. A thorough
search for all possible mechanisms of VT including ARVD must always be
considered and sought.
DISCLOSURE: L.M. Arcement, None.
VALVE-SPARING AORTIC ROOT REPLACEMENT FOR
MARFAN SYNDROME
David G. Affleck, MD*; Cindy Camillo, RN; Marc R. Moon, MD.
Barnes-Jewish Hospital in St. Louis, Saint Louis, MO
INTRODUCTION: There is considerable controversy surrounding
the appropriate survellience, timing and method to repair an aortic root
aneurysm in the Marfan syndrome. The purpose of this case report is to
describe our experience with the valve-sparing aortic root replacement.
This approach allows replacement of the degenerative Marfan aortic root
while sparing the native valve apparatus.
CASE PRESENTATION: Twenty-five year old male with Marfan
syndrome presented with severe chest pain. Computed tomographic scan
demonstrated a 5.5 cm aortic root aneurysm. Repair was accomplished by
resuspending the native aortic commissures inside a 32 mm hemashield
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266S CHEST 2003—Case Reports

tube graft, realigning the valve leaflets. The coronary arteries were
reimpplanted into the left and right neo-coronary ostia. Cross-clamp time
was 149 minutes and no blood transfusions were required. The post-
opperative course was uneventful. The patient was extubated 3 hours after
surgery and discharged to home on the 4th post-operative day. Echocar-
diography demonstrated normal valve leaflet function and no evidence of
aortic insufficiency.
DISCUSSION: Annuloaortic ectasia is common in patients with
connective tissue disorders. Pathologic weakening of the aortic wall can be
detected in patients with marfan syndrome, Ehlers-Danlos, osteogenesis
imperfecta and pseudoxanthoma elasticum.
Evolution in the repair of the degenerative aortic root has been
significant. Historically, the majority of repairs required complete excision
of the valvular apparatus, aortic root, and ascending aorta with placement
of a prosthetic “composite” valved-aortic graft.
CONCLUSIONS: While several options exist for repair of the Marfan
aortic root, remodeling of the native valvular apparatus using this
resuspension technique is an excellent alternative. Although the long-term
results are unknown, this repair reestablishes native aortic valvular
competency thereby avoiding the need for long-standing anticoagulation.
DISCLOSURE: D.G. Affleck, None.
CARDIOMYOPATHY AND CHRONIC COUGH: CARDIAC SAR-
COIDOSIS
Ives R. de Chazal, MD, Pulmonary/Critical Care Mayo Clinic*; Kimberly
Russell, MD, Pulmonary/Critical Care Mayo Clinic; Kaiser Lim, MD,
Pulmonary/Critical Care, Mayo Clinic. Mayo Clinic, Rochester, MN
INTRODUCTION: Cardiomyopathy (CMP) from sarcoidosis occurs
in 2-7% of patients suffering from sarcoidosis, they have a five-year
mortality of 50-60%, and usually is from congestive heart failure (CHF)
and sudden death. We present a case of sarcoid CMP. A trial of
corticosteroids (CS) dramatically improved symptoms and cardiac func-
tion.
CASE REPORT: 55 y/o black male with steroid related hyperglycemia
and hypertension presented with a 3-year history of chronic cough and a
2-week history of worsening dyspnea. Dyspnea was initially with exertion,
later at rest and during sleep (PND). He was diagnosed with CHF and
bifascicular block on admission (see figure 1). Recent evaluation for
chronic cough revealed mild restrictive lung disease (TLC of 62%
predicted & DLCO of 38%). Thoracic CT scan (see figure 2) showed
ground glass opacities in the upper lobes with bronchovascular beading.
Nonischemic CMP was confirmed by cardiac catheterization with a left
ventricular ejection fraction of 25%. Significant laboratory findings
included an elevated ACE of 57 U/L (7-46) and CRP of 2.59 mg/dl
(0.02-0.8).
An endomyocardial biopsy performed was non-diagnostic (see figure 3).
Bronchoscopic transbronchial biopsies revealed non-necrotizing granulo-
matous inflammation raising the possibility of cardiac sarcoidosis (se
figure 4). The patient was started on prednisone, an ACE inhibitor and a
beta-blocker. There was a dramatic improvement in LV function and in
symptoms. Repeat CT scan revealed resolution of the opacities of the
upper lobes and the interstitial infiltrates (see figure 5). Prednisone was
discontinued after three months. Five months later, he had a normal left
ventricular function of 50-60% on echocardiogram and a normal spirom-
etry with a DLCO that improved to 68%.
DISCUSSION: Cardiomyopathy from sarcoidosis is rare. Our patient
presented with CHF with an EF of 25%, bifascicular block, with
non-obstructive coronaries on angiogram. Because of preceding history of
chronic cough, it prompted further diagnostic evaluation. Corticosteroid is
an effective treatment for sarcoid cardiomyopathy. Among steroid treated
patients the 10-year survival rate is 90% if the EF is ⬎50% versus 30% if
the EF is ⬍ 50%.
CONCLUSION: We present a case of cardiomyopathy with a preced-
ing history of chronic cough. A clinical diagnosis of sarcoid cardiomyop-
athy was made based on transbronchial lung biopsy. The patient re-
sponded very well to a short course of steroid.
DISCLOSURE: I.R. de Chazal, None.
Figure 1 ECG
Figure 2 CT on admission
Figure 3 Biopsy of the heart
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LEFT VENTRICULAR PSEUDOANEURYSM
Emad H. Ibrahim, MD*; Khaled Moghazy, MD. Alexandria Faculty of
Medcicine, Alexandria, Egypt
INTRODUCTION: Left ventricular aneurysms are a frequent sequela
of transmural myocardial infarction. The vast majority is true aneurysms
and false are rare (1-2).
CASE PRESENTATIONS: A 53-year-old women presented by recur-
rent left-sided, sharp chest pain for two months. Her medical history and
examination were insignificant. Chest X-ray was unremarkable.
Computed tomography (CT) showed a 3.5-cm diameter pseudoaneu-
rysm emanating from the lateral aspect of the left ventricle.
Magnetic resonance imaging (MRI) defined a 5x3-cm oval pseudoan-
eurysm extending from the anterolateral wall of the left ventricle. A jet
phenomenon is noted at the site of communication between the cavity of
the left ventricle.Cardiac Cineangiography confirmed the presence of a
pseudoaneurysmal cavity connected to the left ventricle.
DISCUSSION: A pseudoaneurysm is the consequence of rupture of
the cardiac wall within extra-cardiac hematoma; its lumen communicates
with the ventricle. In CT, the presence of a typical neck is not always
diagnostic of a pseudoaneurysm, and sometimes the neck is not clearly
demarcated. A keystone in diagnostic imaging with angiography, echocar-
diography, and radionuclide studies is the visualization of a “typical
neck”(1-2).
CONCLUSIONS: The use of combined diagnostic radiology tech-
niques (CT and MRI) and Cardiac cineangiography help in the diagnosis
of ventricular pseudoaneurysm.
REFERENCES:
1. Duvernoy O,et al. Pre- and postoperative CT and MR in pseudoa-
neurysms of the heart. J Comput Assist Tomogr 1992;16:401-9
2. Higgins CB,et al. False aneurysms of the left ventricle. Identifica-
tion of distinctive clinical, radiographic, and angiographic features.
Radiology 1978; 127:21-7.
Figure 4 Biopsy of the lung
Figure 5 CT of follow-up 5 months later
Figure [1]: Chest X-ray PA view.
Figure [2]: Axial CT scan shows a pseudoaneurysm (A) emanating from
the lateral aspect of the left ventricle (LV).
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APICAL HYPERTROPHIC CARDIOMYOPATHY WITH CHEST
PAIN
David M. Safley, MD*; Alan Forker, MD; J A. Grantham, MD. University
of Missouri - Kansas City, Kansas City, MO
INTRODUCTION: Chest pain or suspected myocardial infarction
accounts for up to 20% of emergency department visits. Evaluation for
coronary artery disease may delay the diagnosis of non-cardiac chest
pain.
CASE PRESENTATION: A 69 year old woman presented to the
emergency department with intermittent left sided chest pain for 1
week. It felt like a burning or tightness, without radiation but with
associated lightheadedness. She also had nausea with 1 episode of
emesis. Medical history was significant for hypertension, dyslipidemia,
peripheral vascular disease with iliac angioplasty in 1998, hypothyroid-
ism, polymyositis in remission, bilateral shoulder avascular necrosis
associated with steroid use, right shoulder arthroplasty, peptic ulcer
disease and ischemic colitis. She has a 20-30 pack/year smoking history.
Daily medications include amlodipine 5 mg, atorvastatin 10 mg,
levothyroxine 125 mcg, aspirin 325 mg. Review of systems elicited mild
dyspnea on exertion but no lower extremity edema or orthopnea.
Cardiovascular and pulmonary examination was normal. The pain
resolved with sublingual nitroglycerin and intravenous heparin. Diag-
nostic coronary angiogram performed the next morning revelaed no
occlusive coronary artery disease, but left ventriculogram revealed a
spade-shaped left ventricular cavity. This was confirmed with trans-
thoracic echocardiogram, left ventricular ejection fraction was 80%.
There was no significant outflow obstruction at rest. With valsalva
maneuver, an intracardiac gradient of 45 millimeters of mercury was
evident. BB was discharged home with a presumptive diagnosis of
subendocardial ischemia. Ten days later she returned to the emergency
department with chest pain which improved with fasting and a low-fat
diet. Abdominal computed tomography revealed free air anterior to the
liver capsule, so emergent exploratory laparotomy was performed. Pus
and fluid was present in the abdominal cavity, and a 1.5 x 1.5 cm
perforated duodenal ulcer was repaired. She recovered well and was
discharged home in stable condition.
DISCUSSION: Apical hypertrophic cardiomyopathy, also known as
Yamaguchi disease, was first described in the late 1970’s in Japan, where
it may account for up to 25% of hypertrophic cardiomyopathy. Elsewhere
in the world, it is less that 1%. This disease is usually diagnosed in the 5
th
decade of life, with males greatly outnumbering female cases. Electrocar-
diogram may reveal left ventricular hypertrophy with deeply inverted T
waves, known as “giant negative T waves” in leads V4-V6. There is a good
prognosis, with up to 95% 15-year survival.
CONCLUSIONS: Morphologic heart disease may be identified
during cardiac evaluation for other reasons. In this case it led to a
premature cessation of diagnostic testing for the etiology of chest pain.
Emergency surgery could have been avoided if a more complete
evaluation was undertaken at the initial presentation. Diagnostic
testing should be expanded to include common non-cardiac etiologies
of chest pain.
REFERENCES:
Karlson BW, et al: Patients admitted to the emergency room with
symptoms indicative of acute myocardial infarction. J Intern Med
1991;230:251-58.
Maron BJ. Apical hypertrophic cardiomyopathy: the continuing saga.
J Am Coll Cardiol. 1990 Jan;15(1):91-3.
DISCLOSURE: D.M. Safley, None.
Infectious Diseases I
4:15 PM - 5:45 PM
SINOATRIAL BLOCK COMPLICATING LEGIONNAIRE’S DIS-
EASE
Boris I. Medarov, MD*; Shraddha Tongia, MD; Leonard J. Rossoff, MD.
Long Island Jewish Medical Center, New Hyde Park, NY
INTRODUCTION: Legionellosis is a versatile disease often present-
ing diagnostic challenges. Rhythm and conduction abnormalities are rare
complications.
Figure [3]: Postcontrast axial CT scan shows the pseudoaneurys-
mal cavity (arrow).
Figure [4]: (A, B, C) Cine gradient echo coronal MRI (A) & (B)
ECG-gated horizontal long-axis and short axis (C) spin-echo MR
images show a narrow ostium (arrow) communicating the pseu-
doaneurysm (A) and the left ventricular (LV).
Figure [5]: Cardiac cineangiography shows a pseudoaneurysmal
cavity connected with the left ventricle (arrow).
DISCLOSURE: E.H. Ibrahim, None.
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CASE PRESENTATION: A 59 year-old woman noted the acute
onset of chills, nausea, chest pressure and dizziness. She was hypoten-
sive (82/42 mmHg), tachypneic with heart rate of only 72/minute.Oxy-
gen saturation was 92% on room air. Her chest was clear and there was
no calf tenderness or swelling. The chest radiograph and CT were
unremarkable.
Broad-spectrum antibiotic therapy including azithromycin was initiated.
Electrocardiogram showed junctional rhythm at rate of 72/min (Fig.1).
White blood cell count was 7.7x10
9
/L, hemoglobin 12.2 g/dL, serum
electrolytes were normal. Serial creatine kinase and troponin levels
were within normal limits. Pulmonary embolism was ruled out. An
echocardiogram demonstrated normal left and right systolic ventricular
function. Blood and urine cultures showed no growth. A pharmaceu-
tical stress test showed no ischemia. The urine tested positive for
Legionella pneumophila antigen serotype 1. All antibiotics but azithro-
mycin were discontinued. The patient reverted to sinus rhythm after
48 hours (Fig. 2).
She felt improved, didn’t require supplemental O
2
anymore. One week
after admission the patient was discharged home asymptomatic and in
normal sinus rhythm.
DISCUSSION: Although numerous infectious processes such as
acute rheumatic fever have been associated with conduction abnor-
malities including prolongation of the PR interval and various degrees
of atrioventricular block, only one previous case of isolated sinoatrial
node dysfunction has been reported with Legionella infection.[1]
Rhythm and conduction disturbances due to Legionella appear to be
more common in children and, in some cases, those are associated with
clinical and biochemical evidence of myocarditis. In other cases, as in
ours, the intrinsic pacing system seems to be selectively affected.[1]
CONCLUSIONS: Awareness of the potential electrophysiological
complications of Legionellosis allows establishing an early diagnosis,
initiating the appropriate treatment in a timely manner, preventing this
way further morbidity.
REFERENCES:
1. Karim A, Ahmed S, Rossoff LJ. Legionnaire’s disease associated
with acute encephalitis and arrhythmia. Crit Care Med. 2002 May;
30(5): 1028-9
DISCLOSURE: B.I. Medarov, None.
SARCINA VENTRICULI EMPYEMA
Evan R. Restelli, DO*; Peter Kaplan, MD, FCCP; Kevin Perez, MD;
Robert Keenan, MD. Allegheny General Hospital, Pittsburgh, PA
INTRODUCTION: Empyema, the development of infection within
the pleural space, is a well-known complication of pneumonia. Here, we
present a unique case of empyema due to a gram-positive cocci, Sarcina
ventriculi, occurring after thoracotomy, and its successful treatment.
CASE PRESENTATION: A 63 year-old, obese man with Type II
diabetes presented with a two-week history of fevers and right-sided chest
pain. Four months previously, the patient underwent video-assisted
thoracoscopy (VATS) for the evaluation of recurrent pleural effusion of
unknown etiology. Pleural biopsy revealed fibrinous pleuritis, and cultures
were sterile.
Physical exam revealed decreased breath sounds at the right base with
a tender, indurated, erythematous area over the right lateral chest wall. A
pleural effusion was noted on chest radiograph. White blood count count
was 26,000. CT scan of the chest demonstrated a large, loculated,
gas-filled, right basal pleural collection, along with a pulmonary infiltrate.
Empyema due to staphylocci, Streptococcus or gram negative organisms
was our initial concern.
A repeat VATS was performed. 500 cc of puss was found in the
pleural space which extended into the subcutaneous tissue tracking
down the right flank. Gram stain of the puss revealed numerous
gram-positive cocci and culture grew Sarcina ventriculi. Following
identification of the organism, antibiotic therapy was changed to
penicillin and clindamycin. The patient had an uneventful recovery and
is well eight months later.
DISCUSSION: Sarcina ventriculi is an anaerobic gram-positive cocci,
which is part of the normal flora found on the skin of diabetics. While
other causes of empyema were considered, this organism was cultured
from the pleural space. We speculate that the possible source of infection
was the prior surgical intervention with the organism being introduced
from the skin to the pleura. Beta-lactam antibiotics and surgical interven-
tion appear to be best approach for treating empyema caused by this
organism.
CONCLUSION: This is the first reported case of empyema secondary
to Sarcina ventriculi. Consideration of this organism as a cause for
empyema in the appropriate setting can lead to successful diagnosis and
treatment.
REFERENCES:
Glass, M.: Sarcina species on the skin of the human foreare. Trans St
Johns Hosp Dermatol Soc 1973; 59(1): 56-60
Somerville, DA, Lancaster-Smith, M.: The aerobic cutaneous
microflora of diabetic subjects. Br J Dermatol 1973; Oct; 89(4): 395-
400
Finegold, S. M.: Anaerobic Bacteria in Human Disease. New York,
Academic Press, 1977
Sutter, V. L., Finegold, S. M.: Susceptibility of anerobic bacteria to
23 antimicrobial agents. Antimicrob Agents Chemother 1976; 10:736
DISCLOSURE: E.R. Restelli, None.
ACTINOMYCES IN A BRONCHOLITH; CHICKEN V. EGG
Ziad C. Boujaoude, MD*; Rania N. Aboujaoude, MD; Melvin R. Pratter,
MD; Thaddeus Bartter, MD. Robert Wood Johnson School of Medicine
at Camden, Camden, NJ
INTRODUCTION: Endobronchial actinomycosis, a rare form of an
uncommon disease, may represent a primary infectious process or a
secondary invasion of tissues devitalized by other processes. We are
reporting a case of actinomycosis in a broncholith.
CASE PRESENTATION: A 77-year-old woman was admitted to the
hospital for fever, cough, and a pre-syncopal episode. The cough had
started a week earlier. It had been dry initially and subsequently became
productive of yellow sputum. A pre-syncopal episode while walking in the
mall prompted admission to the hospital.
At time of admission, the patient denied dyspnea, hemoptysis, weight
loss, and other respiratory or systemic symptoms. Her medical history was
significant for multiple syncopal episodes in the preceding 2 years labeled
as ‘neurocardiogenic syncope.’ She had been hospitalized 2 years prior for
pneumonia. She was a non-smoker with no history of travel outside of
New Jersey. There were no occupational exposures or pets at home.
Medications were fosamax and atenolol.
Monday, October 27, 2003
Infectious Diseases I, continued
270S CHEST 2003—Case Reports

On physical examination, the temperature was 102
o
. Dentition was
poor. Chest examination revealed decreased breath sounds and dullness
over left posterior lower lung. There were no wheezes or crackles.
Computed tomographic (CT) scan of chest done two years earlier and
at time of admission are shown in Graphic 1. Hilar calcification and left
lower lobe atelectasis are present on both scans. Graphic 2 demonstrates
the bronchoscopic finding, a white mass completely obstructing the left
lower lobe bronchus. Biopsy was performed – the mass was extremely
hard. Microscopic examination of biopsy material revealed sulfur granules
composed of colonies of filamentous/branching gram-positive bacteria
which were negative on acid-fast staining, the typical appearance of
actinomyces.
DISCUSSION: Broncholithiasis represents a calcified hilar lymph
node eroding into a bronchus. Patients may present with hemoptysis,
localized wheezing, and post-obstructive pneumonia. At times, “stones”
may be coughed up. This patient probably obstructed slowy, leading to
atelectasis of distal lung. The leading cause of broncholithiasis is histoplas-
mosis in the United States and tuberculosis in other parts of the world.(1)
Actinomycosis may be a primary etiology in rare cases, but most com-
monly actinomyces, if present, tends to colonize broncholiths caused by
either tuberculosis (2) or histoplasmosis.(3-4)
Actinomycetes are anaerobic, gram-positive, filamentous, branching
bacilli that normally colonize the mouth, colon, and vagina. Actinomycosis
is an indolent, slowly progressive infection. It was common in the
pre-antibiotic era; the incidence decreased with the introduction of
enicillin. Cervico-facial actinomycosis accounts for 60% of cases, while
thoracic forms accounts for 15% of cases, with rare cases of endobronchial
disease.
CONCLUSION: Broncholiths are rare and broncholiths from pulmo-
nary actinomycosis even rarer. The most likely sequence of events is that
this patient infected an underlying broncholith caused by a prior his-
toplasma infection with actinomyces delivered from the oral cavity to the
lower airways during bouts of aspiration associated with syncope. The
presence of the broncholith for at least 2 years without significant
symptoms favors this sequence. Oddly, the patient’s initial event appears
to have been asymptomatic; the true sequence of events can be surmised
but not proven.
REFERENCES:
1. Nollet AS, Vansteenkiste JF, Demedts MG. Broncholithiasis: rare
but still present. Respir Med 1998;92:963-5
4. Case Records of the Massachusetts General Hospital (Case 14-
2002). N Engl J Med 2002;346:1475-82
2. Lee BY. Actinomycosis of the lung coexisting with pulmonary
tuberculosis. Chest 1996;50:211-13
3. Hirschfield LS, Graver LM, Isenberg HD. Broncholithiasis due to
Histoplasma capsulatum subsequently infected by actinomycetes.
Chest 1989;96:218-9
DISCLOSURE: Z.C. Boujaoude, None.
ENDOBRONCHIAL ACTINOMYCOSIS ASSOCIATED WITH A
FOREIGN BODY ASPIRATION
Alexandre R. Abreu, MD*; Souheil El-Chemaly, MD; Debra P. Fertel,
MD. University of Miami, Miami, FL
INTRODUCTION: Actinomyces are gram-positive anaerobic bacteria
often present in the normal flora of the oropharynx and gastrointestinal
tract. Thoracic actinomycosis most commonly occurs from oropharyngeal
aspiration, although it can occur hematogenously or by direct extension
from adjacent infected tissues. Thoracic infection usually involves lung
parenchyma, pleura, mediastinum and chest wall (1,2). Primary endo-
bronchial actinomycosis has rarely been reported.
CASE PRESENTATION: A 43 year old woman was admitted with
an 18 month history of cough, blood-tinged sputum and a persistent
right middle lobe infiltrate despite multiple courses of antibiotic
therapy. 2 prior bronchoscopies showed an endobronchial lesion
obstructing the bronchus intermedius, but biopsies showed only
granulation tissue and chronic inflammation. On physical examination
she was an afebrile, edentulous patient, with focal wheezing in the
right lower lung field. Her ESR was 30mm/hr and complete blood
count and differential were normal. HIV and PPD tests were negative.
Chest x-ray demonstrated an infiltrate in the right middle lobe. A
flexible bronchoscopy revealed an endobronchial lesion causing a
near-total occlusion of the bronchus intermedius with a distal white,
hard fungating mass (Figure 1). Histologic examination of the mass
showed edematous and chronically inflamed bronchial mucosa with
gram-positive filamentous bacterial clusters consistent with actinomy-
ces (Figure 2). Cytology from the BAL showed sulfur granules (Figure
3). In addition, culures from the BAL yielded pseudomonas aerugi-
nosa. A rigid bronchoscopy was performed and a foreign body (eraser
tip) was removed from the bronchus intermedius. Therapy with
piperacillin-tazobactam to cover both actinomyces and pseudomonal
infections was given for a total of 14 days and then switched to
ampicillin-clavulinic acid for a total of 6 months of therapy.
DISCUSSION: Thoracic actinomycosis, accounting for 15% of all
cases, usually presents as a slow, indolent mass or pneumonitis. Cavitation,
hilar adenopathy and pleural involvement may be present. Extension
across fissures, into mediastinum and to the chest wall with soft tissue
masses can mimic malignant diseases (1). Endobronchial actinomycosis
associated with a foreign body aspiration is rare. In the last 30 years a few
cases have been reported (3-5). As seen in our case, bronchoscopies can
be misleading but with appropriate biopsy sampling, handling of speci-
mens and combining both microbiologic and pathologic findings the
chances for an accurate diagnosis are enhanced. Actinomyces is a
facultative organism, but optimal growth requires strict anaerobic pro-
cessing and when suspected the microbiology laboratorist should be
alerted. Actinomycosis develops when there has been disruption of the
mucosal barrier such as it can occur with the aspiration of the foreign
body. In all prior reports, succesful treatment required removal of the
foreign body via rigid bronchoscopy and antibiotics. Duration of therapy
Monday, October 27, 2003
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 271S
CASE REPORTS

should be extended beyond resolution of clinical disease to prevent
relapses.
CONCLUSION: Thoracic actinomycosis can present as an endobron-
chial lesion mimicking malignant disease. Its presence should alert the
physician to proceed with more invasive testing to rule out foreign body
aspiration.
REFERENCES:
1. Lerner PI. Actinomyces and Arachnia species. In: Mandell GL,
Douglas RG, Bennett JE, eds. Principles and practice of infectious
diseases. New York, Churchill Livingstone, 2000:2645-2652.
2. Bassiri AG, Girgis RE, Theodore J. Actinomyces odontolticus
thoracopulmonary infections. Chest. 1996; 109:1109-1111.
3. Ho JC. Ooi GC. Lam WK. Lam B. Cheung TF. Tsang KW.
Endobronchial actinomycosis associated with a foreign body. Respi-
rology. 5(3): 293-6, 2000 Sep.
4. Mingrone H. Perrone R. La Rosa S. Schtirbu R. Lutzky L. Primary
bronchial actinomycosis and foreign body. Medicina. 55 (4): 337-40,
1995
5. Dicpinigaitis PV. Bleiweiss IJ. Krellenstein DJ. Halton KP. Teirstein
AS. Primary endobronchial actinomycosis associated with foreign
body aspiration. Chest 101 (1): 283-5, 1992 jan.
DISCLOSURE: A.R. Abreu, None.
THE USE OF DROTRECOGIN ALFA (ACTIVATED) IN A PA-
TIENT WITH SEVERE ACUTE RESPIRATORY SYNDROME
Warda Farooq, BSc, MD*; Donna McRitchie, MD, MSc, FRCSC. North
York General Hospital, Toronto, ON, Canada
INTRODUCTION: A 34-year-old female with severe acute respira-
tory syndrome (SARS) developed multi-system organ failure. We discuss
her therapy with drotrecogin alfa (activated) (DrotAA) and her clinical
course. SARS has been recently recognised in Asia, North America and
Europe. The World Health Organisation (WHO), has defined a suspected
case of SARS as: documented fever (temperature ⬎38 C), lower respira-
tory tract symptoms, contact with a person believed to have had SARS or
a history of travel to a geographic area where there has been documented
transmission of the illness. Global efforts to understand the cause, and to
prevent the spread of, SARS were instituted in March 2003. The likely
cause of SARS is believed to be a novel coronavirus. SARS typically
presents as a febrile illness, which may lead to hypoxia, lung infiltrates and
respiratory failure. In the ICU setting, patients requiring intubation and
ventilation have significant associated morbidity and mortality. These
patients may have additional associated organ dysfunction, and may
represent a form of severe sepsis.
CASE PRESENTATION: A 34-year-old female health-care worker
was diagnosed with SARS. The patient presented with a one-week history
of fever, dyspnea, fatigue, non-productive cough and pulmonary infiltrates
on chest radiography. There was progressive deterioration over 24-hours
and the patient required intubation and developed signs of multi-system
organ dysfunction. The patient met the inclusion criteria for treatment
with DrotAA, as published in The Canadian Journal of Infectious
Diseases, “Guidance on patient identification and administration of
recombinant human activated protein C for the treatment of severe
sepsis.”
DISCUSSION: This patient was treated with DrotAA at 24 _g/kg/hour
for 96-hours. The multisystem organ dysfunction involving the renal,
hepatic and coagulation system reversed during her course of therapy with
DrotAA. Creatinine returned to baseline 24-hours after completion of
DrotAA therapy, and metabolic acidosis reversed by day 4 of DrotAA
therapy. AST and ALT improved within 48 hours of initiation of DrotAA
and had returned to baseline by day 4. The patient was weaned from
inotropes and vasopressors by the end of DrotAA therapy, but she
continued to require ventilatory support as single system respiratory
dysfunction persisted. At the time of this report, after 5 weeks of
maximum ventilatory support, she is continuing to slowly improve.
CONCLUSION: Treatment with drotrecogin alfa (activated) appeared
to have benefit in this SARS case. This is the first known reported use of
drotrecogin alfa (activated) in a SARS case.
DISCLOSURE: W. Farooq , None.
ENDOBRONCHIAL MAC AS A MANIFESTATION OF IMMUNE
RECONSTITUTION SYNDROME IN AN HIV PATIENT
Marcos I. Restrepo, MD, MSc*; Angelo A. Cadena, MD; Sandra G.
Adams, MD, MSc; Stephanie M. Levine, MD. UTHSCSA, San Antonio,
TX
INTRODUCTION: Paradoxical reaction with exacerbation of clinical
or radiographic manifestations occur in patients with different infections.
Monday, October 27, 2003
Infectious Diseases I, continued
272S CHEST 2003—Case Reports

Mycobacterium avium-complex (MAC) lung disease is a rare infection in
patients presenting with a solitary pulmonary nodule (SPN) or with an
endobronchial lesion (EBL) as part of a paradoxical reaction in advanced
HIV disease. We describe an HIV patient (CD4 count ⬎100 cells/mm
3
)
with an asymptomatic SPN and evidence of endobronchial invasion due to
an infection with MAC.
CASE PRESENTATION: A 37 year-old male with a history
significant for hepatitis C virus, heavy tobacco use, and HIV (diag-
nosed in 10/2000 with a CD4 count of 41 cells/mm
3
), was admitted in
1/2001 with a 3-month history of watery diarrhea since beginning
antiretroviral therapy (combivir, nelfinavir, and delavirdine) with a
CD4 count of 125 cells/mm
3
. Normal chest radiograph (CXR) was
normal at initiation of medications. Other complaints included inter-
mittent nausea and vomiting, dysphagia, and substernal chest pain for
1 week prior to admission, which all resolved following treatment for
gastroenteritis. He had subjective fever and chills, a nonproductive
cough, and a 40 lb weight loss over the prior 6 months. The physical
examination and laboratory data were unremarkable. His skin test for
tuberculosis (which was nonreactive in 10/00) was now positive (10
mm) this admission. His CXR demonstrated a new SPN in the anterior
segment of the left upper lobe (LUL), which was not present on
previous films in 10/00. The chest CT revealed a 1.5 cm LUL soft
tissue nodule without significant hilar or mediastinal adenopathy. A
large EBL was seen at bronchoscopy, and was completely obstructing
the apical-posterior segment of the LUL. Although the transbronchial
biopsies in the area of the SPN were nondiagnostic, the biopsies of the
EBL revealed caseating granulomatous inflammation with stains pos-
itive for rare acid-fast bacilli. The endobronchial biopsy specimens and
his initial sputum samples eventually all grew MAC organisms. Initial
empiric tuberculosis treatment was changed to clarithromycin and
ethambutol upon identification of MAC. The patient continues on
MAC and antiretroviral therapy with improvement in his clinical
condition and radiographic appearance.
DISCUSSION: Infections with MAC typically occur in a subpopu-
lation of HIV patients with low CD4 counts (⬍100 cells/mm
3
). EBL
caused by mycobacterial infections has been reported with MAC and
other mycobacteria. Only 3 cases of endobronchial MAC have previ-
ously been reported in the literature, but none of this cases was a result
of a paradoxical reaction in HIV patients. This paradoxical reaction
presumably develops as a consequence of reconstitution of immune
response. Histopathologically, these EBLs demonstrate an intense
granulomatous inflammatory reaction, unlike the poor inflammatory
response seen in pulmonary MAC without endobronchial involvement.
The EBL in our case was an incidental finding and was not suspected
prior to the bronchoscopy. A high clinical suspicion and further
evaluation of pulmonary disease with fiberoptic bronchoscopy may
help further define these issues. Treatment for MAC pulmonary
disease consists of a prolonged course of combination therapy with a
newer-generation macrolide, ethambutol, and either rifampin or ri-
fabutin.
CONCLUSIONS: Endobronchial involvement and SPNs are uncom-
mon manifestations of infections with MAC in the HIV population and
have been rarely reported as a consequence of immune reconstitution.
Our patient’s presentation of MAC is unique because he presented with
a new SPN (with an incidental finding of EBL) after initiation of
antiretroviral therapy with evidence of increasing numbers of CD4 cell
count. Therefore, MAC should be considered in the differential diagnosis
of HIV patients with immune reconstitution syndrome presenting as
pulmonary nodules and endobronchial lesions.
REFERENCE
Packer SJ, et al. Mycobacterium avium complex infection presenting
as endobronchial lesions in immunosuppressed patients. Ann Intern
Med 1988;109(5):389-93.
DISCLOSURE: M.I. Restrepo, None.
Tuesday, October 28, 2003
Pleural Diseases
4:15 PM - 5:45 PM
IDIOPATHIC CHYLOTHORAX: SUCCESSFUL TREATMENT
WITH PERCUTANEOUS RETROGRADE CATHETERIZATION
AND EMBOLIZATION OF THE DUCT
Michael C. DeArment, MD*; Gerald R. May, MD; Syed W. Malik, MD.
Pulmonary & Critical Care Medicine, Mayo Clinic, Jacksonville, FL and
Critical Care Medicine, Mayo Clinic, Rochester, MN
INTRODUCTION: A chylothorax is formed by the accumulation of chyle
in the pleural space. We present a case of recurrent idiopathic chylothoraces
successfully treated with percutaneous thoracic duct embolization.
CASE REPORT: A 65 year-old male lifelong never-smoker presented
to pulmonary clinic for evaluation of a persistent left sided pleural
effusion. He complained of exertional dyspnea. His past medical history
included congestive heart failure. The physical exam was notable for
diminished left sided breath sounds and dullness to percussion. Com-
puted tomography (CT) of the chest demonstrated left sided pleural
effusion with compressive atelectasis of the left base. Thoracentesis
revealed a turbid yellow odorless effusion. Pleural fluid analysis were
consistent with a transudative chylothorax. Pleural fluid and serum
characteristics are shown in the following table:
Pleural Fluid and Serum Chemical Characteristics
Source
LDH
(U/L)
Protein
(g/dl)
Triglyceride
(mg/dl)
Cholesterol
(mg/dl)
Albumin
(g/dl)
Glucose
(mg/dl)
WBC
count
Pleural 93 1.7 152 29 1.0 279 240
Serum 322 7.1 87 145 4.0 144 7500
Lymphangiography revealed a leak in the thoracic duct below the level
of the left main stem bronchus. The duct was percutaneously catheterized
in retrograde fashion from the neck and embolized with platinum coils.
DISCUSSION: The leading cause of chylothorax is tumor disrupting
the thoracic duct, with lymphomas comprising the majority of cases. A
significant number of chylothoraces are considered to be idiopathic. A
pleural fluid triglyceride level above 110 mg/dl supports the diagnosis of
chylothorax and a level less than 50 mg/dl makes the diagnosis less likely.
Some patients with chyloform pleural effusions can have triglyceride
levels above 110 mg/dl, but they will likely have cholesterol levels above
200 mg/dl. Our patient successfully underwent lymphangiography fol-
lowed by percutaneous thoracic duct embolization resulting in resolution
of the chylothorax. A unique feature of our case is that the embolization
was performed via retrograde approach to the thoracic duct.
CLINICAL IMPLICATIONS: Chylothorax treated with minimally
invasive percutaneous thoracic duct embolization offers an advantage in
patients unable to tolerate a more aggressive surgical approach. Percuta-
neous coil embolization of the thoracic duct is a potential treatment option
for patients with recurrent idiopathic chylothoraces.
DISCLOSURE: M.C. DeArment, None.
SUCCESSFUL SURGICAL TREATMENT OF RECURRENT
PLEURAL EFFUSIONS FROM SVC SYNDROME RELATED TO
MEDIASTINAL FIBROSIS DIAGNOSED 24 YEARS EARLIER
Sean M. Caples, DO*; James Utz, MD. Mayo Clinic, Rochester, MN
INTRODUCTION: Transudative pleural effusions may result from the
superior vena cava (SVC) syndrome. The most frequent benign etiology of
the SVC syndrome is fibrosing mediastinitis, which is often related to previous
histoplasma infection. Herein, we describe a case of a woman who developed
the SVC syndrome 24 years after she was diagnosed with fibrosing medias-
tinitis, and her excellent response to surgical therapy.
CASE PRESENTATION: An otherwise healthy 53 year-old woman,
non-smoker, presented in late 2002 with progressive dyspnea and hypox-
emia related to recurrent bilateral pleural effusions over the previous six
months. In 1978, because of an abnormality on a routine chest x-ray, she
was diagnosed with fibrosing mediastinitis based upon an open lung
biopsy. She remained asymptomatic, however, until her current presen-
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tation 24 years later. Over this six month period, she underwent multiple
thoracenteses, which revealed transudative effusions. The pleural taps
temporarily relieved her symptoms, only for the effusions to quickly recur.
Physical examination revealed dilated veins along the anterior chest and
upper extremities. CT chest demonstrated calcified mediastinal thicken-
ing and right hilar lymph nodes, obliteration of the SVC, and venous
collateral circulation along the chest wall. Upper extremity venography
(see Figure) showed obliteration of the SVC at the junction of the
innominate veins and a large caliber azygous vein with collaterals. To
exclude chronic pericardial disease as a cause of transudative pleural
effusions, a right and left heart catheterization was performed, which
revealed pulmonary hypertension and otherwise normal chamber pres-
sures and function. Due to worsening symptoms and hypoxemia, the
patient underwent surgical reconstruction of the SVC via a left innominate
vein to right atrium bypass using a spiral saphenous vein graft. Ten months
later, the patient is asymptomatic, chest and abdominal wall veins have
regressed in size, and pleural effusions have not recurred.
DISCUSSION: Although rare, particularly in cases associated with a
non-malignant origin, SVC syndrome can cause transudative pleural
effusions. It should be considered in atypical presentations, such as in
patients without a history of cardiac, renal or hepatic disease. Suspicion
should be high in cases of pre-existing fibrosing mediastinitis, which may
precede the syndrome by several years. The pathophysiology of transu-
dative pleural effusions secondary to SVC syndrome is thought to be
related to elevated central venous pressures, despite often well-developed
collateral circulation.
Symptomatic SVC syndrome due to long standing fibrosing mediasti-
nitis may present a difficult treatment problem. However, in cases with
resultant transudative pleural effusions, large vein bypass graft surgery
may be an effective method of central venous decompression. This case
reports the excellent response to surgical therapy in relieving symptoms,
and preventing recurrence, of pleural effusions secondary to the SVC
syndrome.
CONCLUSION: SVC syndrome resulting from fibrosing mediastinitis
is a relatively uncommon cause of transudative pleural effusions. Venous
bypass surgery may be a very effective form of symptomatic treatment.
REFERENCES
Doty, et al. Superior vena cava obstruction: bypass using spiral vein
graft. Ann Thorac Surg 1999; 67:1111-6.
DISCLOSURE: S.M. Caples, None.
PLEURAL INFECTION DUE TO SCEDOSPORIUM APIOSPER-
MUM/PSEUDALLESCHERIA BOYDII
Pablo E. Molina, MD*; Juan A. Garcia, MD, FCCP; Steven Im, MD.
University of Texas Health Science Center at San Antonio, San Antonio,
TX
INTRODUCTION: Scedosporium apiospermum infections are rare.
This fungi can present causing superficial skin and wound infections, or
with invasive disease. Bone, sinus, prosthetic valve, eye, lung, brain,
pericardium, kidney, and disseminated infections have been described.
Pleural infection has been reported only in 2 cases. Invasive infections
have a high mortality, and have been reported mainly in immunocompro-
missed hosts. The other groups of patients susceptible to invasive disease
are trauma or near-drowning victims, and farmers. Sc. apiospermum is the
asexual form of Pseudallescheria boydii. This microorganism has also been
called Monosporium apiospermum, Petrellidium boydii, and Allescheria
boydii.
CASE PRESENTATION: A seventy-year-old male was admitted for
investigation of left sided pleural thickening and effusion.
One month before, the patient presented to one of our clinics
complaining of dull pain on the left hemithorax for 2 months. Chest
radiograph (CXR) and chest computed tomography (CT) revealed pleural
thickening above the left costophrenic angle. In addition, a small amount
of air was seen inside the thickened pleura on chest CT. The process was
felt to represent an anaerobic infection, and amoxacillin/clavulanate was
prescribed.
Repeat CXR and chest CT done one month later showed progression of
the pleural process with development of effusion. His clinical status was
unchanged.
Past medical history was significant for celiac disease and malnutrition.
The patient was not taking any immunosuppressants.
Physical examination was remarkable for: a chronically ill looking
cachectic patient, poor dentition, decreased breath sounds on the left
lower lung field, and 2 ⫹ ankle and pre-tibial edema. He was not
neutropenic, and his albumin and pre-albumin were 1.3 g/dl and ⬍7.4
mg/dl respectively.
Thoracentesis revealed an exudative effusion with neutrophil predom-
inance. Smears were negative for AFB/fungal/bacteria.
Pleural fluid culture showed masses of septate hyphae, later identified
as Sc. apiospermum.
A second thoracentesis was performed and the culture again was
positive for the fungi. Cardio-thoracic surgery was consulted, and the
patient was deemed not to be a surgical candidate due to his poor
performance and nutritional status.
Treatment with voriconazole was started.
Eight days later the patient became hypotensive and was transferred to
the intensive care unit. Treatment for nosocomial pneumonia was insti-
tuted. On ICU day 4, patient developed left sided hemiparesis. Head CT
demonstrated large hypodense areas in the right basal ganglia, right
frontal, and right parietal lobes with presence of air in those areas. The
patient continued to deteriorate and died on ICU day 6. No permission for
autopsy was granted.
DISCUSSION: We report the third case of pleural infection due to Sc.
apiospermum. There is only one case of primary pleural infection reported
previously; an immunocompetent patient treated with decortication with
good results. The other reported case of pleural involvement, occurred in
a lung transplant recipient that developed lung infection and pleural
effusion secondary to the fungi with subsequent dissemination. We
considered our patient to be immunosupressed due to his severe degree
of malnutrition. His case was complicated by dissemination of the disease.
The large ischemic changes and the presence of air in the brain
parenchyma seen on head CT were most likely secondary to the fungi. Sc
apisopermum is known to cause brain infarcts, abscesses, necrosis, and
mycotic aneurysms.
Surgical debridement/resection and voriconazole are recommended for
the treatment of invasive infections. Other options include intraconazole,
miconazole, and ketoconazole. The fungus is usually resistant to ampho-
tericin B. Unfortunately our patient’s nutritional and performance status
precluded surgical treatment.
CONCLUSION: Sc apiospermum should be considered in the differ-
ential diagnosis of pleural thickening and/or pleural effusion in immuno-
supressed patients. Its isolation from a sterile site represents infection.
Accurate diagnosis is crucial to institute adequate treatment.
DISCLOSURE: P.E. Molina, None.
Tuesday, October 28, 2003
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274S CHEST 2003—Case Reports

UNCOMMON PRESENTATION OF RHEUMATOID PLEURITIS
Frank R. Quijano, MD*; Timothy T. Dwyer, MD; Trenton D. Nauser,
MD, FCCP. University of Kansas Medical Center, Kansas City, KS
INTRODUCTION: Pleural disease preceding arthralgia occurs in
⬍ 7% of patients with rheumatoid pleuritis. Bilateral pleural effusions are
infrequent and massive pleural effusions are rare. We describe a case with
the aforementioned features, confirmed by pathognomonic cytologic
findings to be rheumatoid pleuritis.
CASE PRESENTATION: A 56 year-old alcoholic male presented
with progressive dyspnea and cough. Physical exam revealed a febrile,
tachycardic, unkempt male with poor dentition, left-sided diminished
breath sounds, percussion dullness, and normal musculoskeletal exam.
Chest radiograph confirmed a massive left pleural effusion. Pleural
fluid analysis disclosed pH 7.18, glucose ⬍ 10 mg/dl, LDH 3273 IU/L,
total protein 4.9 g/dL, WBC 930,000 with 68% neutrophils, negative
gram stain, and severe acute inflammation without malignancy on
cytology. Closed tube thoracostomy was performed. Despite ceftriax-
one and clindamycin, fever persisted with development of diffuse
arthralgia. Chest computed tomography (CT) confirmed left pleural
fluid evacuation, small right pleural effusion, and cavitary subpleural
nodule. Right pleural fluid resembled left-sided fluid upon laboratory
evaluation. Pleural fluid Rheumatoid Factor (RF) was 160 IU/ml and
serum RF 320 IU/ml. Cultures remained negative. Cytologic review of
pleural fluid samples revealed the diagnostic triad: elongated macro-
phages, giant multinucleated macrophages, and background of granu-
lar necrotic debris. Daily oral prednisone 40 mg resulted in resolution
of pleuritis and arthralgias.
DISCUSSION: Atypical presentation and aspiration pneumonia risk
factors lead to initial treatment for complicated parapneumonic effusion.
Lack of improvement after pleural fluid drainage and antibiotics, onset of
arthralgias, and subpleural nodule on CT shifted our focus toward
rheumatoid pleuritis. Although no diagnostic pleural to serum RF ratio
exists, the cytologic pathognomonic triad for rheumatoid pleuritis estab-
lished the diagnosis.
CONCLUSION: When rheumatoid pleuritis is suspected, the cyto-
pathologist should be alerted to search for the diagnostic triad.
REFERENCE: Naylor B. The pathognomonic cytologic picture of
rheumatoid pleuritis: the 1989 Maurice Goldblatt Cytology Award Lec-
ture. Act Cytol 1990; 34:465-73.
DISCLOSURE: F.R. Quijano, None.
MASSIVE RECURRENT TRANSUDATIVE PLEURAL EFFU-
SION: ROLE OF MEASUREMENT OF PLEURAL PRESSURE AS
A DIAGNOSTIC TOOL
Vinette E. Coelho-D’Costa, MD*; Sami A. Nachman, MD; John Salazar-
Schicchi, MD. *Harlem Hospital Center and College of Physicians and
Surgeons of Columbia University, New York, NY
INTRODUCTION: Pleural pressure(PIP) is the balance between
outward pull of thoracic cavity and inward pull of the lung. Light et al and
others have reported the measurement of PIP during therapeutic thora-
centesis(TT).
1
Vilhena et al tried to ascertain whether PIP measurement
provides any clue to the etiology of the effusion.
2
We report the technique
of measurement of PIP during TT to rule out trapped lung in a
49-year-old male with hepatitis B, cirrhosis and portal hypertension, who
presented with recurrent pleural effusion. To our knowledge the tech-
nique that we used has not been previousely described in the literature
CASE REPORT: A 49-year-old male with history of hepatitis B for 3
years, cirrhosis, hypertension, chronic renal insufficiency, GI bleed was
referred to us with increasing dyspnea, decreased exercise tolerance and
orthopnea. Similar symptoms 2 months ago had revealed a large Right-
sided pleural effusion with collapse of the right lung (Fig1). He was a
painter with a 1 pack-year smoking history and a family history of lung
cancer. Repeated TT yielded large amounts of transudative pleural
effusion which rapidly reaccumulated. No significant improvement was
noted with furosemide and spironolactone. Abdominal ultrasonograhy
revealed no ascites. Bronchoscopy did not reveal any endobronchial
lesion. Refractory hepatic hydrothorax without ascites and trapped lung
were the diagnoses entertained. Pleural thickening was difficult to assess
on chest CT done immeditely after TT, because of rapid reaccumulation
of fluid.. Therefore it was decided to measure PIP to rule out trapped
lung.
The procedure was performed in ICU with the patient sitting upright A
“Kendall” thoracentesis system with an angiocath catheter “BD insyte-
autoguard” 16G, 1.7 inch was used to perform the TT. This was connected
to an A line transducer which in turn was connected to the “Space lab”
monitor (scale 30). Zero value was set at the thoracic puncture level. PlP
was measured before removing any fluid, after the removal of every 100
ml for the first 500 ml, then after the withdrawal of every 500 ml for the
second liter. A total of 2000ml of fluid was aspirated. The mean of the
Inspiratory and expiratory PIP values was calculated. Clinical signs and
symptoms (chest pain, cough, and chest tightness) were watched for. The
pressures obtained are seen in Table 1.
The initial PIP at the onset of the procedure was -1cm H
2
O; -3cm H
2
O
after withdrawal of the first liter and -8cm H
2
O at the end of 2 liters.
These readings did not meet the criteria for the diagnosis of trapped lung.
Table 1: Pleural pressure
Volume Aspirated
(ml)
End Exp. Pressure
(cm H
2
O)
End Insp. Pressure
(cm H
2
O)
0 ⫹3-5
100 ⫹3-5
200 ⫹3-5
300 ⫹3-5
400 ⫹5-3
500 ⫹1-5
1000 0 -7
1500 0 -10
2000 -3 -13
DISCUSSION: PIP can be measured directly (using needles, trocars,
catheters, balloons) or indirectly (by positioning a balloon / micromanom-
eter in the esophagus).
In trapped lung a fibrous peel may form over the visceral pleura in
response to pleural inflammation and prevent underlying lung from
expanding. It can be diagnosed when the initial PIP during TT is below
-10cm H
2
O and PIP falls more than 20 cm H
2
O per 1000ml as fluid is
removed.
In our patient the initial pressure was around -2cm H
2
O and dropped
by less than 20cm H
2
O after removal of each 1000 ml ruling out Trapped
lung
CONCLUSION: PIP measurement can be easily performed at the
bedside during thoracentesis and can be an important tool to assisst in the
diagnosis of pleural effusion
REFERENCES:
1. Light RW, Jenkinson SG, Minh VD, George RB. Observation on
pleural fluid pressures as fluid is withdrawn during tharacentesis.
Am. Rev. Respir. Dis. 1980;121:799-804
Fig. 1. Before Thoracentesis
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CASE REPORTS

2. Villena V, Lopez-Encuentra A, Pozo F, De-Pablo A, Martin-
Escribano P. Measurement of Pleural Pressure during Therapeutic
Thoracentesis. Am J Respir. Crit Care Med. 2000;162:1534-1538
DISCLOSURE: V.E. Coelho-D’Costa, None.
PERGOLIDE-INDUCED PLEURAL DISEASE IN A PATIENT
WITH ASBESTOS EXPOSURE
Naushad Hirani, MD*; Brian Forzley, MD; Jonathan Langridge, MD;
Charlie Bayliff, PharmD; David G. McCormack, MD, FRCP. University
of Western Ontario, London, ON, Canada
INTRODUCTION: Exposure to ergot derivative compounds has been
associated with pleuropulmonary fibrosis. We report a case of pleural
disease induced by pergolide, initially mimicking asbestos-related pleuri-
tis.
CASE PRESENTATION: A 66-year-old male was referred with a two
month history of progressively worsening dyspnea and dry cough. His past
medical history was significant for restless legs syndrome treated success-
fully with pergolide for 4 years. Occupational history included two years of
low-level asbestos exposure while working in a shipyard 40 years prior, and
no risk factors for tuberculosis. After failing a course of clarithromycin, he
was admitted to hospital with dyspnea, a moderate right-pleural effusion,
and bilateral pleural thickening. Thoracentesis demonstrated an exudate,
but Gram stain and cultures were negative. Baseline pulmonary function
had included an FEV
1
of 1.73L (72%), TLC 3.88L (70%) and preserved
DLCO. Over two months, the TLC had deteriorated to 3.21L (59%). A
thoracic CT-scan showed bilateral pleural thickening with patchy areas of
calcification, a small right-pleural effusion, and no parenchymal disease.
Rounded atelectasis was noted in the right upper, middle and lower lobes.
Pathology from samples obtained at pleuroscopy suggested fibrous pleu-
ritis and no evidence of malignancy or asbestos-bodies. The patient was
readmitted to hospital one month later with worsening dyspnea associated
with worsening extraparenchymal restriction (TLC 2.75L), but no changes
on CT-scan. An echocardiogram revealed normal left-ventricular function
and pericardial appearance. Pergolide was discontinued and the patient
treated with 50 mg of prednisone daily. There was mild symptomatic
improvement initially, and repeat pulmonary function, imaging, and
clinical assessment are pending.
DISCUSSION: Diagnosing drug-induced lung disease is difficult, for
in addition to being a diagnosis of exclusion, pulmonary changes often do
not correlate immediately with exposure and may not readily regress
following withdrawal of an offending agent. There are 8 reported cases of
pergolide-related pulmonary toxicity in the literature,
1-4
although the
experience with other ergoline drugs, such as bromocriptine, is more
extensive. The mechanism of ergot alkaloid toxicity is unknown, but some
have postulated that it may be due to upregulation of serotonin-mediated
fibrosis.
5
The presence of rounded atelectasis and pleural plaques initially
suggested a process related to asbestos exposure as the etiology in our
case. Calcified plaques are sensitive for asbestos-related disease, but
rounded atelectasis has been reported to occur in a number of other
settings. In addition to the absence of asbestos-bodies on pathology, the
minimal duration and low level of exposure to asbestos argued against
asbestos as the only cause, especially considering that rounded atelectasis
is typically associated with over two decades of exposure.
6
Also, the
subacute course and symptomatic improvement following withdrawal
implicated pergolide in the process. Objective improvement may yet be
demonstrable, and has been observed with other ergolines from 2 months
up to 4 years after withdrawal.
5
Several (40%) of the reported cases of pergolide-induced pleural
disease also reported the patient having had some asbestos exposure.
1,3
This raises the possibility of a synergistic relationship between the two
exposures for causing pleural complications, as has been suggested in the
context of other ergot drugs.
7
CONCLUSIONS: Pergolide-induced pleural disease is becoming a
well-recognized entity. There may be a synergistic effect between levels of
asbestos exposure not normally associated with disease and pergolide use.
We submit that it would be prudent to offer patients with pre-existing
pleural disease or asbestos exposure an alternative to long-term pergolide
therapy.
REFERENCES:
(1) Clin Neuropharm 2002; 25(5):290-3
(2) Resp Med 2002; 96:548-50
(3) Chest 2001; 120:313-16
(4) J Neurol Neurosurg Psych 1999; 66:79-81
(5) Eur Resp J 1996; 9:1013-19
(6) Chest 1995; 107:477-81
(7) Eur Resp J 1997; 10:2695-98
DISCLOSURE: N. Hirani, None.
Diffuse Lung Disease
4:15 PM - 5:45 PM
IDIOPATHIC PULMONARY HEMOSIDEROSIS
Dmitriy Vaysman, MD*; Haesoon Lee, MD. Department of Pediatrics,
Winthrop University Hospital, SUNY Stony Brook School of Medicine,
Mineola, NY
INTRODUCTION: Idiopathic pulmonary hemosiderosis (IPH) is an
uncommon disorder of unknown etiology. It manifests as a triad of
hemoptysis, diffuse intra-alveolar infiltrates on chest radiograph (CXR)
Fig. 2. After Multiple Thoracenteses
Tuesday, October 28, 2003
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276S CHEST 2003—Case Reports

and iron deficiency anemia. Although the association with cow’s milk
protein allergy and celiac disease has been reported in some patients, the
etiology of repeated pulmonary hemorrhage in IPH remains unknown.
CASE PRESENTATION: A 3-1/2 year-old girl with an episode of
profound iron deficiency anemia (hemoglobin [Hb] 3.4gm/dL, hematocrit
[Hct] 12.1%) at 1-1/2 years of age, received packed red blood cells
(PRBC), currently presented with symptoms of upper respiratory infec-
tion, cough and fever. In the emergency department, she had temperature
100.7 F, pulse 178 /min, respiratory rate 68 /min, BP 87/52, and oxygen
saturation of 80% on room air. She was pale, lethargic, had moderate
intercostal retractions and clear lung sounds with good air entry. Arterial
blood gas was pH 7.42, PaCO2 32mmHg, and PaO2 75mmHg on 2 L/min
of oxygen via facemask. CBC showed Hb 4.8 gm/dL, Hct 15.2%, MCV 60,
WBC 13.8 k/cumm and reticulocyte count of 6.6 %. CXR showed bilateral
diffuse fluffy infiltrates (Fig.1). She was admitted to the pediatric intensive
care unit, given oxygen and transfused with PRBC. Computerized
tomography (CT) of the chest showed a diffuse airspace disease (Fig.2).
Bronchoscopy with bronchoalveolar lavage (BAL) was followed by an
open lung biopsy. Both showed hemosiderin-laden macrophages. Biopsy
showed acute and chronic intra-alveolar hemorrhage and strongly positive
stain for iron (Fig.3, 4). Bacterial, viral, fungal and acid-fast smear and
culture were all negative as was the stain for pneumocystis carinii.
Work-ups for rheumatologic diseases, hypersensitivity pneumonitis, celiac
disease and bleeding disorders were negative. Stachybotris chartarum IgE
and IgG were within normal limits and anti-cow’s milk proteins IgG was
absent. IgE was elevated at 2290 u/mL. She was treated with intravenous
methylprednisolone 2 mg/kg/day with improvement. Upon discharge from
hospital, Hb and Hct were 13.9mg/dL and 42.7% respectively and CXR
showed resolution of alveolar infiltrates. The prednisolone dose was
tapered off over a 4-months period when Hb decreased to 10.6 gm/dL.
She appeared pale but had no hemoptysis. A CXR revealed prominent
interstitial markings. High resolution CT (HRCT) of the chest showed a
fine nodular pattern throughout the lung fields. She was then started on
azathioprine 4 mg/kg/day and has done well since then.
DISCUSSION: The prognosis of IPH is difficult to predict because of
paucity of the reported data. Death may occur suddenly from an acute
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CASE REPORTS

pulmonary hemorrhage or from chronic respiratory failure secondary to
pulmonary fibrosis. Survival varies among the reported series from 2.5
years to 86% 5 years survival rate. Efficacy of the treatment is also difficult
to assess due to the variable clinical course and rarity of the disease.
Corticosteroids (CS) are most commonly used. However, the disease can
progress even while the patient is on it. Other immunosuppressants are
occasionally employed for those who have poor response to CS. Lung
transplantation has been reported in one patient with unsuccessful
outcome.
CONCLUSION: IPH may present atypically in children who do not
have a history of hemoptysis because they swallow sputum. IPH is a
diagnosis of exclusion. The diagnosis is considered in the presence of
hemosiderin-laden macrophages in BAL and in the lung biopsy
specimen when other common causes of hemorrhage are excluded.
The lung biopsy should not show any evidence of vasculitis, capillaritis,
granulomas or immunoglobulin deposition. CS and other immunosup-
pressants are used in acute episode and for chronic maintenance
therapy but the response is variable. A diagnosis of IPH should be
considered for a patient with intermittent dyspnea, pulmonary infil-
trates and iron deficiency anemia.
DISCLOSURE: D. Vaysman, None.
PULMONARY NECROBIOTIC NODULE (PNN) RELATED TO
CHRONIC EXPOSURE TO BLAST FURNACE FLUE DUST
Samer T. Morcos, MD*; Alan Betensley, MD. Henry Ford Hospital,
Detroit, MI
INTRODUCTION: Pulmonary necrobiotic nodules (PNN) are rare
and are usually associated with rheumatoid arthritis (RA).
1,2
PNN in
the absence of arthritis is an even rarer entity and may precede the
evidence of RA by as many as 11 years.
3
Several cases are described in
the literature as idiopathic.
3
CASE PRESENTATION: A 50 year-old asymptomatic white male
was found to have right upper lobe opacity on a chest x-ray. He had a
100-pack year smoking history and a 22-year history of occupational
exposure to flue dust from a steel blast furnace in a motor company.
Physical examination revealed severe finger and toe clubbing. There
was no evidence of joint tenderness, effusion, or synovitis. Laboratory
studies including ANA and rheumatoid factor were unremarkable.
Computed tomography of the chest showed a 2.8 cm nodule in the
right upper lobe in addition to extensive emphysema and numerous
subpleural bullae. Right upper lobectomy was performed and the
histopathology showed necrobiotic granulomatous inflamation and
patchy fibrosis.
There were ferruginous bodies on iron stain and numerous iron-
containing macrophages. His clubbing remarkably improved within
months of retirement from his work.
DISCUSSION: Our patient was exposed to blast furnace flue dust
containing 20-30% iron oxide for a period of 22 years. The histopatholog-
ical findings of ferruginous bodies and iron-containing macrophages
suggest that the necrobiotic nodule is related to the patient’s exposure,
and we have excluded the most common cause of PNN, rhematoid
arthritis. The rapid improvement of the clubbing following the patient’s
early retirement also supports the relationship between the disease
process and the inhalational exposure.
CONCLUSION: In our patient, we have shown evidence to support
the relationship between the exposure to blast furnace flue dust and PNN,
which has not been previously described in the literature. Futher case
studies are needed to support our findings.
REFERENCES:
1. Sarkar TK et al.Pulmonary necrobiotic nodule.J Rheumatology
1984;11:557-8.
2. Johnson TS et al.Endobronchial necrobiotic nodule.Chest 1982;82:
199-200.
3. Eraut D et al.Pulmonary necrobitic nodules without RA.Br J Dis
Chest 1978;72:301-6.
DISCLOSURE: S.T. Morcos, None.
Tuesday, October 28, 2003
Diffuse Lung Disease, continued
278S CHEST 2003—Case Reports

SMUT LUNG: AN ATYPICAL HYPERSENSITIVITY PNEUMONI-
TIS
Scott E. Evans, MD*; Andrew H. Limper, MD. Mayo Clinic and
Foundation, Rochester, MN
INTRODUCTION: Hypersensitivity pneumonitis develops following
sensitization to innumerable organic aerosols and chemical antigens, many
found ubiquitously. An illustrative case is presented, demonstrating
atypical hypersensitivity pneumonitis resulting from a commonly found
organism.
CASE PRESENTATION: A 55 year-old woman with no smoking
history reported five years’ progressive exertional dyspnea. Shortly pre-
ceding the onset of her dyspnea, she abruptly developed a minimally
productive cough, which persisted until her presentation. Multiple
courses of antibiotics and ␤-agonists failed to relieve her symptoms, which
varied over time. She denied fevers or sweats, and no ill-contacts were
identified. No relevant occupational or environmental exposure history
was elicited. Examination revealed only trace, scattered wheezes on
forced expiration. Pulmonary function testing demonstrated low-normal
lung volumes with a diffusing capacity of 53% predicted, but no inducible
airflows obstruction. High resolution CT images demonstrated mosaic
attenuation of both lungs, with regional air trapping, scattered ground
glass infiltrates, and mild lymphadenopathy. Chronic interstitial pneumo-
nia was seen on lung biopsy, with non-necrotizing granulomata, bronchi-
olitis, and multinucleated giant cells. Pseudozyma was cultured from the
biopsy specimen. The patient was treated with corticosteroids for
Pseudozyma-induced hypersensitivity pneumonitis, with prompt symp-
tomatic, radiographic and gas exchange improvement.
DISCUSSION: Fungal organisms are among the more typical precip-
itants of immune-mediated lung injury. The fungal order Ustilaginales,
including Pseudozyma/Tilletiopsis species, contains the phytopathogenic
corn and grain smuts which cause crop losses worldwide. Yet, despite their
ubiquity, these organisms have rarely been reported to induce hypersen-
sitivity pneumonitis. While antigen avoidance was impossible, this patient
responded favorably to corticosteroid therapy.
CLINICAL IMPLICATIONS: The intriguing rarity of corn and grain
smut-induced lung injury may eventually offer insight into the pathogen-
esis of hypersensitivity pneumonitis. It also reminds clinicians that the
absence of an obvious exposure history cannot exclude the diagnosis of
hypersensitivity pneumonitis, due to its countless causes.
REFERENCES: Yoshida, et al. Thorax 51:650-1, 1996. Martinez-
Espinoza, et al. Fungal Genetics and Biology 35:1-20, 2002.
DISCLOSURE: S.E. Evans, None.
ALLERGIC BRONCHOPULMONARY MYCOSIS SECONDARY
TO SCEDOSPORIUM APIOSPERMUM IN A PATIENT WITH
PARTIAL IMMOTILE CILIA SYNDROME
Amro Y. Al-Astal, MD*; R. Assaly MD; D. Olson MD, PhD; J. Hammer-
sley, MD. Medical College of Ohio, Toledo, OH
INTRODUCTION: Following the description of allergic bronchopul-
monary Aspergillosis (ABPA), patients with clinical pictures similar to
those with ABPA have been described leading to the new term allergic
bronchopulmonary Mycosis (ABPM).
Implicated fungi in the pathogenesis of ABPM include Aspergillus Sp.
other than A. fumigatus, Candida albicans, Curvularia lunata, Mucor Sp.,
Penicillium, Torulopsis glabrata, and Scedosporium apiospermum. (1)
CASE PRESENTATION: A 42-year-old white female, known to have
childhood asthma, with more frequent attacks for the last 8 years.
The patient was treated in the past 4 years intermittently with
Prednisone and itraconazole, was presumed to have ABPA based on the
isolation of aspergillus in the sputum, asthma, the central Bronchectasis,
and LgE level of 3534 mg/dl.
The precipitin test was weakly positive for Aspergillus (1 band).
The patient’s family history is notable for a 1-year-older sister with
Kartagener syndrome.
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Electronmicroscopy of bronchial wall brush showed dynine arms
abnormalities and absent inner arms c/w partial immotile cilia disorder.
(Fig.1)
The BAL grew Scedosporium Apiopremum (Fig. 2) and Mycobacte-
rium Chelonae.
Her IgE level was 3085 mg/dl.
The patient was restarted on Itraconazole, prednisone, and Clarythro-
mycin and showed significant improvement thereafter.
DISCUSSION: Scedosporium apiospermum is a ubiquitous sapro-
phyte that has been isolated from soil, animal feces, and polluted water.
Pulmonary scedosporiosis may be caused by S. apiospermum, a rare
event that usually occurs on a previous damaged lung tissue such as a
cavity, bronchiectasis or a lung cyst, leading to the development of a
mycetoma (2). It may also cause allergic bronchopulmonary reaction (3).
In immunocompromised patients, invasive infections may sometimes
occur (4).
CONCLUSION: Scedosporium apiospermum can cause ABPM and
can be easily confused with Aspergillus. Because S. apiospermum is most
often resistant to amphotericin B and sometimes sensitive to azole
derivatives, differentiation is essential.
REFERENCES:
1) Am Rev respire Dis Vol 148. pp 810-12, 1993.
2) Travis L.B., Roberts G.D., Wilson W.R., Mayo Clin Proc, Volume:
60, (1985), pp. 531-537
3) Lake F.R., Tribe A.E., McAleer R., Froudist J., Thompson P.J.,
“Mixed allergic bronchopulmonary fungal disease due to Pseudallescheria
boydii and Aspergillus”, Thorax, Volume: 45, (1990), pp. 489-491.
4) Rubin R.H., “Fungal infection in the immunocompromised host”,
Fishman A.P., (Ed.) Pulmonary diseases and disorders, (1988), pp.
1761-1764 McGraw-Hill.
DISCLOSURE: A.Y. Al-Astal, None.
AN UNUSUAL PRESENTATION OF AN UNCOMMON DISEASE:
A DIFFUSE CYSTIC RADIOLOGIC PATTERN IN A PATIENT
WITH LOCALIZED PULMONARY AMYLOIDOSIS
Jennifer S. Wink, MD*. Walter Reed Army Medical Center, Washington,
DC
INTRODUCTION: Pulmonary amyloidosis is found in both systemic
and localized forms. A diffuse interstitial pattern is typically found in
systemic disease. The characteristic radiologic patterns in interstitial
amyloidosis include diffuse micronodular or reticulonodular. We present
a case of amyloidosis which is unique in that the CT has a predominantly
cystic pattern and the presence of interstitial lung disease is the only
manifestation of amyloidosis.
CASE PRESENTATION: A 47 yo male with no significant past
medical history presented to his primary physician in 1999 complaining of
increasing shortness of breath. He was diagnosed with reactive airways
disease and was placed on inhaled steroids and beta-agonists without
improvement. He was referred to a pulmonary physician in 2000, where
he underwent PFT’s and a CT scan. PFT’s showed a restrictive pattern
with significant air trapping. The CT scan showed diffuse interstitial
disease with diffuse cysts. He was sent for open lung biopsy in October
2000, which revealed parenchymal nodules with amorphous eosinophilic
extracellular material consistent with amyloid. An extensive search for
systemic disease was negative. The patient reported to WRAMC in
January 2003. He complained of increasing shortness of breath. PFT’sat
that time showed an 800cc decrement in his FVC. At Walter Reed, the
patient has undergone a second full evaluation searching for systemic
amyloidosis. Again, he has no evidence of systemic disease. He is currently
awaiting bone marrow biopsy.
DISCUSSION: Pulmonary amyloidosis can occur in two distinct
settings. Most commonly, it is seen as manifestation of a systemic disorder.
A distinct entity of localized pulmonary amyloidosis has also been
described. In this subset of patients, the pulmonary manifestations include
tracheobronchial amyloidosis or nodular amyloid lesions. In general, the
prognosis is excellent for these disorders. A diffuse interstitial pattern is
decidedly rare without systemic disease. In the most recent series, Utz et
al. evaluated all of the patients with pulmonary amyloidosis at Mayo Clinic
from 1980 to 19931. Fifty-five patients were found to have pulmonary
amyloidosis. In thirty-eight of these, the pulmonary symptoms were a
manifestation of systemic disease. Of the seventeen patients with localized
pulmonary disease, no patient had a diffuse interstitial pattern (4 tracheo-
bronchial, 7 nodular, 6 senile). Hui et al published a retrospective study of
48 patients with amyloidosis localized to the lower respiratory tract2. They
found 6 patients who had a diffuse interstitial pattern of involvement.
However, in this series, there was a paucity of clinical information and the
investigators were sure of the localized nature of the amyloidosis in only
6/48 patients. Therefore, the 6 cases of diffuse interstitial disease may not
have had amyloidosis localized the the pulmonary tract.
The reported cases with diffuse interstitial changes amyloidosis have
rarely shown a predominance of cysts. Our patient presented with cystic
lung disease with obstruction and air trapping. Review of the literature
only show 2 cases that report a predominance of cysts, and only 1 of these
was patient with localized pulmonary disease 3,4.
CONCLUSION: This case illustrates three important points to prac-
ticing pulmonologists. First, it argues for the need to take atypical cases of
interstitial lung disease to lung biopsy. Secondly, it demonstrates that
localized pulmonary amyloidosis can present as diffuse ILD and changes
on HRCT may be predominantly cystic.
REFERENCES:
1 Utz. Pulmonary Amyloidosis. The Mayo Clinic Experience from
1980 to 1993. Ann Intern Med 1996; 124:407-413
2 Hui. Amyloidosis Presenting in the lower respiratory tract. Arch
Pathol Lab Med 1986; 110:212-218
3 Koybayashi. Sjogren’s Syndrome with Mulitple Bullae and Pulmo-
nary Nodular Amyloidosis. Chest 1988; 94:438-440
4 Ohdama. Primary diffuse alveolar septal amyloidosis with multiple
cysts and calcification. Eur Respir J 1996; 9:1569-1571
DISCLOSURE: J.S. Wink, None.
AN AFRICAN AMERICAN WOMAN WITH TUBEROUS SCLERO-
SIS COMPLEX AND LYMPHANGIOLEIOMYOMATOSIS: A
RARE CASE
Ripujit K. Gill, DO*; Jeffrey Liu, MD; Feroza Daroowalla, MD. Stony
Brook University, Stony Brook, NY
INTRODUCTION: Only 21 cases of women with tuberous sclerosis
complex (TSC) and lymphangioleiomyomatosis (LAM) are known to the
LAM registry at the National Institutes of Health. Of these cases, only two
are in African American women (1). Due to the small number of
diagnosed cases, there are limited published data estimating the true
incidence or prevalence of the disease. Review of literature revealed no
further data regarding racial distribution.
CASE PRESENTATION: The patient is a 35-year-old African Amer-
ican female who presented with complaints of progressively worsening
dyspnea and back pain for several weeks. There is no family history of
TSC. Remarkable physical findings included fibrous papules on face and
neck. An initial radiograph revealed a pneumothorax involving 50% of
right hemithorax. Blood chemistries and blood counts revealed no abnor-
malities. Urinalysis showed hematuria but no signs of infection. CAT scan
of chest, abdomen and pelvis was performed. Multiple intraparenchymal
lung cysts and bilateral renal masses were noted. The patient underwent
thoracoscopic surgery with mechanical pleurodesis. Lung and renal
biopsies were performed. The right lung was completely re-expanded.
The patient had an uneventful post-operative course and was discharged
home in stable condition.
Lung biopsy was consistent with LAM with bronchoalveolar cell
proliferation as interpreted by the LAM Clinical Center at NIH. Immu-
nochemical stains revealed LAM cells to be positive for smooth muscle
actin and negative for HMB-45. The renal biopsy was consistent with
angiomyolipoma.
DISCUSSION: Sporadic lymphangioleiomyomatosis (S-LAM) is a
non-inheritable disease characterized by peribroncho-vascular, and
perilymphatic proliferation of smooth muscle with loss of lung paren-
chyma leading to cystic lesions. S-LAM occurs exclusively in women of
childbearing age. There are about 400 cases in the US. The radio-
graphic appearance of LAM can range from normal chest radiograph
(early in the disease) to interstitial opacities, honeycomb changes, and
hyperinflation.
TSC is sporadic or inherited as an autosomal dominant syndrome
characterized by hamartomas of skin, eyes, kidney and central nervous
system. It is caused by mutations of tumor suppressor genes TSC1
(9q34.3) and TSC2 (16p13.3). There are an estimated 20-25,000 women
with TSC in the US. Lung involvement in TSC can be cystic (LAM) or
nodular or both. The LAM in many cases of TSC may be asymptomatic
and therefore undetected (1). TSC-LAM and S-LAM are clinically,
histologically, and radiographically similar in nature.
TSC patients with symptomatic LAM present with dyspnea. Onset of
disease may be heralded by a spontaneous pneumothorax, which occurs in
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280S CHEST 2003—Case Reports

approximately one-third of patients. It is characterized pathologically by
the proliferation of atypical pulmonary interstitial smooth muscle and by
cystic changes. Recent research has demonstrated that abnormalities in
synthesis of catecholamines may play a part in the pathophysiology of the
disease.
Symptomatic TSC-LAM carries a poor prognosis, with progressive
disease being common. No effective treatment has been found. However,
treatment with progesterone, luteinizing hormone-releasing hormone
analogs, and/or oophorectomy is recommended. Lung transplantation may
be an option for some patients. Death occurs secondary to respiratory
insufficiency, often within five years of the onset of symptoms.
CONCLUSION: This is a case of symptomatic LAM in an African
American woman with TSC. In data published by the NIH, only 6% of
diagnosed TSC-LAM cases are African American. TSC-LAM is either a
very rare disorder among African American women or it has been
under-diagnosed. Aggressive efforts at case finding would provide a more
accurate assessment of racial distribution and opportunity for early
detection and transplant referral for this fatal disease.
REFERENCE
1. Moss J, et. al Prevalence and clinical characteristics of lymphangio-
leiomyomatosis (LAM) in patients with tuberous sclerosis complex.
AJRCCM 164:669-71
DISCLOSURE: R.K. Gill, None.
Cancer II
4:15 PM - 5:45 PM
A LARGE MEDIASTINAL TUMOR IN A 42-YEAR-OLD MAN
COMPOSED OF FOUR DIFFERENT HISTOLOGIC TYPES: A
CASE REPORT
Everardo Fernandez-Rangel, MD*; Sergio Garza-Salinas, MD; Guada-
lupe E. Cedillo-Salazar, MD; Arturo Carranza-Rebollar, MD; Carlos
Gonzalez-Uscanga, MD. Hospital San Jose-Tec de Monterrey, Monter-
rey, Mexico
INTRODUCTION: Mediastinal germ-cell tumors in adults are un-
usual, representing 2-5% of germ-cell tumors and only 1-10% of all
mediastinal tumors. We report a case of a large mediastinal mixed
germ-cell tumor composed of seminoma, mature teratoma, ganglioneu-
roblastoma and an invasive squamous cell carcinoma. Such an association
is extremely rare in adult patients.
CASE PRESENTATION: A 42-year-old Mexican man with a medical
history of type 2 diabetes, hypertension and occasional tobacco use
presented with chest pain and dyspnea. A chest CT scan showed a large
anterior mediastinal tumor (12.0x9.5x7.0 cm) This finding was confirmed
with a MRI of chest. Theta fraction of human chorionic gonadotropin
(␤-HCG) and alpha-fetoprotein (AFP) were within normal values. The
patient was referred to our hospital for surgical management. Testicular
ultrasound revealed no abnormalities, and staging studies revealed no
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evidence of metastases. The tumor was approached through a median
sternotomy and resected. Histopathological reports of our hospital and
M.D. Anderson Cancer Center reported four different histologic types:
seminoma, teratoma, and an invasive squamous cell carcinoma and a
ganglioneuroblastoma arising from the teratoma. The patient received a
double chemotherapeutic regime consisting of four courses of platinum-
based therapy and etoposide (plus radiotherapy), followed by four courses
of Docetaxel. At all times the ␤-HCG and AFP were within normal values.
Nine months after diagnosis the patient has no signs of recurrence or
metastasis.
DISCUSSION: The sole presence of a primary mediastinal mixed
germ-cell tumor is associated with a poor prognosis, with only 30% of
patients achieving cure. But the presence of multiple histologic types
represented a therapeutic challenge. Prognosis is especially poor for
mediastinal teratomas with malignant transformation and for those with
neural differentiation.
CONCLUSSION: Mediastinal germ-cell tumors in adults are unusual,
and often associated with poor prognosis. The management of mixed
germ-cell tumors of the mediastinum represents a challenge for clinicians.
This case has presented a unique combination of four histologic types that
we believe to be the first described in literature.
DISCLOSURE: E. Fernandez-Rangel, None.
PULMONARY BLASTOMA: A CASE REPORT
Todd M. Henderson, MD*; Octavius Polk, MD; Michael Brancaccio,
MD. Howard University, Silver Spring, MD
INTRODUCTION: Pulmonary blastoma(PB) is a rare primary non-
small cell lung cancer(NSCLC) that is composed of both malignant glands
and mesenchyme that are primitive/embryonal in appearence. It involves
less than 1% of all lung cancers with a frequency about 0.25-0.5%
1
.
CASE PRESENTATION: This is a case of a 57 year-old female that
presented as an outpatient referral for an abnormal chest x-ray(CXR)
during pre-operative surgical clearence for knee arthroscopy. She was
asymptomatic at presentation with no history of tuberculosis exposure or
previous pneumonia. She has a history of asthma/bronchitis requiring
bronchodilator therapy and infrequent courses of oral steroids. There was
a remote history of smoking tobacco for a year during her teens, otherwise
social, occupational, and family history were unremarkable. Physical
examination and laboratory values were normal.
CT revealed a 2.2-cm enhancing soft-tissue mass in the apex of the
RUL with no evidence of mediastinal or pleural disease present. Pulmo-
nary function testing showed a mild obstructive ventilatory defect with
significant reversibility, FEV
1
of 1.84- 68% of predicted. A whole body
positive emission tomography scan(PET) was negative- no focal area of
abnormal radionuclide uptake suggesting a benign process. She had a
RUL lobectomy performed for possible curative treatment without any
complications. Pathological examination of the specimen was positive for
biphasic PB. The surgical margins were clear, and 8 regional lymph nodes
were negative for metastasis. The tumor measured 2.8-cm in diameter
(well-circumscribed, firm, rubbery mass). Follow-up, currently, involves
serial CXR’s every 3 months with a chest CT every 12 months depending
on the results of the CXR’s.
DISCUSSION: PB is a rare NSCLC which morphologically resembles
fetal lung structure. It is subdivided into 2 categories: classic biphasic PB
and well-differentiated fetal adenocarcinoma
2
. They may occur at any age,
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Cancer II, continued
282S CHEST 2003—Case Reports

but biphasic PB tends to occur between 35-40 years of age
3
. There is
usually a history of tobacco use with common symptoms of hemoptysis,
cough, and fever. 25-40% of cases are asymptomatic, usually an incidental
finding
4
. There maybe pleuritic chest pain associated with pleural effu-
sions
5
. Radiographic findings are usually a well-demarcated, peripheral
lesion favoring upper lobes. It can be large, averaging 10-cm (2.5-25-cm)
2
with a rapid growth curve. Biphasic PB has a worse prognosis when
compared to fetal adenocarcinoma due to its higher incidence of metas-
tasis with a survival comparable to common NSCLC’s. The treatment is
similar as well. Surgical resection is the treatment of choice. There are no
other therapeutic or adjuvant guidelines which exist
6
.
CONCLUSION: PET is an accurate, noninvasive means for diagnosis
of pulmonary nodules and larger mass lesions
7
. PB can present as a
pulmonary nodule. In this case of PB, it was a false-negative result via
PET. The lung cancers that are known in literature to cause a false-
negative PET are broncho-alveolar carcinoma and carcinoid tumors.
REFERENCES
1. Hasleton, P.S.: Spencer’s Pathology of the Lung, 5th Edition. New
York, McGraw-Hill, 1996.
2. Zaidi, et al. PB: Medium-term Results from a Regional Center.
Annuals of Thoracic Surgery 2002; 73:1572-1575.
3. Zavala-Alarcon, et al. Extension of a PB into the Left Atrium. Mayo
Clinic Proceedings 2001; 76: 657-660.
4. Chin, et al. PB in an Adult Presenting as a Chronic Loculated
Effusion: a diagnostic problem. Thorax 1994; 49: 838-839.
5. Vassilopoulos, et al. PB Presenting with Massive Hemothorax. Chest
1992; 102: 649-650.
6. Ghaffer, et al. PB: A Report of Two Cases. Chest 1975. 67: 600-602.
7. Gould, et al. Accuracy of PET for Diagnosis of Pulmonary Nodules
and Mass Lesions. JAMA 2001;285(7): 914-924.
DISCLOSURE: T.M. Henderson, None.
Figure 1. Posteroanterior(top) and lateral(bottom) CXR’s demonstrating
an approximately 2-cm RUL nodule.
Figure 2. Chest-CT characterizing RUL lesion, mediastinal(top) and
lung(bottom) windows.
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CHORIOCARCINOMA OF THE PULMONARY ARTERY
TREATED WITH PULMONARY EMBOLECTOMY AND CHE-
MOTHERAPY
Andres Pelaez, MD*; Rade Tomic, MD; Sergio Alvarez, MD; Andres
Palacio, MD. Emory University School of Medicine, Atlanta, GA
INTRODUCTION: Tumors of the pulmonary arteries are often
incorrectly diagnosed as pulmonary thromboembolism, and are thus
seldom diagnosed during a patient’s lifetime. We report a patient with
a choriocarcinoma mimicking pulmonary embolism, who required
pulmonary embolectomy and had remission with chemotherapy there-
after.
CASE PRESENTATION: A 27-year-old woman presented with exer-
tional dyspnea and mild hemoptysis. She had one previous molar preg-
nancy. The initial chest radiographs was unremarkable. She was treated
for bronchitis; however, her symptoms became worse. The subsequent
chest radiographs showed a bilateral diffuse lower lobe infiltrative shad-
ows. On admission, she was hypoxemic with resting O2 sat of 88%. The
chest enhanced CT showed a filling defect at left pulmonary artery (LPA)
[Fig 1]. After negative evaluation for the source for the pulmonary emboli
and multiple ovarian varices, a choriocarcinoma was considered because
her prior molar pregnancy. Pulmonary embolectomy was done. A whitish,
thrombus was found occluding the MPA and extending into the LPA. The
embolus was removed. Pulmonary arterial pressure dropped from 80 to 50
mm Hg immediately after the operation. However, the patient required
ventilator support for subsequent respiratory failure. Microscopically, the
specimens showed “syncytiotrophoblastic cells”. [Fig 2]. After a diagnosis
of pulmonary artery choriocarcinoma was made, a serum HCG values
was ⬎ 80,000 mIU/mL, and a gynecologic examination was performed.
After a 6-month period of chemotherapy, the residual lung tumor
decreased in size and lung fields of the chest radiographs became almost
clear.
DISCUSSION: Choriocarcinoma is a germ cell tumor containing
syncytiotrophoblastic giant cells and often secreting a biological tumor
marker. (␤-HCG). It usually occurs in women. Development of
pulmonary choriocarcinoma can come from retained primordial germ
cells, metastasis from a primary gonadal tumor that regressed sponta-
neously, trophoblastic emboli related to molar pregnancy after long
periods of latency or metaplasia to the trophoblast from a non-gonadal
tissue, such as primary lung cancer. The natural history of untreated
choriocarcinoma is characterized by the development of early hema-
togenous metastases. Some of the vagaries of this tumor have attracted
attention, and failure to find a primary growth in the uterus, even on
careful microscopic examination, is not infrequent. A few cases of
primary choriocarcinoma have been reported
1
. However, this is the
second patient who underwent pulmonary embolectomy and was
treated with subsequent chemotherapy after a confirming pathologic
diagnosis
2
. However, although rare, choriocarcinoma should be con-
sidered in the differential diagnosis of fertile women presenting with
pulmonary embolism or pulmonary artery hypertension. Furthermore,
Savage et al
3
emphasized the importance of considering choriocarci-
noma and measuring serum hCG in the investigation of fertile women
presenting with pulmonary emboli or pulmonary artery hypertension.
In our case, choriocarcinoma was not diagnosed in an early stage.
However, chemotherapy was initiated as soon as the diagnosis was
made and the patient responded positively, with serum ␤- HCG falling
to within normal levels. Our results indicate that although rare,
choriocarcinoma should be considered in the differential diagnosis of
fertile women presenting with pulmonary embolism, because complete
remission can be achieved with appropriate chemotherapy.
CONCLUSION: Choriocarcinoma should be considered in the
differential diagnosis of fertile women presenting with pulmonary
embolism. Due to their rarity, tumors of the pulmonary arteries are
often incorrectly diagnosed as more common diseases, such as pulmo-
nary thromboembolism, and are thus seldom diagnosed during a
patient’s lifetime.
REFERENCES:
1 Graham, JP, Rotman, HH (1976) Tumor emboli presenting as
pulmonary hypertension: a diagnostic dilemma. Chest 69,229-230
2 Shun-ichi Watanabe, et all., (2002) Choriocarcinoma in the Pulmo-
nary Artery Treated With Emergency Pulmonary Embolectomy
Chest 121, 654-656.
3 Savage, P, Roddie, M, Seckl, MJ (1998) A 28-year-old woman with
a pulmonary embolus. Lancet 352,30
DISCLOSURE: A. Pelaez, None.
Figure 3. Low(top) and high(bottom) power magnification of the RUL
specimen.
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284S CHEST 2003—Case Reports

PRIMARY T-CELL LYMPHOMA OF THE LUNG
Petey Laohaburanakit, MD*. University of California, Davis, Sacramento,
CA
INTRODUCTION: Primary lymphoma of the lung is rare and most
are B-cells. We present a case of primary pulmonary T-cell lymphoma
(PTCL).
CASE PRESENTATIONS: 72-year-old female presented with mul-
tilobar pneumonia refractory to antibiotic treatment. Patient under-
went 3 fiberoptic bronchoscopies. Bronchoalveolar lavage (BAL) and
transbronchial biopsy (TBB) showed mixed T lymphocytes, which was
believed to be a reactive process. Right pleural effusion and progres-
sive hilar adenopathy ensued. Transthoracic need biopsy (TNB) of the
right hilum showed atypical T lymphocytes. Monoclonality could not
be established by flow cytometry. Gene rearrangement was unsuccess-
ful due to cell paucity. An open lung biopsy was planned, however,
patient declined and desired to go home. She was readmitted with
respiratory distress, sepsis and later succumbed. Autopsy revealed
PTCL of both lungs and invasive aspergillosis in right upper lobe.
DISCUSSION: Pulmonary lymphoma is a rare entity. Most are of
B-cell origin and have indolent courses. PTCL is found in ⬍ 5% of all
pulmonary lymphomas and portends much worse prognosis. Most patients
present with B symptoms and ’refractory pneumonia’. Chest CT often
shows diffuse air-space disease with lower lobe predilection. Pleural
effusion, hilar adenopathy and cavitary lesion have been reported. BAL,
TBB, and TNB show mixed, atypical T-cells, which are often regarded as
reactive T-cells due to infection. Establishment of monoclonality by flow
cytometry and gene rearrangement is vital to the diagnosis. This almost
always requires large amount of sample via open lung biopsy. Effective
treatment is not known. Most patients succumb within months after
diagnosis.
CONCLUSION: Although rare, PTCL should be considered in pa-
tients who deteriorate despite appropriate treatment and persistently
show T lymphocytosis in lung specimens. Because of its aggressive nature,
timely diagnosis is crucial. Open lung biopsy is often required to establish
monoclonality.
REFERENCES:
1. Tamura A et al. Primary pulmonary lymphoma: relationship be-
tween clinical features and pathologic findings in 24 cases. Jpn J Clin
Onc 1995:25;140-152.
2. Maejima S et al. T-cell non-Hodgkin’s lymphoma of the lung. Int
Med 1993:32;403-407.
DISCLOSURE: P. Laohaburanakit, None.
A HARD PALATE MASS AS THE FIRST MANIFESTATION OF
LUNG ADENOCARCINOMA
Pablo E. Molina, MD*; Juan A. Garcia, MD, FCCP; Steven Im, MD.
University of Texas Health Science Center at San Antonio, San Antonio,
TX
INTRODUCTION: Metastasis within the buccal cavity is an uncom-
mon manifestation of non-head and neck tumors. Hard palate metastasis
often have been associated with melanoma and lymphomas. We present
the first case of a hard palate mass as the first manifestation of lung
adenocarcinoma.
CASE PRESENTATION: A 43 year-old female smoker presented
with an enlarging, intermittently bleeding hard palate mass and 70
pound weight loss over a period of 2 months. She denied pulmonary
symptoms. She had a 25 pack-year tobacco history. Physical examina-
tion was significant for a large mass on her hard palate and decreased
breath sounds of the right lung base. Magnetic resonance imaging of
the oral area revealed a large enhancing mass centered on the right
hard palate with invasion into the right maxillary sinus, pterygopalate
fossa, and the masticator space. Chest computed tomography revealed
narrowing in the right lower lobe bronchus, subcarinal and bilateral
hilar lymphadenopathy, and a right pleural effusion. Pleural effusion
cytology was negative for malignant cells. Bronchoscopy revealed an
endobronchial mass in the superior segment of the right lower lobe.
Transbronchial needle aspirate of the subcarinal area and a hard palate
biopsy of the mass revealed poorly differentiated adenocarcinoma.
Thyroid transcription factor marker (TTF-1) of the hard palate biopsy
was positive indicating that lung adenocarcinoma was the primary
malignancy. She received chemoradiation with partial remission of her
oral obstruction.
DISCUSSION: Lung cancer rarely metastasizes to the hard palate.
Malignant melanoma is the most common malignancy to metastasize to
the hard palate, but has also been reported with other organ malignancies
including renal cell cancer, colon cancer, lymphomas, gastric cancer, small
cell lung cancer and follicular thyroid carcinoma.
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CONCLUSION: Metastasic hard palate lesions are unusual. Lung
cancer should be considered in the differential of patients with hard palate
masses.
DISCLOSURE: P.E. Molina, None.
A MEDIASTINAL ANGIOSARCOMA IN A 36 YEAR OLD MEXI-
CAN MAN: A CASE REPORT
Everardo Fernandez-Rangel, MD*; Sergio Garza-Salinas, MD; Guada-
lupe E. Cedillo-Salazar, MD; Arturo Carranza-Rebollar, MD; Carlos
Gonzalez-Uscanga, MD. Affiliate, Hospital San Jose-Tec de Monterrey,
Monterrey, Mexico
INTRODUCTION: Mesenchymal tumors of the mediastinum account
for 6% of all mediastinal tumors. Lesions of vascular origin may arise
anywhere in the mediastinum and are extremely rare. Angiosarcomas may
exhibit a variety of histologic appearances including carcinoma, other
sarcomas, melanoma, and even benign reactive processes. We describe a
case.
CASE PRESENTATION: A 36-year-old man with medical history
of peptic ulcer disease presented to the emergency department with
chest pain and dyspnea. Four months before he had developed
postprandial epigastric pain, with no adrenergic symptoms associated.
The pain was not associated with breathing or physical activity but
worsened on prone or right lateral decubitus position. He denied
weight loss or fever, and was in general good condition. On admission
a chest CT scan was done, revealing a large mass located at the anterior
mediastinum and pericardial effusion. Alpha-fetoprotein and beta
fraction of chorionic gonadotrophin were normal. Testicular ultra-
sound was normal and staging studies revealed no evidence of
metastases. The tumor was biopsied via a median sternotomy, but it
could not be resected because it involved pericardium and great
vessels. Transoperatory biopsy reported a myxoid sarcoma. Definitive
histopathological report (M.D. Anderson Cancer Center) reported a
high-grade angiosarcoma. The patient received neoadjuvant chemo-
therapy with four courses of etoposide and carboplatinum, achieving a
reduction of 80% of the tumoral size after the third chemotherapy
course.
DISCUSSION: Angiosarcomas may arise anywhere in the mediasti-
num and are extremely rare. They usually have direct extension of tumor
into adjacent structures, and local recurrence is common. Complete
resection may be difficult but is usually curative. In this case, direct
infiltration of mediastinal vascular structures and pericardium prevented
resection.
CONCLUSION: Clinicians should be aware and include in the
differential diagnosis of a mediastinal mass those tumors of vascular origin,
even if they are rare. Given the unusual presentation of mediastinal
angiosarcomas this case represented a clinical and therapeutic challenge.
Four sessions of chemotherapy with etoposide and carboplatinum were
given with good clinical response.
DISCLOSURE: E. Fernandez-Rangel, None.
Metastatic Cancer
4:15 PM - 5:45 PM
AN INTERVENTIONAL APPROACH TO PULMONARY METAS-
TASIS OF COLON CANCER: A CASE REPORT
Mandeep S. Bakshi, MBBS, MD*; Ron R. Allison, MD; Rosa E. Cuenca,
MD; Gordon H. Downie, MD, PhD. East Carolina University School of
Medicine, Greenville, NC
INTRODUCTION: Direct endobronchial metastases from non-
pulmonary primaries are unusual, with a range of presentations. Of
non-pulmonary sites of origin, colon, renal and breast are the most
frequent. Colon cancer spreads by lymphatic and hematogenous
dissemination to regional lymph nodes, liver and lungs. These lesions
usually appear remote from the primary occurrence (⬎5 years).
Treatments for endobronchial colon metastases are dependent on the
tumors behavior and responsiveness to the palliative measures. Long-
term survival with local regional control of the metastases has been
reported, although mean survival is short. We describe a case of
Gardner’s syndrome with subsequent endobronchial metastases caus-
ing respiratory insufficiency.
CASE REPORT: A 26-year-old female with Gardner’s syndrome,
s/p colectomy with ileocecal anastomosis in 1997 presented in 8/02
with respiratory insufficiency. A CT scan showed collapse of left lung
secondary to solitary endobronchial metastases involving the left main
stem bronchus. Patient underwent bronchoscopy with balloon dilata-
tion and stent placement at this location. She had a dramatic response
to intervention with post-procedure CT scan demonstrating complete
expansion of left lung. Rapid tumor in growth through the stent
required four weekly sessions of intraluminal high dose brachytherapy
(500 cGy) followed by a session of intraluminal photodynamic therapy
(PDT). Patient’s functional status improved post consolidation photo-
dynamic therapy, and she has remained disease free at this site 6
months out.
DISCUSSION: Direct endobronchial metastasis from extrapulmonary
primaries is a rare occurrence. Interventional bronchoscopy with stent
placement improved the clinical status of the patient. Restored lumen
allowed the patient to undergo brachytherapy and photodynamic therapy.
Stent placement with brachytherapy and PDT provided local regional
disease control without significant side effects.
CONCLUSION: Based on our experience with this case: brachyther-
apy and PDT is safe, and may extend survival with local regional control
in this subgroup of patients with endobronchial metastasis.
DISCLOSURE: M.S. Bakshi, None.
PULMONARY TUMOR EMBOLISM PRESENTING AS A COM-
PLICATION OF ADENOID CYSTIC CARCINOMA
James A. Graham, MD*; Jeffrey G. Walls, MD; Joanna D. Lusk, MD;
Lorine M. LaGatta, MD; Brian S. Kendall, MD. Affiliate, Wilford Hall
Medical Center, Lackland AFB, TX
INTRODUCTION: Pulmonary tumor embolism occurs most com-
monly as a complication of breast, lung, and prostate carcinoma. An
incidence rate of 0.9% to 8% has been reported; however, the true
incidence and contribution to mortality of this entity is likely unknown.
We report a case of myocardial infarction and cardiac arrest due to
pulmonary and cardiac vessel tumor embolism from an adenoid cystic
carcinoma.
CASE PRESENTATIONS: A 50-year-old male with adenoid cystic
carcinoma presented complaining of progressive dyspnea on exertion. His
oxyhemoglobin saturation was 74% and his chest exam was normal. Sinus
tachycardia with lateral ST depression was noted and cardiac enzymes
were mildly elevated. The chest radiograph and heart catheterization were
normal. CT-angiogram and pulmonary arteriogram were negative for
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286S CHEST 2003—Case Reports

pulmonary embolism, and the pulmonary artery pressure was 54/21. The
patient became asystolic and expired 14 hours after intubation for
refractory hypoxia. Microscopic examination of the lung and heart noted
widespread occlusion of pulmonary arteries, arterioles, capillaries (Figure
1) and myocardial arterioles (Figure 2) with tumor emboli. Acute
myocardial ischemic changes were seen in the left ventricle and left
papillary muscle, with subacute changes noted in the interventricular
septum. Special stains of the tumor emboli matched the staining pattern
of the original tumor.
DISCUSSIONS: Pulmonary tumor embolism has been reported as a
complication of multiple malignancies, but only once before when
associated with adenoid cystic carcinoma. The presentation of this patient
was typical for this entity, with subacute onset of dyspnea in the setting of
a known malignancy. Radiographic studies are generally unrevealing, as
was the case here.
CONCLUSIONS: This case highlights that a high index of suspicion is
required to make this diagnosis and possibly spare unnecessary anticoag-
ulation. Chemotherapy, though not prospectively studied in this setting
for any malignancy, may offer benefit in select patients.
DISCLOSURE: J.A. Graham, None.
BILATERAL PULMONARY NODULES SECONDARY TO
LEIOMYOMATOSIS
Glena Cheng, MD*; Janine E. Vintch, MD. Harbor-UCLA Medical
Center, Torrance, CA
INTRODUCTION: While most patients with widespread parenchy-
mal lung metastases are symptomatic, the following case report describes
a relatively asymptomatic individual with metastases arising from a
primary uterine leiomyoma.
CASE PRESENTATION: The patient is a 48 year old female with
type 2 diabetes mellitus who was referred from an outside clinic for
further work-up of a cough, positive tuberculin skin test and abnormal
chest x-ray (CXR). She denied any associated symptoms, recent travel, or
ill contacts.
The patient had a history of menorrhagia that resolved after a total
abdominal hysterectomy five years ago. She was taking an oral hypogly-
cemic and denied any drug allergies, bad habits, or significant family
history. She immigrated from Mexico ten years ago and has lived in Los
Angeles since.
The patient was afebrile and hemodynamically stable. She was a well
appearing, mildly obese female with normal cardiovascular and pulmonary
exams. She had an abdominal surgical scar from the prior hysterectomy.
Laboratory studies were only remarkable for a mild peripheral eosino-
philia and transaminitis. The patient had three negative sputum samples
for acid-fast bacilli.
The CXR revealed multiple pulmonary nodules bilaterally of various
sizes. Computerized tomography (CT) scan of the chest demonstrated
multiple well circumscribed pulmonary masses/nodules in bilateral
lung fields, suggesting metastatic disease. The largest lung nodule
measured 7.0 cm x 3.5 cm x 5.5 cm in the right lower lobe; no
mediastinal or hilar adenopathy was present. The patient had normal
spirometry.
The CT guided biopsy of the right lower lobe lung mass revealed a
smooth muscle tumor with benign features: estrogen receptor (ER) and
progesterone receptor (PR) were positive and a low mitotic index was
present. The prior hysterectomy specimen demonstrated the presence of
intravenous leiomyomatosis.
DISCUSSION: Three types of smooth muscle tumors exist in
Martin
1
’s classification system: leiomyomatosis, metastatic leiomyoma,
and multiple fibroleiomyomatous hamartomas. Leiomyomatosis occurs
exclusively in women with uterine leiomyomas and is further subdivided
into benign metastasizing leiomyoma, lymphangiomyomatosis (lym-
phangioleiomyomatosis), leiomyomatosis peritoneali disseminata, and in-
travenous leiomyomatosis.
Birch-Hirschfield
2
describes intravenous leiomyomatosis as a uterine
tumor with benign-appearing smooth muscle within veins or lymphatics
which can enter the inferior vena cava, heart, and lung. Benign metasta-
sizing leiomyoma is described by Steiner
3
and others as a well-differen-
tiated tumor composed of smooth muscle cells and dense connective
tissue which can also travel to the lung. Canzonieri
4
believes the two are
“the same pathological entity with different clinical features.”
In other case reports of pulmonary metastases secondary to leiomyomato-
sis, patients often have no pulmonary symptoms and have no-
dules detected incidentally. Martin
1
and Matsumoto
5
report two such cases.
The prognosis of leiomyomatosis with pulmonary metastases is good as
patients often live years after initial diagnoses, as can be seen from Table
1. One prognostic factor is the patient’s hormonal status. Idelson
10
and
Horstmann
11
found that postmenopausal women and women in later
stages of pregnancy do better than their counterparts.
Table 1. Characteristics of Patients with Leiomyomatosis and
Pulmonary Metastases
Author
Age of Pt
(yrs) at
time pulm
mets*
detected
Time of
hysterectomy
(years
prior
to
detection
of pulm
mets)
Treatment
of pulm
mets Follow-up
Martin
1
(1983)
46 14 Bilateral BSO Alive at 9
months
Mulvany
9
(1994)
28 3 Resection of lung
mass
Alive, 5 yrs
post-op
Canzonieri
4
(1994)
(Case 1)
46 6 Resection of
recurrent pelvic
mass
Alive, 3 yrs
post-op
Canzonieri
4
(1994)
(Case 2)
46 0 Resection of RML
for increase in size
of pulm mets 20
years after initial
detection of pulm
mets
Alive 3 yrs post-
op with slight
enlargement of
pulm nodules
Clement
12
(1998)
(Case 2)
44 8 Unknown Alive 7 yrs after
detection lung
mets
Kullo
7
(1999)
52 12
(concurrent
diagnosis:
mass in
IVC/RA)
Megestrol acetate Alive at 6 yrs
Matsumoto
5
(2001)
58 0 TAH/BSO
Chemo
Thoracotomy five
yrs later for
increasing pulm
mets
Unknown
Cheng
(2003)
48 5 None Alive at 2
months after
detection of
lung mets
*pulm mets: pulmonary metastases
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There are no standard treatment regimens for this disease. It is
generally accepted that hysterectomy and bilateral salpingo-oophorec-
tomy be performed to remove the primary tumor and decrease estrogen
and progesterone levels. Martin
1
and Matsumoto
5
found that the pulmo-
nary lesions remained stable in size after hysterectomy/bilateral salpingo-
oophorectomy. Kullo
7
report using megestrol as a hormonal agent to treat
these hormone sensitive tumors. Surgical resection is feasible if the
pulmonary lesions are localized.
CONCLUSION: Leiomyomatosis can metastasize and cause asymp-
tomatic bilateral pulmonary nodules. Although the prognosis of patients
with lung involvement is good, these individuals should be followed
closely to detect changes in symptoms, radiologic findings, or pulmonary
function tests as significant changes may warrant treatment.
DISCLOSURE: G. Cheng, None.
METASTATIC MALIGNANT MELANOMA: A RARE MANIFES-
TATION
Majid M. Mughal, MD*; Carol Farver, MD; Omar A. Minai, MD.
Cleveland Clinic Foundation, Cleveland, OH
INTRODUCTION: Klippel Trenaunay (KT) syndrome is a rare,
sporadic, congenital vascular disease of unknown etiology. We describe a
45 year old male patient with KT syndrome presenting with exertional
dyspnea, interstitial infiltrates, and severe restriction due to metastatic
malignant melanoma from an unknown primary.
CASE PRESENTATION: A 45-year-old white male was seen in the
clinic for increasing dyspnea for the last 6 months. He had history of KT
syndrome and had resection of a large left neck and shoulder cystic
hygroma at the age of 10 days followed by radiation therapy when he was
4 years old. Two years back, he had a basal cell carcinoma removed from
the left scapular region.
The patient had been well until 6 months ago, when he started feeling
shortness of breath on exertion. He was unable to exercise the same way
he used to do. During the next several weeks, the dyspnea progressed over
the next several weeks and was accompanied by non-productive cough or
low-grade fever. He denied weight loss, night sweats, chest pain, hemop-
tysis, and lower extremity edema. He had been treated with Azithromycin,
Levaquin, and a 2 weeks course of Itraconazole and prednisone with
minimal improvement. There was no history of asthma, chronic bronchi-
tis, smoking, or use of oxygen at home.
The temperature was 37.2°C, the pulse was 100, and the respirations
were 20 with 94% oxygen saturation on room air. The blood pressure was
100/60 mm Hg. General physical examination revealed extensive skeletal
deformities with hard swelling at the posterior aspect of the left scapula.
Chest auscultation revealed bibasilar crackles with no wheezes or pleural
rub.
X-ray chest showed bilateral extensive reticulonodular infiltrates. There
was a left apical pleural thickening with loss of volume in the left upper
lung. There was deformity/erosion of the left scapula at the region of the
glenoid fossa. Computed tomographic (CT) examination of the chest
revealed bilateral reticulo-nodular infiltrates more prominent in the
mid-lower lung zone. No lymphadenopathy or pleural deformities were
noted.
Spirometry revealed severe restriction with FVC of 1.75 (45% pre-
dicted) and FEV1 of 1.05(48% predicted) which was a significant change
from previous spirometry performed 2 years ago, when FVC was 3.15
(81% predicted) and FEV1 of 2.59 (78% predicted).
Bronchoscopy was performed which revealed normal airways and no
endobronchial lesions. Bronchoalveolar lavage and transbronchial biopsies
from the right lung were obtained. Microscopical examination of the
specimen revealed large epitheloid cells with eosinophilic cytoplasm and
nuclei with scattered intranuclear inclusions. Immunohistochemical stud-
ies revealed these cells are immunoreactive to S-100 and focally immu-
noreactiveto HMB-45. This pattern was felt to be metastatic malignant
melanoma of the lung.
DISCUSSION: To our knowledge, this is the first report of a
metastatic malignant melanoma presenting as an interstitial lung disease
with severe restriction. Malignant melanoma most commonly presents as
a primary neoplasm of the skin but has been described in such mucosal
sites as the oral cavity, esophagus, larynx, vagina, and anorectal region.
Primary malignant melanoma arising in the lung has been described but
is a rare entity. Malignant melanomas arising in extra-pulmonary sites may
metastasize to the lungs with the most common manifestations being
solitary or multiple pulmonary nodules. The present case revealed no
evidence of an extrapulmonary primary melanoma during the clinical
examination and there was no past history of melanoma. Spontaneous
regression of the skin melanomas after regional lymph node metastases
have been occurred and is well documented and it is possible in our case.
CONCLUSION: We report the first case of metastatic malignant
melanoma presenting as diffuse reticulonodular infiltrates on chest radio-
graphs and a severe restrictive ventilatory defect on spirometry.
DISCLOSURE: M.M. Mughal, None.
RECURRENT MELANOMA PRESENTING AS PLEURAL ME-
TASTASES FOURTEEN YEARS AFTER INITIAL CUTANEOUS
PRESENTATION
Andre´ D. Sotelo, MD*; Joseph Cicenia, MD, FCCP; Patricia A. Tietjen,
MD, FCCP. Saint Vincent Catholic Medical Centers, New York, NY
INTRODUCTION: Although visceral metastases of malignant mela-
noma are common, pleural metastases without pulmonary parenchymal
metastatic involvement is rare. Here we describe a case of recurrent
disease that presented as a large pleural effusion without concomitant
lung involvement.
CASE PRESENTATION: An otherwise healthy 37 year-old fair-
skinned man presented with progressive dyspnea. He had a history of a
single scalp melanoma resected fourteen years prior with no complications
or known spread. He did not receive adjuvant chemotherapy. On exam, he
appeared slightly uncomfortable with normal vital signs and an SpO
2
of
96% on 2 liters/min nasal cannulaO
2
. His left hemithorax was dull and
without breath sounds. The remainder of his exam was normal. His
radiograph revealed left hemithoracic opacification with contralateral
mediastinal shift. Thoracentesis revealed a lymphocytic exudate with
negative cultures and cytology. Pleural biopsy was non-diagnostic of
microbes or tumor. One month later he again required thoracentesis of a
lymphocytic exudate for symptoms of dyspnea. CT scan of the chest
showed nodules lining the left parietal and visceral pleura and enlarged
left hilar and subcarinal lymph nodes. PET scan of the chest demonstrated
increased radiotracer uptake consistent with metastatic spread in all of
these areas with no uptake in the lung itself. Video-assisted thoracoscopic
exploration showed the pleural surfaces to be studded with tumor. Biopsy
of this tissue revealed melanoma. Skin survey did not reveal any new nevi
or lesions.
DISCUSSION: Malignant exudative pleural effusions are com-
monly found in lung and breast carcinomas and in lymphoma.
Melanoma is known to easily metastasize to the lung parenchyma
through hematogenous and lymphangitic spread. These cases can be
associated with lymphocytic malignant pleural effusions. However,
effusions associated with isolated pleural metastases are rare, espe-
cially so long after primary diagnosis. The ipsilateral lymphadenopathy
without radiotracer uptake in the lung in this case is consistent with
pleural lymphatic drainage without concomitant pulmonary parenchy-
mal spread.
CONCLUSION: Metastatic melanoma is an extremely difficult disease
to detect, partly because visceral metastatic spread does not manifest itself
early. It is extremely challenging to treat because it is strongly resistant to
chemotherapy and radiation. In this case recurrent metastatic melanoma
to the pleura resulted in a massive exudative pleural effusion in an unusual
Tuesday, October 28, 2003
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288S CHEST 2003—Case Reports

manifestation of the disease. The long period of time between initial
diagnosis and the presentation of metastatic symptoms in this case
underlines the difficulty in detection and management of this disease.
DISCLOSURE: A.D. Sotelo, None.
CHYLOTHORAX AND STERNAL DESTRUCTION SECONDARY
TO LYMPHANGIOMA (GORHAM’S SYNDROME)
Eduardo Velez, MD*; Robert Moses, MD; Paul K. Deranien, MD;
Bartolome Celli, MD; Frank Chong, MD; Anneliese Gonzalez, MD;
Naim Aoun, MD. St. Elizabeth’s Medical Center, Boston, MA
INTRODUCTION: Neoplasms of lymphatic vessels have varied pre-
sentations; a case of lymphangioma causing chylothorax and bone destruc-
tion, known as Gorham’s syndrome, will be discussed.
CASE PRESENTATION: 22 year-old male with 8 months of progres-
sive retrosternal discomfort and dry cough. He had no medical or surgical
problems, or allergies. Denied toxic exposures, chest trauma or weight
loss.
He was well-built, hemodinamically stable and afebrile. Decreased
breath sounds were noted in the right hemithorax; the sternum appeared
to sink on inspiration. Routine labs were normal; chest x-ray revealed a
large right-sided pleural effussion. Thoracentesis yielded four liters of
brown fluid; analysis showed normal lactic dehydrogenase and protein, no
organisms, negative culture and cytology, and a triglyceride level of 1450.
Tuberculin test, autoimmune workup, and human immunodeficiency
virus assay were negative. Galium scan, blood flow cytommetry, and bone
marrow biopsy were normal. Thoracoscopy was unremarkable. A lym-
phatic scan showed no activity in the area of thoracic duct.
Chest CT scan (fig.1) showed irregularity and heterogeneity of the
sternum. Sternal biopsy revealed destruction of the manubrium, and
replacement by a fibrous mass. Pathology revealed sponge-like channels
lined by factor VIII (⫹) endothelial cells (fig.2) and destruction of
adjacent bone, compatible with lymphangioma.
Thoracic duct ligation and pleurectomy were done, after which the
chylothorax disappeared. Outpatient radiotherapy was started, with good
results.
DISCUSSION: Trauma and neoplasms are the most frequent causes
of chylothorax (1-2).
Lymphangiomas typically occur in children, but may develop at any age.
Symptoms arise from compression of adjacent structures (3, 4).
Radiologically, lymphangiomas appear as cystic masses; mediastinal
widening and pleural effussions are common (5). Lymphangiogram may
show lesions of the thoracic duct and dylated lymphatics (6).
Histologically, there are large, irregular vascular spaces lined by
well-differentiated endothelial cells (7) that stain positive for Factor VIII
and CD31 (8).
The association between localized bone destruction and chylothorax
with lymphangiomas was described by Gorham and Stout in 1955 (9).
Surgical exploration and resection, with low, right-sided ligation of the
thoracic duct and pleurodesis is recommended. Treatment of bone
destruction consists of resection, bone implants, and/or radiotherapy (4).
Adjuvant use of clodronate and alfa 2b interferon has been reported (10).
CONCLUSION: Chylothorax in association with localized bone de-
struction suggests the presence of lymphangioma. The manifestations of
these tumors will depend on the structures affected. Multiple therapeutic
interventions are recommended.
REFERENCES:
1) Cohen et.al.The Pleura. In Sabiston & Spencer’s Surgery of the
Chest, 6
th
edition. W.B. Saunders Co. Philadelphia, 1995
2) Light, Richard W.: Physiology of Pleural Fluid Production and
Benign Pleural Effussion. In General Thoracic Surgery, 5
th
edition.
Lippincott, Williams & Wilkins, Philadelphia, 2000, 687-698
3) Faul, John l. et. al. Thoracic Lymphangiomas, Lymphangiectasis,
Lymphangiomatosis, and Lymphatic Dysplasia Syndrome. Am J.
Respir, Crit. Care Med, 2000, 161; 1037-1046
4) Tie, Mark L.H. et. al. Chylothorax in Gorham’s Syndrome. A
Common Complication of a Rare Disease. Chest, January 1994, 105;
1: 208-213
5) Fraser & Pare. Diagnosis of Diseases of the chest; 4
th
edition, vol. 4,
W.B. Saunders & Co., 1999, p. 2921-2925.
6) Jang, H.J. et. al. Intravascular Lymphomatosis of the Lung: Radio-
logic Findings. J Comput Assist Tomogr, 1998, 22: 427-429.
7) Carrie Y. Innwards, K. Krishtan Unni, in: Diagnostic Surgical
Pathology, 3
rd
edition, Lippincott, Williams & Wilkins, Philadelphia,
1999, p. 294, 1153.
8) Hillerdal, G. Chylothorax and Pseudochylothorax. Eur Respir J.
1997, 10: 1157-1152.
9) Gorham, L.W. et. al. Massive Osteolysis: its Relation to Hemangi-
omatosis. J Bone Joint Surg (Am), 1955; 37: 985-1004.
10) Hagberg, H. et. al. Alpha-2b Interferon and Oral Clodronate for
Gorham’s Disease. Lancet, 1997; 350: 1822-1823.
DISCLOSURE: E. Velez, None.
Interventional Pulmonology
4:15 PM - 5:45 PM
COMPLETE RESECTION OF ENDOBRONCHIAL HAMAR-
TOMA BY ELECTROCAUTERY AND ARGON PLASMA ABLA-
TION VIA FIBEROPTIC BRONCHOSCOPY
Joseph B. Jura, MD*; Thomas A. Dillard, MD FCCP. Medical College of
Georgia, Augusta, GA
INTRODUCTION: Endobronchial hamartoma is a rare tumor which
may present with bronchial obstruction
1
. We present a case of endobron-
chial hamartoma that was completely resected and ablated with electro-
cautery and argon plasma coagulation by fiberoptic bronchoscopy.
CASE PRESENTATION: A 63 year old white male initially presented
to the pulmonary clinic with progressive dyspnea on exertion and chest
pain. A CT scan of the chest revealed a filling defect in the proximal left
lower lobe bronchus with partial atelectasis of the posterior medial
segment of the left lower lobe. An initial bronchoscopy showed a large
polypoid lesion within the left mainstem bronchus arising from the origin
of the left lower lobe. Biopsy specimens revealed benign polyp. Interven-
tional bronchoscopy with loop electrocautery resected the polyp in
approximately four pieces followed by argon plasma ablation of the base.
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Pathology of the resected lesion diagnosed bronchial hamartoma with
lipomatous component. The patient’s dyspnea resolved. Repeat bronchos-
copy 2 months later revealed patency of all airways with no residual tumor.
DISCUSSION: Endobronchial tumors may present with obstruction
of a bronchus and resultant atelectasis and/or bronchiectasis. Pulmonary
hamartomas are benign, and rarely recur after complete surgical excision
1
.
Surgical resection has been recommended for these patients, but may
involve the surgical risks inherent to a thoracotomy. Nd-YAG Laser
resection by flexible bronchoscopy has been attempted
2
. Our case dem-
onstrates complete resection of an endobronchial hamartoma by electro-
cautery with argon plasma coagulation.
CONCLUSIONS: Electrocautery and argon plasma coagulation may
represent an alternative therapy for resection of selected benign endo-
bronchial tumors.
REFERENCES:
1. Gjevre JA, Myers JL, Prakash UBS. Pulmonary hamartomas. Mayo
Clin Proc 1996; 71:14-20
2. Stey CA, Vogt P, Russi EW. Endobronchial lipomatous hamartoma.
Chest 1998; 113:254-255
DISCLOSURE: J.B. Jura, None.
UTILIZATION OF A SYNTHETIC ABSORBABLE SEALANT AC-
TIVATED BY HELIUM CADMIUM LASER BRONCHOSCOPY
FOR SUCCESSFUL CLOSURE OF A PERSISTENT BRONCHO-
PLEURAL FISTULA FOLLOWING LOBECTOMY
Darren S. Hoffberger, DO*; Frank Walsh, MD; Eric Sommers, MD;
Mark Rolfe, MD. University of South Florida, Wesley Chapel, FL
INTRODUCTION: A bronchopleural fistula (BPF) may be one of
the most disappointing complications of pneumonectomy or lobec-
tomy. The clinical sequela may include incomplete lung expansion,
empyema, prolonged ventilator support, and often gas exchange
problems in addition to increased morbidity and mortality. Numerous
modalities using chemical agents and mechanical devices have been
reported for fistula closure.
CASE PRESENTATION: A 72-year-old female underwent left upper
lobectomy for squamous cell carcinoma. Following prolonged air leak, a
flexible fiberoptic bronchoscopy was performed which revealed the
presence of a 4mm dehiscence in the bronchial closure representing a
bronchopleural fistula. Upon repeat bronchoscopy, FocalSeal-L®,a
synthetic absorbable sealant, was instilled under direct visualization
through a bronchoscope, and activated using a helium cadmium laser.
DISCUSSION: FocalSeal-L®, a water-soluble polyethylene glycol-
based gel, comes as a polymer and sealant activated by a xenon-generated
light wand. The wand provides light in the spectrum of 440nm to 550nm.
Intra-operatively, the wand is to be held for 45 seconds about 2cm from
the area in which the sealant and polymer has been applied. Crosslinking
of the compounds then occurs. Currently FocalSeal-L® has an indication
for the use as adjunct to standard closure of visceral pleural air leaks
incurred during elective pulmonary resection. The LIFE system, which is
used for autofluorescence bronchoscopy, emits blue light from a helium
cadmium laser at a wavelength of 442 nm with 150mW of output.
Image 1. 4mm bronchopleural fistula
Image 2. BPF following instillation of polymer and sealant
Image 3. Helium cadmium laser light source applied (442 nm wave-
length) to photoinitiate activation
Tuesday, October 28, 2003
Interventional Pulmonology, continued
290S CHEST 2003—Case Reports

Therefore we used this system as our light source to bronchoscopically
photoinitiate activation of the compound
CONCLUSION: We believe this is the first report of the use of
bronchoscopically delivered FocalSeal-L® in conjunction with LIFE light
activation for the repair of a bronchopleural fistula. This technique may
provide physicians treating patients with BPFs another non-surgical
approach.
DISCLOSURE: D.S. Hoffberger, None.
INTERVENTIONAL ENDOSCOPIC MODALITIES FOR PRI-
MARY TRACHEAL MALIGNANCY: PHOTODYNAMIC THER-
APY AND TEMPORARY STENTING
Samer A. Kseibi, MD*; Carter J. Childs, MD; Ron R. Allison, MD; Rosa
E. Cuenca, MD; Gordon H. Downie, MD., PhD. Brody School of
Medicine at East Carolina University, Greenville, NC
INTRODUCTION: Primary malignant neoplasms of the trachea are
rare, accounting for 0.1% of all malignancies. Primary resection and
reconstruction of the trachea with post-operative radiation offers in-
creased survival. Interventional endoscopy provides an important alterna-
tive to surgery in selected patients. We describe a patient with a
life-threatening tracheal obstruction from a primary tracheal malignancy
that was successfully palliated and treated with interventional endoscopy
including temporary stenting with self-expanding metallic nitinol stent
(SEMS) and photodynamic therapy (PDT).
CASE PRESENTATION: A 52-year-old male presented with a
6-month history of intermittent cough and wheezing. Despite treatment
he became increasingly dyspneic with severe respiratory distress and was
diagnosed with a near-complete occlusion of the distal trachea from a
primary squamous cell tracheal malignancy, (cT4N0M0) with extension to
right and left main stem bronchi. Endoluminal core-out was attempted
using electrocautery and high dose brachytherapy with unsatisfactory
response. A SEMS was deployed followed by external beam radiation
therapy (EBRT). PDT and cryotherapy to salvage necrotic tissue consol-
idated treatment. The SEMS was used as temporary bridge to therapy and
was removed post-PDT without complications. This treatment achieved
complete response; biopsies showed necrotic neoplasm. Our patient’s
quality of life improved markedly by both Karnofsky performance scale
and dyspnea index.
DISCUSSION: Tracheal malignancies present diagnostic and thera-
peutic challenges. Surgical resection is the preferred therapy but it carries
a high mortality rate (10-15%). PDT provides a minimally invasive local
treatment with the potential use as curative modality. SEMS has excellent
characteristics but are permanent because of rapid neoepithelization.
Tissue necrosis secondary to PDT enabled us to extract the SEMS without
complications.
CONCLUSION: This case shows the important palliative and thera-
peutic role of interventional endoscopy. PDT achieved an excellent
therapeutic response and in association with EBRT it offered an alterna-
tive modality sparing the patient from the morbidity and mortality
associated with surgery.
DISCLOSURE: S.A. Kseibi, None.
SUCCESSFUL TREATMENT OF AN ESOPHAGO-BRONCHIAL
FISTULA USING A COVERED, SELF-EXPANDING METAL
STENT IN THE AIRWAY AFTER INABILITY TO PLACE AN
ESOPHAGEAL STENT IN A PATIENT WITH ESOPHAGEAL
CARCINOMA
Jeff B. Hales, MD*; Rex C. W. Yung, MD. Johns Hopkins University,
Baltimore, MD
INTRODUCTION: The incidence of esophageal carcinoma in the
United States is estimated at 3.2 / 100,000 (1). With tumor involvment of
the proximal esophagus, bronchoscopy is recommended, as case series
have shown that esophago-bronchial fistulae will be present approximately
5% of the time (2,3). Additionally, fistulae will occur in up to 12 % of
patients at some point during treatment (1,2). If esophageal-bronchial
fistulae develop, the patient will suffer with continuous coughing, exhaus-
tion, and recurrent aspiration pneumonia. Without sealing of the fistula
tract, death can frequently be expected within several days time (4).
CASE PRESENTATION: A 62 year old man diagnosed with locally
advanced esophageal cancer had been treated with radiation therapy and
successive cycles of chemotherapy, including carboplatin and 5-fluorou-
racil followed by taxotere. Because of progressive dysphagia, he then
received esophageal dilation treatments. For two weeks prior to presen-
tation he suffered from persistent cough, worse with swallowing, and was
maintained on antibiotics for aspiration pneumonia. A cine-esophogram
revealed 1.2 cm fistula tract to the left mainstem bronchus. Attempted
esophageal stent placement was unsuccessful because of inability to pass
the endoscope due to high-grade obstruction and non-localization of the
fistula. After the patient swallowed 10 cc of methylene blue dye, fiberoptic
bronchoscopy was performed under conscious sedation. The fistula was
easily located in the posterior wall of the left mainstem bronchus
approximately 2 cm from the main carina. After estimation of airway
diameter and lesion length, a self-expanding, covered nitinol stent (14 mm
diameterx6cmlength) was placed under direct visualization. The
patient’s symptoms of cough and sputum production resolved, and with
repeated esophageal dilations he has been able to enjoy eating a regular
diet and attending a family vacation. A recent, 2-month follow-up visit
confirmed continued response.
DISCUSSION: Esophageal cancer usually presents in an advanced
and incurable stage with a poor prognosis—the 5-year survival rate
remains dismally low, around 5% (1). A major goal of therapy is
maintaining a patient’s ability to swallow thus preserving quality of life.
Combined radiation and chemotherapy are frequently employed treat-
ment regimens, with locally delivered palliative measures used as neces-
sary (1). When esphago-bronchial fistulae occur, they signal an ominous
prognostic sign and are challenging to manage. Treatment of these fistulae
in the past has focused on esophageal stenting; however, acute, total
airway obstruction as a complication from posterior membrane compres-
sion has occurred (5,6). Therefore, for patient safety, a dual stenting
approach should always be attempted. Combined airway and esophageal
stenting has been evaluated using the silicone and dynamic Y-stent models
in the tracheobronchial tree (4,5). The increased radial force of self-
expanding metal stents, may help seal the fistula tract and improve
outcomes in these patients.
CONCLUSION: Airway covered, self-expandable metal stents can seal
esophago-bronchial fistula and prevent persistent aspiration by them-
selves. This should always be considered as a combined procedure with
esophageal stenting to prevent airway occlusion, and as a single procedure
if the esophageal stent cannot be placed.
REFERENCES:
(1) Daly JM, et.al. National Cancer Data Base reports on esophageal
carcinoma. Cancer 1996; 78: 1820-8.
Image 4. BPF following activation
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(2) Muto M, et.al. Concurrent chemoradiotherapy for esophageal car-
cinoma patients with malignant fistulae. Cancer 1999; 86: 1406.
(3) Bolton JS, et.al. Esophageal resection for cancer. Surg Clin North
Am 1998; 78: 773.
(4) Freitag L, et.al. Management of Malignant Esophagotracheal Fis-
tulas with Airway Stenting and Double Stenting. Chest 1996; 110:
1155-60.
(5) Colt HG, et.al. Double Stents for Carcinoma of the Esophagus
involving the Tracheobronchial Tree. Gastroint Endo, 1992; 38:
485-9.
(6) Raijman I, et.al. Palliation of malignant dysphagia and fistulae with
coated expandable metal stents. Gastrointest Endosc 1998; 48: 172.
DISCLOSURE: J.B. Hales, None.
SUCCESSFUL CLOSURE OF A BRONCHOPLEURAL FISTULA
WITH PLATINUM VASCULAR OCCLUSION COILS AND N-
BUTYL-CYANOACRYLATE GLUE
Shaheen U. Islam, MD*; John F. Beamis, MD; In Sup Choi, MD. Lahey
Clinic, Burlington, MA
INTRODUCTION: The incidence of bronchopleural fistula (BPF)
varies from 4.5% to 20% after pneumonectomy and 0.5% after lobectomy
(1). Surgical closure has a success rate of 85.7% (2) with associated risks
of open thoracotomy. Various materials have been used to attempt
endobronchial closure. We report a method of endobronchial closure with
vascular occlusion coils and cyanoacrylate glue using flexible broncho-
scope and will demonstrate the technique.
CASE PRESENTATION: A 70 year old female with previous history
of sarcoidosis presented with massive hemoptysis. Bleeding was initially
controlled with embolization of her bronchial artery. She returned in two
months with recurrent hemoptysis. After a failed second bronchial artery
embolization, a right lower lobe (RLL) resection was performed. Two
weeks after surgery she developed persistent pneumothorax that required
chest tube placement. Pleurodesis with doxycycline failed. Bronchoscopy
revealed a leak in the RLL stump.
Because of high operative risk we decided to close the fistula with an
endobronchial approach. A 0.5mm diameter Rapid Transit microcatheter
was placed into the stump through the working channel of a flexible
bronchoscope. Three 5mm, one 7mm C-TRUFIL and three 3mm
complex TRUFIL platinum vascular occlusion coils were propelled into
the leaking stump under fluoroscopic guidance.
Two cc of 30% n-butyl-cyanoacrylate (n-BCA) glue was then dripped
into the stump around the coils. The air-leak ceased immediately. A chest
radiograph revealed complete resolution of pneumothorax and the chest
tube was removed. Follow-up bronchoscopy after 5 days revealed solidi-
fied glue within the stump.
DISCUSSION: Cyanoacrylate glue has been used during intraopera-
tive stump strengthening after pneumonectomy (3,4) and is suggested for
endobronchial closure of proximal BPF(5). However, use of coils in
combination with n-BCA is reported in only three cases(6,7). N-BCA
polymerizes into a solid material upon contact with body fluids or tissue.
Iodized oil is added to delay polymerization to about 10-30 seconds. Coils
act as a matrix to fix the filling site with glue. The rapid glue solidification
mechanically occludes the fistula. Reactive proliferation of bronchial
mucosa further contributes to effective closure. Efficacy, safety and lack of
toxicity in human use of cyanoacrylate has been demonstrated and n-BCA
liquid embolic system is approved by Food and Drug Administraion for
embolization of cerebral arteriovenous malformations. Other adhesives
such as fibrin, gelfoam, autologous blood patch, coils alone or cyanoacry-
late glue alone have been used with variable success.
CONCLUSIONS: Endobronchial closure with vascular occlusion coils
and n-butyl-cyanoacrylate glue may be an effective therapeutic option for
occlusion of a postresectional BPF in patients with high operative risk. It
is safe, non-toxic, relatively non-invasive and inexpensive compared to
surgery. Large scale randomized studies are needed to document efficacy.
REFERENCES:
1. Cerfolio RJ. The incidence, etiology and prevention of postresec-
tional bronchopleural fistula. Semin Thorac Cardiovasc Surg. 2001;
13(1):3-7.
2. Pairolero PC et al. Postpneumonectomy empyema, The role of
intrathoracic muscle transposition. J Thorac Cardiovasc Surg 1990;
99:958-68.
3. Eng J, Sabanathan S. Tissue adhesives in bronchial closure. Ann
Thorac Surg. 1989;48:683-5.
4. Sabanathan S, Richardson J. Management of postpneumonectomy
bronchopleural fistula. J Cardiovasc Surg 1994;35:449-57.
5. McManigle JE et al. Bronchoscopy in the management of broncho-
pleural fistula. Chest 1990;97:1235-8.
6. Hirata et al. Endobronchial closure of postoperative bronchopleural
Tuesday, October 28, 2003
Interventional Pulmonology, continued
292S CHEST 2003—Case Reports

fistula using vascular occluding coils and n-butyl-2-cyanoacrylate.
Ann Thorac Surg 2002;74:2174-5.7. Jain et al. Endobronchial
closure of a bronchopleural cutaneous fistula using angiography
catheters. AJR 2000;175:1646-48.
DISCLOSURE: S.U. Islam, None.
DYSPNEA DUE TO SEVERE KYPHOSCOLIOSIS; BRONCHIAL
OCCLUSION TREATED WITH SELF-EXPANDING METALLIC
STENT
John Perry, MD*; Leonard C. Moses, MD. Virginia Commonwealth
University Health System, Richmond, VA
INTRODUCTION: Abnormalities of the thoracic cage are an unusual
cause of dyspnea. This is a rare case of extrinsic compression of a proximal
bronchus due to vascular infringement from severe kyphoscoliosis.
CASE PRESENTATION: An 82-year-old female patient was referred
to the Medical College of Virginia Hospital for evaluation of progressive
shortness of breath. The patient’s history was remarkable for severe
kyphoscoliosis s/p lumbar spine surgery and immobilization in the 1930’s.
She also has a history of intermittent atrial fibrillation and breast cancer
status-post mastectomy in 1999. The family history was remarkable for
scoliosis in her children and grandchildren. Medications included tamox-
ifen.
The patient describes symptoms of breathlessness with a persistent,
non-productive cough. She was previously healthy and independent, but
is now non-ambulatory and reports dyspnea at rest. She was twice
hospitalized in recent months for pneumonia. A chest radiograph revealed
profound skeletal abnormalities and a right pleural effusion with right
lower lobe collapse. Chest computed tomography confirmed the skeletal
abnormalities and the lower lobe collapse with effusion. Given her history
of breast cancer, malignancy was suspected. Fiberoptic bronchoscopy was
performed at the outside hospital and was negative for malignancy. She
was referred to the University for further evaluation.
On physical exam, the patient was an elderly, frail woman in moderate
respiratory distress. Breathing pattern was rapid and shallow, and the
patient had difficulty speaking in full sentences. Oximetry was 94% on
room air. Weight was 98 pounds. HR was 82 and regular. The chest was
asymmetric, with the right hemithorax much smaller than the left. Severe
kyphoscoliosis with dextro-rotation was noted. The right mastectomy scar
was observed. Breath-sounds were absent in the right base, with dullness
to percussion over half of the right hemithorax. Egophony was not
present. Left lung was clear to auscultation and percussion. Repeat
computed tomography of the chest revealed partial atelectasis of right
middle lobe and total collapse of the right lower lobe with a moderate
right pleural effusion. There was marked rotation of the thoracic cage and
mediastinal structures, with narrowing and extrinsic compression of the
right bronchus intermedius between the right pulmonary artery and the
rightward descending aorta. No mediastinal masses or adenopathy were
noted. The patient underwent repeat bronchoscopy under general anes-
thesia. The airway inspection confirmed subtotal compression of the right
bronchus intermedius. The distal airways beyond the bronchus interme-
dius were patent but filled with secretions. The patient underwent
placement of a self-expanding metallic stent under fluoroscopic guidance
(Ultraflex 12mm diameter, 20mm length). Re-expansion of the right lower
lobe was noted immediately, and pulmonary toilet of retained secretions
was performed. The patient was extubated after the procedure and was
discharged to home the following day.
Follow-up evaluation revealed both clinical and radiographic improve-
ment. The patient regained her previous functional status, becoming fully
independent and ambulatory. She reports walking “a mile a day”.
DISCUSSION: Abnormalities of the thoracic cage are an unusual
cause of dyspnea. Often, the respiratory limitation is due to restrictive
pulmonary physiology or diaphragmatic dysfunction. Rarely, bronchial or
vascular occlusion due to rotation of the thoracic cage has been reported,
with vital structures trapped between vascular structures and the vertebral
column. Bronchial obstruction due to vascular abnormalities is uncommon
and is attributed mostly to enlarging thoracic aortic aneurysms or
anomalous vascular anatomy. This is a rare case of extrinsic compression
of a proximal bronchus due to vascular encroachment from severe
kyphoscoliosis, treated with a self-expanding metallic stent.
CONCLUSION: This is an unusual case of kyphoscoliosis causing
vascular distortion and bronchial obstruction, treated with a self-expand-
ing metallic stent.
DISCLOSURE: J. Perry, None.
Thoracotomizing
4:15 PM - 5:45 PM
THORACIC SCHWANNOMA IN PREGNANCY
Isabella N. Stumpf-Cabot, DO*; William B. Williams, MD, FCCP. Pulmo-
nary and Critical Care Medicine, Maine Medical Center, Portland, ME
INTRODUCTION: Thoracic schwannoma is a rare condition in a
pregnant patient. Most tumors are benign and asymptomatic. This case
report describes a pregnant patient presenting with symptoms of chest
pain and dyspnea.
CASE PRESENTATION: This 35 year old woman presented at 12 weeks
gestation with two weeks of pleuritic pain and dyspnea. There was no history
of cough, hemoptysis, constitutional symptoms or tobacco use. Percussion
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 293S
CASE REPORTS

exam of the chest revealed localized dullness over the left posterior thorax.
Chest imaging studies identified a left pleural effusion and chest mass. A
thoracentesis showed exudative characteristics and non-diagnostic cytology.
The patient was transferred to this institution for further diagnosis and
management. A MRI of the chest characterized the mass as: 12cm in greatest
diameter, extrapleural in location, of heterogeneous appearance, with small
calcifications, and vertebral body remodeling. Differential diagnosis included
neurogenic tumor, sarcoma, blastoma, teratoma, lymphoma, hamartoma,
mesenchymal neoplasm, and bronchogenic carcinoma.
The patient underwent a left thoracotomy for resection of the tumor.
Histologic examination was consistent with a schwannoma Antoni type A
cellular pattern.
DISCUSSION: This is the first reported case of a thoracic schwan-
noma in pregnancy. Thoracic schwannomas comprise about 31% of
neurogenic tumors. Mean age of occurrence is 38 years, with a
predominance of caucasian females. Greater than 90% are benign. The
potential for malignant transformation occurs in 30% of these cases. A
posterior mediastinal location and tumor mass spanning at least two
intercostal spaces are the most common described features on chest
CT. MRI is the imaging study of choice for preoperative evaluation.
CONCLUSION: Despite rare occurrence, schwannomas are the most
common neurogenic tumor of the thorax. Surgical intervention is gener-
ally curative in benign tumors and eliminates the risk of malignant
transformation. Recurrence of benign tumors is unusual.
REFERENCES:
1. Ribet et al., Annals of Thoracic Surgery. 1994; 58: 1091-5.
2. Strollo et al., Chest. 1997; 112: 1344-57.
3. Ko et al., Journal of Thoracic Imaging. 1998; 13:21-6.
DISCLOSURE: I.N. Stumpf-Cabot, None.
DELAYED SLEEVE RESECTION OF DISTAL LEFT MAIN
BRONCHUS INJURY IN A CHILD AFTER A SHORT COURSE
OF STEROIDAL TREATMENT: CASE REPORT
Henry A. Kirk, MD*; Walter O’ Hara, MD; Giorgio M. Aru, MD.
University of MS Medical Center, Jackson, MS
INTRODUCTION: Tracheal bronchial injury (TBI) is an uncom-
mon event after blunt thoracic trauma, especially in the pediatric age
group. No reports of a delayed bronchial repair after treatment with
low dose steroids were found in the literature. Also, no reports of a
sleeve resection at this age for trauma were found. Therefore, we were
prompted to report on an eight year old female with delayed diagnosis
of traumatic bronchial injury subsequently treated with steroids prior
to definitive surgical repair.
CASE PRESENTATION: An 8 year old girl suffered from blunt chest
trauma with a distal left main bronchus injury diagnosed by flexible
bronchoscopy 10 days out from initial injury. The child was then treated
with a short course of low dose steroids followed by a sleeve resection and
primary anastamosis of the distal bronchus Now, two and a half years out,
the patient is doing well with no respiratory difficulties, a normal CXR,
and is actively participating in sporting events.
DISCUSSION: The initial delay in the diagnosis, as well as the clinical
stability of the patient, led to the consideration of using low dose steroids
in an attempt to limit the scarring and possible stenosis of the involved
bronchus. Also, the site of injury was in the distal left main bronchus just
at the bifurcation and the possibility of a pneumonectomy, during an early
surgical repair of inflamed tissues, persuaded us to delay the repair. We
felt that a course of low dose steroids could provide decreased inflamma-
tion and scarring at the periphery of the injury; therefore, allowing
resection of less bronchus.
CONCLUSION: We conclude that a sleeve resection for trauma can
be done successfully in the pediatric population. Also, we believe that in
selected cases of TBI, a short course of low dose steroids can reduce the
inflammation related to trauma, allows some healing, and reduces the
amount of bronchus to be resected.
DISCLOSURE: H.A. Kirk, None.
DIFFUSE ALVEOLAR HEMORRHAGE AFTER OPEN HEART
SURGERY
Shoaib Alam, MD*; Robert L. Vender, MD; Kathleen M. Chaisson, MD.
Penn State University-Milton S. Hershey Medical Center, Hershey, PA
INTRODUCTION: Cardiopulmonary bypass circuits cause mor-
bidity during and after cardiac operations. Bio-incompatibility of
heparin-coated extracorporeal bypass circuits affects plasma proteins
and cellular components and can contribute to increased risk of
abnormal bleeding and postperfusion syndrome. This is especially true
for the patients undergoing re-operative cardiac procedures, which
carries a higher risk of postoperative bleeding and prolonged ventila-
tion compared with the primary cardiac surgical procedures. The use
of methylprednisolone has been shown to attenuate the systemic
inflammatory response but no conclusive studies are available to
suggest additional clinical benefit.
CASE PRESENTATION: We present a case of a 31-year-old male
with idiopathic isolated congenital aortic stenosis who had an aortic
commissurotomy performed at the age of two. At the age of eleven, he
underwent aortic valve replacement with a 23mm concavoconvex Bjork-
Shiley prosthesis (23ABC15137). Secondary to high risk of strut fracture
of the above-mentioned prosthesis, the patient had an elective Re-do
aortic valve replacement with 23mm St Jude prosthesis on June 05, 2002.
The patient did not have any symptoms pre-operatively and the surgery
remained uneventful. Patient was placed on intravenous heparin for antico-
agulation. On the second post-operative day, he started having increasing
oxygen requirement, respiratory distress and severe hemoptysis. Chest radio-
graph initially showed left lower lung zone haziness and later diffuse bilateral
infiltrates. Patient’s oxygen requirement gradually increased to 100% (via
non-rebreather mask) and hematocrit decreased from 29 to 23 by fourth
post-op day. The patient remained afebrile and laboratory data did not show
any significant leukocytosis. A diagnosis of diffuse alveolar hemorrhage was
made, based on severe hemoptysis, diffuse bilateral infiltrates, fall in hemat-
ocrit, increased oxygen requirement, absence of fever or significant leukocy-
tosis and absence of an alternative explanation. Platelet count, prothrombin
time and serum creatinine remained normal. Partial thromboplastin time was
never extraordinarily above the theraputic value. No other sites of abnormal
bleeding were noted. Urine analysis did not show any hematuria or red cell
casts. Erythrocytes sedimentation rate was 96. Anti-nuclear antibody(ANA),
cytoplasmic immunofluorescent staining pattern anti-neutrophil antibody-
(cANCA) and antiglomerular basement membrane antibody(anti-GBM) tests
were non revealing. Heparin was discontinued and it was decided that any
further diagnostic or theraputic intervention will be done only if the patient’s
condition does not improve by the discontinuation of heparin. His dyspnea,
hemoptysis and oxygen requirement started improving within 24 hours of
discontinuing anticoagulation and chest radiograph also started showing
improvement. He was transferred out of surgical intensive care unit in
another 24 hours. After stabilization he was restarted on anticoagulation and
was eventually discharged to home without any oxygen supplementation on
post-operative day number eleven.
DISCUSSION: Diffuse alveolar hemorrhage after open-heart surgery
with the use of cardiopulmonary bypass is uncommon but reported in
literature. We are aware of one case report, where the patient was treated
with steroids along with discontinuation of anticoagulation and had
complete clinical and radiographic recovery.
CONCLUSION: Diffuse alveolar hemorrhage can develop after open-
heart surgery with the use of extracorporeal cardiopulmonary bypass
circuit, on anticoagulation. In our case, transiently holding anticoagulation
resulted in complete clinical and radiographic recovery, without any use of
steroids or any immunosuppressive therapy. Furthermore, no thrombo-
embolic complications were observed as a consequence of transiently
holding the anticoagulation.
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294S CHEST 2003—Case Reports

REFERENCES:
1. Biocompatibility of heparin-coated extracorporeal bypass circuits: a
randomized, masked clinical trial. J Thorac Cardiovasc Surg 1996
Aug; 112(2): 472-8.
2. Does high dose methylprednisolone in aprotinin-treated patients
attenuate the systemic inflammatory response during coronary
artery bypass grafting procedure? J Cardiothorac Vasc Anesth 1999
Apr; 13(2): 165-72.
3. A case of diffuse alveolar hemorrhage developing after open-heart
surgery. Kyobu Geka 2001 Sep;54(10)892-4.
DISCLOSURE: S. Alam, None.
ALTERNATIVE SURGICAL METHOD FOR REPAIR OF POSTIN-
FARCTIONAL LEFT VENTRICULAR FREE WALL RUPTURE
Laszlo Szekely, MD, PhD*; Laszlo Vandor, MD; Andras Haan, MD;
Andrea Varga, MD; Tibor Szonyi, MD; Zsolt Piroth, MD; Ferenc Horkay,
MD. Gottsegen Gy Hungarian Institute of Cardiology, Section of Cardiac
Surgery, Budapest, Hungary
INTRODUCTION: Left ventricular free wall rupture (LVFWR) is a
rare, but even serious complication of acute myocardial infarction, which
is associated with significant mortality and morbidity. Despite of improve-
ments in surgical techniques, it remains a therapeutic challenge. In this
report, we discuss the history of a patient, whose LVFWR was repaired by
a sutureless technique.
CASE PRESENTATIONS: 61-years old male patient was hospitalized
after acute myocardial infarction on the regions of anterior wall, apex and
septum due to a sudden closure of left anterior descending artery. The
coronary arteries, elsewhere, were intact. The left ventricular ejection
fraction was reduced to 25%. After recognition of his increasing level of
pericardial blood and tamponade, cardiac surgery intervention was imme-
diately carried out. Euroscore: 13, Parsonnet: 37. On the anterior wall, we
identified 3 independent bleeding holes in a 4X6 cm region. Utilizing a
5X7 cm PTFE Felt patch and a double component bio adhesive, we glued
and successfully repaired the ruptured wall without using fixing sutures.
The length of surgery was 63 minutes. The outcome was favorable. At the
end of surgery, there was no remaining bleedings from the heart. His
recovery period was uneventful and remained free of symptoms through-
out the five months of follow up period except a wound healing problem,
which occurred in the lower third of his incision and fixed.
CONCLUSIONS: Our case demonstrates a possible, alternative way to
repair left ventricular free wall rupture, which is safely applicable even if
in selected cases.
REFERENCES:
Canovas SJ, Lim E, Hornero F, Montero J.: Surgery for left
ventricular free wall rupture: patch glue repair without extracorporeal
circulation. Eur J Cardiothorac Surg 2003;23(4):639-41.
Mariani MA, D’Alfonso A, Nardi C, Grandjean JG.: Left ventricular
free wall rupture: off-pump sutureless patch and glue technique. Ital
Heart J 2002;3(12):755-7.
Lachapelle K, deVarennes B, Ergina PL, Cecere R. Sutureless patch
technique for postinfarction left ventricular rupture. Ann Thorac
Surg 2002;74(1):96-101.
Tomcsanyi I, Hajdu L, Szilasy Z, Szekely A, Medgyesy M: Successful
new method for surgery of the postinfarctional left ventricular free
wall rupture. Orv Hetil 2001;142(24):1269-71.
DISCLOSURE: L. Szekely, None.
SURGICAL MANAGEMENT OF MULTIPLE RIB FRACTURES
Mario G. Gasparri, MD*; G. Hossein Almassi, MD; George B. Haasler,
MD. Medical College of Wisconsin, Milwaukee, WI
INTRODUCTION: Multiple rib fractures remain life-threatening
injuries with considerable morbidity in survivors. There has been renewed
enthusiasm for surgical fixation of rib fractures as reports suggest im-
proved short and long-term outcomes. This case report illustrates the
Medical College of Wisconsin approach to these injuries using plates to
internally fixate the fractures.
CASE PRESENTATIONS: 49 year-old white male involved in mo-
torcycle accident. Seen at outside institution, found to have multiple rib
fractures, and discharged with Oxycontin. Represented within 24 hours
with pain and shortness of breath.
Taken to operating room for rib stabilization. Via left thoracotomy, left
ribs 3-9 were stabilized using plates.
Patient discharged home post-operative day (POD) 4, back to work
POD 10, and off all pain medications POD 14.
Pulmonary Function Tests
PFTs Preoperative Postop Day 11 Postop Day 30
FVC 57% 94% 96%
FEV1 63% 87% 90%
DLCO 58% 55% 98%
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DISCUSSION: The current standard treatment for multiple rib
fractures consists of aggressive pulmonary toilet, vigorous pain control,
cautious fluid administration, and selective intubation. Despite this
approach, these injuries remain lethal with in-hospital mortalities of
roughly 15%
1,2
and pulmonary morbidities (pneumonia, empyema,
ARDS) of roughly 25%
3,4
. Additionally, in survivors, long-term morbidity
is significant with 30-50% complaining of chronic pain and dyspnea and
25-35% remaining permanently disabled
5,6
. Studies have suggested that
surgical fixation of rib fractures rapidly restores normal chest wall
dynamics and decreases pain resulting in decreased mortality, decreased
need for mechanical ventilation, acceleration of in-hospital recovery, and
decreased long term morbidity
7,8
. Also, current surgical techniques and
innavative hardware have allowed this procedure to be performed safely
with minimal surgical morbidity
9
. This case illustrates these points as
fixation of the patient’s rib fractures allowed rapid recovery with early
normalization of pulmonary function tests and resumption of normal
activity.
CONCLUSIONS: Multiple rib fractures carry with them high mor-
bidity and mortality rates and in survivors, long-term morbidity is
significant. Operative rib stabilization has a role in treatment and studies
suggest improved short and long-term outcomes. Further studies are
required to better delineate treatment effects and allow optimal patient
selection.
REFERENCES
1. Ziegler DW, Agarwal NN. The morbidity and mortality of rib
fractures. J Trauma 1994; 37:975-979
2. Ciraulo DL, Elliott D, Mitchell KA, Rodriguez A. Flail chest as a
marker for significant injuries. J Am Coll Surg 1994; 178:466-70.
3. Albaugh G, Kann B, Puc MM, et al. Age-adjusted outcomes in
traumatic flail chest injuries in the elderly. Am Surg 2000; 66:978-81
4. Bulger EM, Arneson MA, Mock CN, Jurkovich GJ. Rib fractures in
the elderly. J Trauma. 2000; 48:1040-1047
5. Landercasper J, Cogbill TH, Lindesmith LA. Long-Term Disability
After Flail Chest Injury. J Trauma 1984; 5:410-4
6. Beal SL, Oreskovich MR. Long-Term Disability Associated With
Flail Chest Injury. Am J Surg 1985; 150:324-326
7. Tanaka H, Yukioka T, Yamaguti Y, et al. Surgical stabilization of
internal pneumatic stabilization? A prospective randomized study of
management of severe flail chest patients. J Trauma 2002; 52:727-
732
8. Ahmed Z, Mohyuddin Z. Management of flail chest injury: internal
fixation versus endotracheal intubation and ventilation. J Thorac
Cardiovasc Surg 1995; 110:1676-80
9. Oyarzun JR, Bush AP, McCormick JR, Bolanowski PJ. Use of
3.5-mm Acetabular Reconstruction Plates for Internal Fixation of
Flail Chest Injuries. Ann Thorac Surg 1998; 65:1471-4
DISCLOSURE: M.G. Gasparri, None.
LIMITATIONS OF STAPLED TRACTOTOMY FOR CENTRAL
LUNG PENETRATING INJURY
Eric J. Kuncir, MD, MS, FACS*; Howard Belzberg, MD. Affiliate,
University of Southern California, Los Angeles, CA
INTRODUCTION: The operative management of penetrating injury
by pulmonary tractotomy with selective vascular ligation has been well
described. We report a case of stapled tractotomy resulting in contralat-
eral lung collapse.
CASE PRESENTATION: The patient, a 19 year old male, sus-
tained multiple gunshot wounds: four head and neck and four chest
wounds. He had shortness of breath, bilateral decreased breath
sounds, GCS 14, BP 130/82, pulse 140 and respirations 26. Bilateral
chest tubes were placed and he was emergently intubated. The
abdomen was soft and nontender, FAST examination was negative
twice and Hg levels were stable.
Total left sided output increased to 1400 cc and head CT was aborted
and he was taken to the OR. A diaphram laceration, two lower lobe
injuries and a central anterior segment injury were found at left thoracot-
omy. Bullet tracts were divided with a GIA stapler and bleeding pulmo-
nary vessels were oversewn. No residual airleak or bleeding was observed.
Laparotomy was performed with splenectomy and repair of injuries to the
stomach, colon, jejunum, duodenum and ureter.
The patient returned to the SICU with a temperature of 34.1
o
C and
a pH of 7.19 and was placed on a high frequency percussive ventilator.
Soon thereafter, the airleak increased and oxygen saturation decreased
and bedside bronchoscopy confirmed proper ETT position and dem-
onstrated no active bleeding. Desaturation persisted and the patient
was returned to the OR. Chest exploration was negative and oxygen
saturation dropped further prompting right thoracotomy showing total
lung collapse. Anatomical left upper lobe resection was performed and
bronchoscopy cleared the airway and oxygenation and ventilation
improved.
DISCUSSION: Nearly 80% of penetrating lung injuries can be
treated with chest tube and observation alone. The remaining 20% of
penetrating lung injuries require operation. In the past, this meant
formal anatomical lung resection. However, with the advent of stapled
tractotomy, 80% of operative lung injuries can be managed without
formal lung resection.
1
Wall outlines the application of stapled tractotomy.
1
In general, it is
unacceptable to simply oversew the entrance and exit lung wounds as this
may lead to a large intrapulmonary hematoma, infection, abcess, life
threatening air-embolism and persistant airleak and difficulty ventilating.
The technique of tractotomy is non-anatomical and is dictated by the
bullet trajectory. Adequate arterial and venous collateral circulation allows
the lung to remain viable and functional. The goal is to expose and ligate
injured broncheoles and deep bleeding vessels and preserve lung and
avoid formal lobe resection or pneumonectomy. Uncontrolled bleeding
can result in internal blood aspiration and difficulty ventilating. Wall
recommends using tractotomy only for wounds located from one half to
two thirds centrally toward the hilum.
1
Once the tract is opened the hilar
structures should be inspected to assure that formal resection is not
needed.
1
In this case, inspection revealed no major bronchial or pulmonary
vascular compromise such that formal resection was not initially done.
However, as is pointed out by Richardson, superficial suturing of paren-
chymal lung wound, as in the edges of the tractotomy, may only stop
bleeding externally and can inadvertently convert the bleeding to an
endobroncheal nature.
2
He advises that the endobrocheal tube be care-
fully suctioned before the end of the operation to ensure that internal
bronchial bleeding has not occurred.
2
CONCLUSION: Application of stapled tractotomy to central lung
injuries is potentially dangerous as this case demonstrates. Unrecognized
intra-broncheal bleeding can occur.
REFERENCE:
1. Wall MJ et al. Pulmonary Tractotomy with Selective Vascular
Ligation for Penetrating Injuries to the Lung. Am Surg. 168:665-669,
1994.
2. Richardson JD et al. Injury to the Lung and Pleura. In: Mattox KL
et al, eds. Trauma.4
th
ed. New York: McGraw-Hill, 2000:523-543.
DISCLOSURE: E.J. Kuncir, None.
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296S CHEST 2003—Case Reports

MASSIVE PULMONARY EMBOLISM WITH A MOBILE THROM-
BUS IN THE RIGHT ATRIUM EXTENDING THROUGH THE
ATRIAL SEPTUM TO THE LEFT ATRIUM INTO THE LEFT
VENTRICLE
Hassan A. Haddadin, MD*; Habibur Rahman, MD, FCCP; Farshid
Radparvar, MD. Department of Pulmonary and Critical Care, Mount
Sinai School of Medicine, New York, NY
INTRODUCTION: The debate regarding treatment of massive pul-
monary embolism (PE) with thrombolytic agent is ongoing and clear cut
indications still lacking and what makes the decision even harder is the
presence of right side heart thrombemboli. Massive pulmonary embolism
and hemodynamic instability still the most definite indication for throm-
bolytics therapy even though there is no strong evidence to support this
approach.
CASE REPORT: A 73-year-old woman admitted to our hospital with
left leg pain and sudden onset of shortness of breath. She has a past
medical history of left breast cancer treated with mastectomy, radiation
therapy and chemotherapy.
Physical exam revealed a blood pressure of 122/76, heart rate of 113
beat per minute, respiratory rate of 24-30 and an O2 Sat of 86% on
room air (RA). Electrocardiogram shows a sinus tachycardia with left
ventricular hypertrophy. Chest radiograph reveals left side pleural
effusion. Spiral computer tomograph (CT) of the chest demonstrated
a right pulmonary artery thrombus extending to upper, middle and
lower lobe branches.
A transthoracic echocardiogram (ECHO) shows a large amorphous
mobile thrombus in the left atrium prolapsing through the mitral valve
into the left ventricle during systole and protruding across aneuryismal
intraatrial septum into the right ventricle. The right atrium appeared to be
hypokinetic and there was a decrease left ventricular dysfunction. A
Doppler ultrasound of the lower extremities was negative for deep venous
thrombosis.
After placement of vena cava filter the decision was made to throm-
bolize the patient with tissue plasminogen activator and she was moni-
tored in our intensive care unit. Unfractioned heparin was started after the
thrombolytic infusion. Four hours after thrombolysis the patient became
aphasic and develops right-sided weakness. Subsequent CT of the head
reveals an ischemic stroke.
A repeat ECHO demonstrated the disappearance of the thrombus and
improved right ventricular function. The patient saturation improved to
94% on RA.
Patient neurological status improved and she was started on coumadin
and transferred to inpatient rehabilitation facility.
DISCUSSION: The increased use of ECHO in patients with PE
resulted in more frequent identification of right-sided heart thrombem-
boli, with reported incidence of 3% to 23%(1).
The majority are found in the right atrium. Most report recommends
aggressive treatment as discussed below.
In our case the thrombus was going through intraatrial septum to the
left atrium into the left ventricle which poses the risk for paradoxical
emboli. Therapeutic options in this case were 1. Anticoagulation 2.
Thrombolytic therapy with anticoagulation 3. Surgical removal of the
thrombus. Recent review identified 177 patients with right hear throm-
bemboli reported an overall mortality rate of 27%. The mortality rates in
patients treated with anticoagulation, surgery, or thrombolytic therapy
were 28.6%, 23%, and 11.3%, respectively(2). Our consensus was to use
thrombolytic agent taking into account that the benefit would overweight
the risk of motility from the massive PE.
Anticoagulation remains the standard of care for venous thrombembo-
lism which prevent clot propagation. Thrombolytic therapy, in contrast,
produces more rapid clot lysis and may result in earlier improvement in
pulmonary perfusion, hemodynamics, gas exchange, and right ventricular
function
CONCLUSION: The American College of Chest Physicians consensus
statement on antithrombotic therapy published in 2001 outlined appro-
priate regimen for the use of thrombolytic agent. There was a continued
emphasis that the use of these agents should be highly individualized. Our
patient could have been managed differently depending on different
approach, experience and available resources.
REFERENCES:
(1) Chakko S, Richards 3rd F. Right-sided cardiac thrombi and pulmo-
nary embolism. Am J Cardiol 1987;59:195-6
(2) Rose PS, Punijabi NM, Pearse DB. Treatment of right heart
thrombemboli. Chest 2002;121:806-14.
DISCLOSURE: H.A. Haddadin, None.
A PREGNANT WOMAN WITH GALLSTONE PANCREATITIS
AND PULMONARY EMBOLISM
Daniel Kass, MD*. Columbia University College of Physicians and
Surgeons, New York, NY
INTRODUCTION: This case will illustrate some of the difficult
decisions facing clinicians managing the pregnant patient with pulmonary
embolism (PE).
CASE PRESENTATION: An 18 year old woman at 30 weeks of an
uncomplicated, first pregnancy presented to the emergency department
complaining of abdominal pain. Physical exam was notable for right upper
quadrant abdominal tenderness. Laboratory studies revealed a markedly
elevated amylase and lipase. Abdominal ultrasound showed cholelithiasis.
All fetal parameters were normal. She was admitted with the diagnosis of
gallstone pancreatitis. Total parenteral nutrition was started, and she was
placed on bed rest. Five days into her admission, she was noted to be
dyspneic. Oxygen saturation of hemogloblin (SaO
2
) was 92% on room air.
Computed Tomographic Angiogram of the chest (Figures 1 and 2) show
multiple pulmonary emboli at the bifurcation of the right and left main
pulmonary arteries. Bedside echocardiogram revealed a dilated and
hypokinetic right ventricle. Enoxaparin was administered subcutaneously
1 mg/kg every 12 hours.
DISCUSSION: There are several difficult questions facing the clini-
cian in the management of PE in pregnancy, including the risk of radiation
exposure to the fetus in diagnosing PE and the safety of anticoagulation
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and thrombolysis, especially if an emergent delivery is needed or for
maternal hemodynamic collapse. Unfractionated and low molecular
weight heparins have no known teratogenic effects and do not anticoag-
ulate the fetus. Turrentine in 1995 reviewed 172 cases of women with
pregnancy-associated venous thromboembolism treated with thrombolyt-
ics. Data on efficacy of these agents are culled from the literature on
non-pregnant patients. More studies are necessary to elucidate guidelines
for therapy in this population.
CONCLUSION: After one week of therapy, oxygenation and RV
function normalized. A healthy boy was delivered electively at 33 weeks.
The pancreatitis resolved after placement of a stent.
REFERENCES:
Howie PW. Clin Obstet Gynaecol.1986 Jun;13(2):349-63.
Melissari E et al. Thromb Haemost. 1992 Dec 7;68(6):652-6.
Turrentine, MA. Obstet Gynecol Surv. 1995; 50:534.
Arterial Blood Gas Analysis
pH 7.49
PaCO
2
29 mm Hg
PaO
2
57 mm Hg
SaO
2
92%
DISCLOSURE: D. Kass, None.
ISOLATED TAKAYASU DISEASE OF THE PULMONARY AR-
TERY MIMICKING RECURRENT PULMONARY EMBOLISM
Juan E. Morales, MD*; Daniel Iltchev, MD; James N. Allen, MD; Namita
Sood, MD. Ohio State University Medical Center, Columbus, OH
INTRODUCTION: Isolated Takayasu arteritis (TA) of the pulmonary
circulation is extremely rare. We present a case of isolated TA of the
pulmonary artery mimicking recurrent pulmonary embolism.
CASE PRESENTATION: A 30-year-old-man with a history of inter-
mittent fatigue, arthralgias and myalgias for several years presented with
fever, night sweats, pleuritic chest pain and a ESR of 100 mm/hr.
Vasculitis serology was negative. A ventilation-perfusion scan (V/Q scan)
was high probability for pulmonary embolism (PE). He was started on
anticoagulation, as well as prednisone and hydroxychloroquine for non-
specific connective tissue disease. Four months later the prednisone was
tapered and he presented again with pleuritic chest pain and dyspnea.
Physical exam and laboratory data were unremarkable. V/Q scan again
showed a perfusion defect. Dopplers of lower extremities were negative
for deep venous thrombosis and CT angiogram was negative for throm-
boembolism. He subsequently complained of progressive dyspnea with
exertion. Echocardiogram showed mild pulmonary hypertension. An
arteriogram showed diffuse narrowing of the pulmonary arteries compat-
ible with vasculitis. MRA did not show involvement of other large vessels.
The patient was started in high doses steroids with some improvement of
his symptoms.
DISCUSSION: Both giant cell arteritis and TA can present with
systemic manifestations and involvement of the pulmonary artery. There
are no specific serological markers for these conditions and the diagnosis
often needs to be done on a clinical basis. Because of the patient’s age and
the lack of classic symptoms of giant cell arteritis, a diagnosis of TA was
made.
TA is a chronic inflammatory disease of unknown origin that involves
the aorta and its main branches. Although abnormal pulmonary angiog-
raphy is described in 40-85% of the time, only few cases of isolated TA of
the pulmonary artery have been described. The combination of symp-
toms, pulmonary hypertension and abnormal V/Q scan can make difficult
the differentiation with chronic thromboembolic disease. Some of the
clues to suspect the diagnosis of TA in this case were: the presence of
chronic constitutional symptoms, a very high ESR, the absence of risk
factors for PE, apparent failure of anticoagulation treatment and the fact
that a thrombotic site was never found.
CONCLUSION: Isolated TA of the pulmonary artery is a rare
condition that can mimic the clinical presentation of PE. Failure to
respond to anticoagulation, presence of chronic constitutional symptoms,
unclear thrombotic site and high ESR should prompt a more extensive
work up.
DISCLOSURE: J.E. Morales, None.
UNUSUAL CARDIAC MANIFESTATION IN WEGENER’S GRAN-
ULOMATOSIS
Mariselly Medina, MD*. Affiliate, LSU Health Science Center, Shreve-
port , Louisiana, Shreveport, LA
INTRODUCTION: Wegener’s granulomatosis (WG) is a disease
whose target organs are the upper and lower respiratory tracts and the
kidney. Some reports have documented other organ involvement, includ-
ing the heart. Cardiac involvement in WG is unusual but the most
common cardiac manifestations are pericarditis and coronary arteritis.We
describe a patient with Wegener’s and no history of heart disease who
developed a cardiomyopathy resulting in cardiac failure.
CASE PRESENTATION: 50 y/old white woman who presented with
increased shortness of breath, cough and hemoptysis for two weeks . She
also had lower extremities edema. WG was diagnosed since 1989 by lung
biopsy and she had 3-4 relapse since the diagnosis was made. She has been
treated with methotrexate, prednisone, and bactrim(intermittently). Last
round of medications include methotrexate 2-3 weeks prior to amission
but she was changed to cytoxan 100mg and prednisone 20mg one week
prior to admission due to increased dyspnea. Her history was significant
for 12years smoking history. No cardiac history. Initial evaluation revealed
a patient with mild tachypnea, afebrile. Cardiovascular exam was negative.
Bilateral crackles and wheezes were noted. There was marked leg edema
up to the thigh and erythematous macules with necrotic center on
extremities. The rest of the physical exam was negative. Labs obtained
showed leukocytosis and thrombocytopenia. Hgb, coagulation panel,
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cardiac enzymes and electrolytes were normal. C-ANCA was 1:160. EKG
showed sinus tachycardia. Admission blood gas analysis showed PaO2
77mmHg sat 95% on RA. CXR revealed multiple bilateral lung cavitations
with cardiomegaly . She had a spiral chest CT scan that showed nodules
bilaterally, biventricular chamber enlargement with R atrial distention and
evidence of intramural thrombi on R atrium and both ventricular
chambers. An echocardiogram showed LV ejection fraction of 25% and
thrombus in the left ventricle . On admission thrombophilia work up was
obtained revealing only abnormal protein S(45). She received anticoagu-
lation and diuretics improving her dyspnea initially. During the hospital
stay her condition deteriorated and she became increasingly short of
breath and hypoxic despite of treatment and subsequently she died. The
autopsy revealed myocardial hypertrophy and ventricular dilatation, seg-
mental pulmonary emboli , necrosis of pulmonary vascular walls and lung
parenchyma, and severe hemorrhage.
DISCUSSION: WG involving the heart has been described, ranging
from 6 to 44% of cases, but significant cardiac complications during the
course of the disease are rare. Forstot et al retrospectively analyzed
cases of patients with cardiac involvement and they found about 50%
had pericarditis, 50% had coronary arteritis, 25% focal myocarditis and
21% valvulitis or endocarditis, conduction system was involved in 17%
and myocardial infarction in 11%. Myocarditis with granulomas had
been described producing acute cardiac failure that may progress to
cardiomyopathy. To our knowledge, intrinsic heart muscle involvement
leading to cardiomyopathy and cardiac failure is very rare in WG and
this has rarely been reported . On her autopsy the predominant
pathology was found in the cardiopulmonary system. Clearly in the
lungs there was vasculitis suggestive of Wegeners. The most evident
finding in the heart was the presence of diffuse fibrosis of myocardium
without inflammation or arteritis. The most likely explanation for the
sequence of events is the combination of multiple factors: cardiomy-
opathy with poor systolic function and tendency to develop mural
thrombi secondary to low flow state and second the possibility of
thrombophilic state associated with Protein S deficiency.
CONCLUSION: This case shows that although the clinical manifesta-
tion of cardiac disease in WG is not usually encountered, it should be
considered in patients with this clinical presentation. We think that the
cardiac condition in this patient could be directly attributable to WG in
association with a thrombophilic state .
DISCLOSURE: M. Medina, None.
PROGRESSION OF A DEGENERATED ATHEROSCLEROTIC
VEIN GRAFT ANEURYSM
Steven C. Kessel, MD*; Janah I. Aji, MD, FACC. Cooper Hospital,
Camden, NJ
INTRODUCTION: 70-year-old male with a remote history of CABG
and a new mediastinal mass.
CASE PRESENTATION: Patient presented to our institution for a
coronary angiogram. He had a past history of coronary atherosclerosis and
a four-vessel CABG thirteen years ago. A recent chest x-ray showed a new
mass measuring about 4 cm located at the right heart border, which was
not present from a film three months prior. Subsequent CT scan of the
chest confirmed the presence of a large vascular structure.A cardiac
catheterization was done three months ago due to unstable angina. It
showed a patent SVG to the distal RCA, which was dilated and ectatic.
The mid- portion of the graft had a 1.5 cm ulcerated pocket with
thrombus. The SVG to D1 & D2 were occluded and the OM1 graft &
LIMA to LAD were patent. Subsequent to the injection of the LIMA the
patient developed anterior ST elevations. An intraaortic balloon pump was
placed, and balloon angioplasty was done to the distal LAD. He also
experienced an embolic event to his right lower extremity, which neces-
sitated an embolectomy to his right femoral artery.He was placed on
coumadin prior to discharge.
Patient also had a history multiple vascular aneurysms with a history of
AAA repair twice and left iliac artery aneurysm coil embolization. A
transthoracic echocardiogram showed an ejection fraction of 30% with
moderate aortic and mitral valve insufficiency. He also has hypercholes-
terolemia and COPD with a 50 pack-year history of smoking.
Examination showed a BP of 101/56, a pulse of 82. He had a normal
JVP, clear lungs, a laterally displaced impulse with a 2/6 diastolic
murmur, and palpable peripheral pulses. ECG showed sinus rhythm
and LVH. Cardiac catheterization showed an aneurysm in the RCA
vein graft, measuring about 4X6 cm in the vicinity of the ulcer seen in
the coronary angiogram from five months ago. The LIMA and distal
LAD were patent, and he had moderate to severe aortic insufficiency
on aortography.
CONCLUSION: The coumadin was halted and he was referred for
combined aortic valve replacement and aneurysmectomy. He also had a
new vein graft to the RCA and a marginal artery. Upon visual inspection,
the vascular mass appeared to be a thin-walled structure consistent with a
pseudoaneurysm. Five months later, the patient is doing fine.
DISCUSSION: This case illustrates the development of a pseudoan-
eurysm in a heavily atherosclerotic and degenerated coronary vein graft
thirteen years post-CABG. It is reported in the literature that atheroscle-
rosis is the etiology of the late vein graft aneurysms.
1
Possible treatment
options include surgery, percutaneous coil embolization, or covered
stenting.
2
The recent initiation of coumadin may have caused expansion
through dissolution of endovascular thrombus and increased Laplacian
forces on the vein graft wall. This case also underscores the importance of
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ruling out vascular aneurysms in patients with prior coronary bypass
grafting when new mediastinal masses are seen on noninvasive imaging.
REFERENCES:
1. “Clinical Conference on Management Dilemmas - An Expanding
Vascular Mass”, Gupta, et.al. Chest, Dec.2000 pg.1769-75.
2. “Occlusion of a SVG Aneurysm with a Covered Stent”, Journal of
Interventional Cardiology, June 2002 pgs.202-204
DISCLOSURE: S.C. Kessel, None.
SHOCK DUE TO MASSIVE PULMONARY EMBOLISM
TREATED WITH A PIGTAIL CATHETER
Kamran I. Hamirani, MD*; UMDNJ (Cooper Hospital. University Med-
ical Center), Camden, NJ Ronald Gottlieb MD. Graduate Hospital.
Philadelphia, PA. Robert Kleiman MD. Graduate Hospital. Philadelphia,
PA.
INTRODUCTION: Massive pulmonary embolism is a risk for cardiac
death from right heart failure and survival depends on rapid re-canaliza-
tion. Traditionally this is achieved with thrombolytics but in severe cases
even lytic therapy may fail. In these cases mechanical fragmentation with
percutaneous catheter treatment is an option.
CASE PRESENTATION: 86-year-old male smoker with past med-
ical history of deep vein thrombosis, pulmonary embolism, oral cavity
CA admitted with syncope. In the emergency room his BP was
82/40mmhg, pulse of 80/m and a pulse ox of 80%. He was treated with
intravenous fluids and oxygen. Initial exam showed left neck lymph-
adenopathy. Chest was clear bilaterally. S1 and S2 were normal and
there was no peripheral edema. Chest X ray was without any acute
process. EKG with RBBB and first-degree heart block. D-dimers
were ⬎ 500.Lower extremity ultrasound showed right superficial
femoral, popliteal, posterior tibial and left popliteal vein DVT. A VQ
scan was requested but the patient became severely hypotensive and
dyspneic resulting in intubation. Pulmonary angiogram showed oc-
cluded right pulmonary trunk. The RAP was 21, PAP 65-70/25, PCWP
of 17. The patient was treated with mechanical fragmentation of the
clot with a pigtail catheter, followed by TPA and I/V heparin. A repeat
pulmonary angiogram showed near normal right pulmonary trunk with
a PAP of 40 mmhg.
DISCUSSION: Patients with massive pulmonary embolism frequently
have acute right heart failure. If untreated the out come is frequently fatal.
Thrombolytics is the gold standard for treating pulmonary embolism. In
severe cases thrombolytics may fail to prevent fatal outcome. Survival
depends upon rapid recanalization. The pigtail catheter allows easy
manipulation into and within the pulmonary artery. Its tip avoids perfo-
ration of the pulmonary artery. Rapid recanalization can be safely
achieved with mechanical fragmentation of the clot with the pigtail
catheter. Moreover the increased total surface area of the fragments may
accelerate the efficacy of the lytics. The average procedure time is 45
minutes and an average fragmentation time of 15 minutes. This recana-
lization rate achieved by the pigtail compares well with the results of lytics.
Recently newer especially designed rotational pigtail catheters have been
used with improved success.
CONCLUSIONS: Mechanical fragmentation of the thrombus in mas-
sive pulmonary embolism with a pigtail catheter is a safe and rapid method
to recanalize the pulmonary vasculature and improve the hemodynamics.
It is a synergistic adjunct to lytics and an alternative to surgical embolec-
tomy.
DISCLOSURE: K.I. Hamirani, None.
Critical Care II
4:15 PM - 5:45 PM
LEMIERRE’S SYNDROME (LS): SEPSIS COMPLICATING A
DENTAL PROCEDURE
Mir T. Ali, MD*; Pankaj Jain, MD; Gopal Narayanswami, MD. St. Luke’s
Roosevelt Hospital, New York, NY
INTRODUCTION: Lemierre’s syndrome (LS) is a suppurative infec-
tion caused by Fusobacterium necrophorum. Metastatic abscesses result-
ing from septic embolization secondary to internal jugular vein thrombo-
phlebitis characterize this syndrome.
CASE PRESENTATION: A 39-year-old black male presented to the
Emergency Department with a five-day history of constipation, abdominal
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300S CHEST 2003—Case Reports

pain, high-grade fever and left leg swelling with pain. Past medical history
was non-contributory except for dental work three weeks prior to
admission.
Initial vital signs were temperature 103F, heart rate 130/min, respira-
tory rate 26/min and a blood pressure of 126/75. Oropharynx was normal.
Notable findings were icterus, abdominal tenderness and swelling in the
left leg and left side of neck. Admission labs revealed a white blood cell
count of 21,000/cu.mm, hemoglobin of 12.9g/dL, platelets of 59,000/
cu.mm, bilirubin of 5.3mg/dL, alkaline phosphatase of 521IU, blood urea
nitrogen of 68mg/dl and creatinine of 2.4mg/dL.
A computerized tomogram (CT) scan of the abdomen revealed multiple
abscesses in the liver and lung. A transesophageal echocardiogram was
unremarkable. Blood cultures were positive for Fusobacterium necropho-
rum, and he was treated with imipenem and clindamycin. Ultrasound of
the internal jugular vein revealed no thrombophlebitis. A CT scan of the
left lower extremity revealed myositis.
After four weeks of intravenous antibiotics he was discharged on oral
amoxicillin/clavulanate.
DISCUSSION: Lemierre’s syndrome is characterized by an acute
oropharyngeal infection, suppurative thrombophlebitis of the internal
jugular vein, anaerobic sepsis and metastatic abscesses. It was A. Lemierre
in 1936 who described the syndrome in detail [1].
Pulmonary involvement is reported in up to 85% of cases [2,3]. Hepatic
involvement may present as jaundice, abdominal pain and hepatomegaly
with abscesses. Hyperbilirubinemia may be either due to hepatic ab-
scesses or a direct effect of the toxin on the biliary tree [4]. Joint and bone
involvement may present as septic arthritis or osteomyelitis.
Blood cultures may take several days to become positive, as in this case
[5]. Ultrasonography, CT scan, or magnetic resonance angiography of the
neck can diagnose thrombosis of internal jugular vein. Ultrasonography
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was negative for thrombosis of the internal jugular vein in our patient. It
is probable that the thrombus was present in an area relatively inaccessible
to ultrasound, like the skull base, where the mandible inhibits full
visualization or behind the clavicle [6].
The recommended treatment of Lemierre’s syndrome is a combination
of high dose penicillin and metronidazole or monotherapy with Clinda-
mycin for 2-6 weeks [7].
Drainage of abscesses is often necessary. Anticoagulation is controver-
sial and carries the risk of extending the infection [8].
CONCLUSION: Lemierre’s Syndrome should be suspected in a young
patient with antecedent oropharyngeal infection presenting with features
of systemic septic embolization. Imaging of internal jugular vein helps in
establishing a diagnosis. Early institution of appropriate antibiotics results
in a favorable outcome.
REFERENCES:
1. Lemierre A. On certain septicemias due to anaerobic organisms.
Lancet 1936; 1:701-3.
2. Morenzo S, Altozano JG, Pinilla B. Lemierre’s Disease: Post anginal
bacteremia and pulmonary involvement caused by Fusobacterium
necrophorum. Rev Infect Disease 1989; 11:319-24.
3. Hagelskjaer LH, Prag J, Malczynski J. Incidence and clinical
epidemiology of necrobacillosis including Lemierre’s Syndrome in
Denmark 1990-1995. Eur J Clin Microbiol Infect Dis 1998; 17:
561-5.
4. Baddour LM, Land MA, Barrett FF. Hepatobiliary abnormalities
associated with post anginal sepsis. Diagn Microbiol Infect Dis
1986; 4:19-28.
5. Henry S, DeMaria A, McCabe WR. Bacteremia due to Fusobacte-
rium species. Am J Med 1983; 75:225-31.
6. Screaton N, Ravenel J, Lehner P. Radiology Nov 1999; 213:2:369-
374.
7. Guidol F, Manresa F, Pallares R. Clindamycin vs. Penicillin for
anaerobic lung infections. Arch Intern Med 1990; 150:2525-9
8. Moore-Gillon J, Lee TH, Dykyn SJ. Necrobacillosis: A forgotten
disease. Br Med J 1984; 288:1526-27.
DISCLOSURE: M.T. Ali, None.
DROTRECOGIN ALFA USE FOR SEVERE SEPSIS FOLLOWING
CORONARY ARTERY BYPASS GRAFT SURGERY
Shahid Yakoob, MBBS*; Bryan Veynovich, DO, FCCP; Brian Carlin, MD,
FCCP; Khalid Malik, MD, FCCP; Lawrence Crist, DO; Deepak Singh,
MD. Allegheny General Hospital, Pittsburgh, PA
INTRODUCTION: Drotrecogin alfa is a newer therapy available for
the treatment of severe sepsis. Its use is not well studied in patients
following coronary artery bypass graft surgery (CABG). We present a
patient who developed sepsis following CABG in which treatment with
drotrecogin alfa resulted in significant improvement.
CASE PRESENTATION: A 78 year-old male presented with an acute
myocardial infarction and left ventricular dysfunction (ejection fraction
10%) treated with heparin, aspirin and clopidogrel. He developed hypo-
tension during a cardiac catheterization and required intra-aortic balloon
pump support. He underwent emergent CABG. Following surgery his
cardiac function improved (EF 30%). He required mechanical ventilatory
assistance and four days later developed increased tracheal secretions
which decreased following antibiotic therapy. Six days later he developed
fever (39.1c), hypotension, leukocytosis, renal failure, and a left upper
lobe infiltrate. Intravenous fluids, dobutamine, norepinephrine, phenyl-
ephrine and epinephrine were needed to maintain a mean arterial
pressure of 60 torr. A transesophageal echo showed biventricular dysfunc-
tion (EF 10%). Sepsis due to pneumonia was diagnosed and drotrecogin
alfa was started. His hemodynamics improved significantly and he was
able to be weaned from the pressor support within five hours. No
significant bleeding occurred thereafter. He was transferred from the
intensive care unit but he required ventilatory and dialysis support. A
decision was made by the family not to maintain ongoing dialytic support.
He died thirty days later.
DISCUSSION: This case represents the use of drotrecogin alfa in a
patient with severe sepsis developing soon after CABG. Drotrecogin alfa
is useful for patients with severe sepsis but its use for patients treated with
anticoagulant and antiplatelet agents and/or CABG has not been studied.
Our patient with cardiogenic shock was treated with aspirin, clopidogrel
and CABG. Sepsis, unresponsive to pressor agents, was treated with
drotrecogin alfa with dramatic improvement in hemodynamics within
several hours and without significant bleeding.
CONCLUSION: Drotrecogin alfa should be considered for the treat-
ment of severe sepsis in a patient following CABG.
REFERENCES:
Gordon R. Bernard. Efficacy and safety of Recombinant human
activated Protein C for severe sepsis. New England Journal of
Medicine 344(10) 2001 699-709
Gordon R. Bernard. Safety and close relationship of recombinant
human activated protein C for coagulopathy in severe sepsis. Critical
Care Medicine (29) 2001 2051-2059
DISCLOSURE: S. Yakoob, None.
PAINLESS AORTIC DISSECTION PRESENTED AS CEREBRAL
INFARCTION
Joe G. Zein, MD*; Linda Kirschenbaum, DO; Mark E. Astiz, MD, Saint
Vincent Hospital and Medical Center, New York, NY
INTRODUCTION: Aortic dissection is a serious condition with
considerable morbidity and mortality. Frequently it manifests with severe
chest pain, however 15 % of patients may present with painless aortic
dissection (1). Aortic dissection may rarely extend into the common
carotid artery and lead to cerebral infarction. We are reporting a case of
painless aortic dissection presenting as right cerebral hemispheric infarct.
CASE PRESENTATION: A 43-year-old man with a history of diet-
controlled hypercholesterolemia collapsed after ice-skating. When he
arrived at the emergency department he was alert and awake. He
complained only of sudden onset of left-sided weakness. He denied any
trauma, chest pain, or shortness of breath. He was noted to have a left
hemiplegia. Arterial pulses were normal. The rest of physical exam and
laboratories values were normal. Urine sent for toxicology was negative.
Shortly thereafter the patient became obtunded, developed generalized
tonic clonic seizures and was intubated for airway protection. At this time
he was found to have right forced gaze deviation, increased left side deep
tendon reflex, and clonus was elicited while testing the left Achilles’ reflex.
A non-contrast head CT showed well-defined areas of decreased attenu-
ation within the right anterior cerebral artery (ACA) and middle cerebral
artery (MCA) territories. A portable chest radiograph was normal. After
sedation, he required low doses of vasopressors for blood pressure
support. Over the next twenty-four hours with hydration, the BP im-
proved to 125/75 mmHg, and the vasopressors were tapered off. However
urine output remained marginal, and gross hematuria was observed. His
repeat neurologic exam was unchanged. Mild pulmonary vascular conges-
tion appeared on a follow up chest x-ray and a transthoracic echocardio-
gram revealed a prominent aorta with mild to moderate aortic insuffi-
ciency. A repeat head CT was unchanged. A brain MRI-MRA showed
acute infarction within right ACA and MCA territories. Bilateral Comon
carotid and right Internal carotid artery dissection was seen. An emergent
transesophageal echocardiography revealed an ascending aortic dissec-
tion. There was no evidence of congenital aortic valve disease. On hospital
day 3, the patient acutely became bradycardic and both pupils were noted
to be fixed and dilated. He subsequently developed asystole and died.
DISCUSSION: Acute aortic dissection is the most common fatal
condition that involves the aorta. Systemic hypertension is a coexisting
condition in 50% of patients presenting with aortic dissection. Other
common predisposing factors include congenital unicuspid or bicuspid
valve disease, coarctation of the aorta, Marfan or Ehlers-Danlos syn-
drome, cystic medionecrosis, and cocaine use. Acute onset chest pain,
which is the predominant symptom in approximately 90% pf patients, is
often described as very severe, tearing, and radiating to the back. .
Although hypertension is present in more than 50% of patients who
present in ascending aortic dissection, only 10-30% of the patients are
hypertensive on presentation. In fact, 20-25% may present with hypoten-
sion.
The clinical manifestations may reflect complications resulting from the
occlusion of major arteries by the false lumen formed by the dissection.
Neurologic findings may be due to carotid artery obstruction (hemiplegia,
hemianesthesia) or spinal cord ischemia (paraplegia). Horner’s syndrome,
superior vena caval syndrome, hoarseness, dysphagia, and airway compro-
mise may result from the compression of adjacent structures by the
expanding dissection. Proximal aortic dissection is frequently complicated
by acute MI, acute aortic regurgitation (⬎50%) and hemopericardium.
CONCLUSION: Painless aortic dissection is a rare cause of cerebro-
vascular accidents. It should be suspected in patients presenting with
stroke mainly in the absence of other risk factors. Earlier diagnosis and
treatment may be lifesaving.
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REFERENCES:
1. Spittell PC, Spittell JA, et al. Clinical features and differential
diagnosis of aortic dissection: experience with 236 cases (1980
through 1990). Mayo Clin Proc 1993Jul;68(7):642-51
DISCLOSURE: J.G. Zein, None.
SPONTANEOUS MEDIASTINAL HEMATOMA IN AN ANTICO-
AGULATED PATIENT
Evan R. Restelli, DO*; Brian Carlin, MD, FCCP; Lawrence Crist, DO;
Robert Keenan, MD. Allegheny General Hospital, Pittsburgh, PA
INTRODUCTION: Mediastinal hematoma is most commonly associ-
ated with trauma, thoracic surgery, cancer, cardiac catheterizations, and
bronchoscopy. They are rarely associated with anticoagulation alone. We
present a case of spontaneous mediastinal hematoma occurring in a
patient who was receiving warfarin.
CASE PRESENTATION: A 79-year-old female with a history of
recent onset atrial fibrillation, managed with warfarin, presented with
abdominal and lower chest pain. A chest roentgenogram showed a
widened mediastinum. Her hemoglobin was 7.4 mg/dl and INR 1.7. A CT
scan showed a large retrocardiac lower mediastinal abnormality (consis-
tency of hematoma) with extension into the subcarina and right hilar
region, and an enlarged paratracheal lymph node. A thoracic aortic
dissection was suspected, but a thoracic aortogram was normal. A
mediastinal hematoma was suspected and a mediastinoscopy was pre-
formed demonstrating blood clots in the subcarinal region. A left pleural
chest tube was placed with removal of bloody fluid (800 cc). No apparent
etiology of the hematoma was found. A level 4R lymph node and
subcarinal tissue were benign. Pleural fluid analysis revealed: red blood
cells 1440000 U/L, white blood cells 3700 U/L and subsequent cultures
were negative. She had an uneventful post-operative course and has been
managed with aspirin therapy. She is doing well eighteen months later.
DISCUSSION: Spontaneous mediastinal hematomas are rare compli-
cations associated with anticoagulation. They are most commonly associ-
ated with trauma and aortic dissection/rupture. The optimal management
of mediastinal hematoma is to exclude catastrophic aortic disease. Once
such causes of the hematoma are excluded, surgical intervention may be
necessary to determine the etiology. Small hematomas have been docu-
mented to resolve spontaneously, but large hematomas can cause signif-
icant morbidity by causing compression of the heart and pulmonary
outflow tract. Due to these potential complications, surgical evacuation is
recommended even if the patient is asymptomatic.
CONCLUSION: This unique case of spontaneous mediastinal hema-
toma occurred in a patient who was anticoagulated. Recognition of this as
a potential cause for mediastinal widening can lead to appropriate
diagnosis and successful treatment.
REFERENCES:
Sloan, Timothy J., Burch, Buford H., Large Mediastinal Hematomas
Not Associated with Aortic Rupture. Chest 83(1): 1983 109-111
Turetz, F., Steinberg, H, Kahnn, A., Spontaneous Anterior
Mediastinal Hematoma: A Complication of Heparin Therapy. Journal
of American Medical Womens Association 32(2) : 1979 85-8
Agil,L.L., Trisolini R., Burzi M., Patelli M., Mediastinal hematoma
follwoing transbronchial needle aspiration. Chest 122(3) : 2002 1106-7
DISCLOSURE: E.R. Restelli, None.
FULMINANT CLOSTRIDIAL SEPSIS PRESENTING AS HEMO-
LYSIS IN A PATIENT UNDERGOING CHEMOTHERAPY FOR
MANTLE-CELL LYMPHOMA
James E. Shamiyeh, MD*. University of Alabama at Birmingham, Bir-
mingham, AL
INTRODUCTION: When caring for critically-ill patients with hema-
tologic malignancy, the physician must be especially aware of the behavior
of the disease itself as well as side effects of various chemotherapeutic
regimens. However, even when the illness involves primarily hematologic
abnormalities, another process with hematologic features may be respon-
sible. Here we report a case of fulminant clostridial sepsis in a patient with
mantle-cell lymphoma.
CASE PRESENTATIONS: The patient is a 40 year-old white male
with no past medical history other than mantle-cell lymphoma. He
recently began a chemotherapy protocol involving treatment with an
anti-BCL-2 antibody. He developed painful abdominal lymphadenitis (the
most common side-effect of the antibody) and was readmitted for pain
control. At that time physical exam was remarkable only for abdominal
tenderness and a palpable spleen. Cat scan (CT) of the abdomen revealed
that marked splenomegaly and abdominal adenopathy had not worsened
since a prior exam. During the middle of the night before anticipated
discharge, he developed fever of 101.4. Cultures were obtained from
peripheral site and Hickman catheter along with routine labs. Broad
spectrum antibiotics were begun. He then developed altered mental
status including slurring of his speech and lethargy and underwent
noncontrast head CT which was normal. Shortly after return from CT, he
became hypotensive and less responsive and was intubated. At this time
lab work revealed that his hematocrit (HCT) had dropped from 25 to 12.
Patient received O-negative packed-red-blood-cells (PRBCs) and under-
went CT scan of the abdomen because of concern for splenic hemorrhage.
CT scan revealed interval development of multiple small parenchymal gas
collections within the liver suggestive of septic embolic disease. Upon
return from CT scan patient became progressively more hemodynamically
unstable. Following transfusion of four units his HCT had dropped to a
value quantified as “extreme low.” Further labs indicated indirect biliru-
bin 7.4, AST 283, ALT 85, LDH 3434. Arterial blood gases revealed
steady increase in percent methemoglobin from negligible to 5% to 9%.
Urine output became dark-brown, and patient became anuric with
subsequent initiation of continuous-veno-venous-hemodialysis. Blood cul-
tures returned after 12 hours positive for Gram-positive rods, subse-
quently identified as clostridium perfringens. Methemoglobinemia was
treated twice with methylene blue with partial response. However, patient
developed hyperkalemia refractory to all forms of medical therapy, and he
expired following establishment of do-not-resuscitate status.
DISCUSSIONS: Clostridium species account for less than 3% of all
blood cultures, but the higher rates seen in cancer hospitals reflect the
importance of underlying intestinal carcinoma and leukemia. Most cases
of clostridial bacteremia do not result in the fulminant picture described,
but when associated with intravascular hemolysis the condition is almost
universally fatal.(1). Massive hemolysis is this case was presumably due to
phospholipase C, one of many toxins that can be produced by the
organism. Methemoglobinemia was an unusual feature of this case but is
not specific to clostridial sepsis. It has been reported that large amounts
of nitric oxide (NO) are released in patients with sepsis, and NO is
converted to methemoglobin(2). The decision to treat methemoglobin-
emia is this case was based on the presence of multiple other insults to
oxygen delivery.
CONCLUSIONS: Fulminant sepsis with associated intravascular he-
molysis due to bacteremia from clostridium perfringens is an almost
universally fatal condition despite appropriate antibiotic treatment and
supportive care. However, recognition of this unusual entity can lead to
directed therapy and potentially aid in counseling of family members of
the critically ill patient.
REFERENCES:
1. Rechner, P et al. Clinical features of clostridial bacteremia. A review
from a rural area. Clinical Infect Dis 2001;33:349-353.
2. Ohashi, K et al. Elevated methemoglobin in patients with sepsis.
Acta Anaesthesiologica Scandinavica. 1998;42:713-716.
DISCLOSURE: J.E. Shamiyeh, None.
ACUTE LUNG INJURY SECONDARY TO VENOUS AIR EMBO-
LISM FOLLOWING REMOVAL OF VASCULAR CATHETER
Muhammad Shibli, MD.*; Ruth Minkin, MD.; Hassan Khouli, MD;
Raymonde Jean, MD. Columbia University College of Physicians &
Surgeons/St. Luke’s-Roosevelt Hospital Center, New York, NY
INTRODUCTION: Venous air embolism (VAE) is an iatrogenic
process involving the entrapment of air into the venous system causing
acute occlusion of pulmonary arterial circulation resulting in sudden
cardiovascular collapse. We report a case of VAE resulting in acute lung
injury following removal of a vascular catheter.
CASE PRESENTATIONS: 38 years old woman with history of
multiple sclerosis admitted for plasmapheresis treatment. She underwent
placement of a right intra-jugular 15-cm Schon XL vascular catheter. She
received 3 cycles of plasmapheresis, and the catheter was removed while
she was in the supine position. Shortly after that, she reported sudden
severe chest pain, shortness of breath, dizziness and near fainting. She was
in severe distress, tachycardic and hypotensive that responded to fluids.
Arterial PO2 was 39mm Hg on room air. Chest x-ray was normal.
Echocardiography revealed dilated right ventricle (RV). She was placed
on 100% oxygen; put in supine position, started on IV hydration and
catheter site was securely covered. She continued to be tachypneic, her
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CASE REPORTS

WBC count increased to 66200 and hematocrit to 51.1 mg/dl. Repeated
chest x-ray revealed bilateral vascular congestion and large bilateral
pleural effusion. Her clinical picture was consistent with non-cardiogenic
pulmonary edema from capillary leak syndrome secondary to gas embo-
lism. She improved with supportive measures; and her laboratory values,
radiographic and echocardiographic abnormalities normalized. She was
discharged 7 days later.
DISCUSSIONS: VAE is known to occur during catheter insertion
however, it could also happen during catheter removal due to pressure
gradient difference between the atmosphere and the venous system. Fatal air
embolus volumes causing circulatory collapse are estimated to be 3-8 ml/Kg.
Predictors of severity of VAE include volume of air embolus, rate of entry,
duration of air entrapment, pre-existing medical conditions and body position.
RV outflow obstruction with air causing severe gas exchange abnormalities,
right heart strain, paradoxical embolism and may lead to circulatory arrest.
The presence of air in the RV causes RBC and platelets aggregation with
formation of fibrin and microthrombi, which later embolize to the distal
pulmonary capillary bed. That causes local endothelial damage and comple-
ment cascade activation with the release of different inflammatory and
cytotoxic mediators. The end result of this process in the lung is capillary leak
syndrome with extravascular fluid shift precipitating a picture of non-
cardiogenic pulmonary edema. Diagnosis relies on the recognition of the
clinical picture in the proper setting. Transesophageal echocardiography is a
sensitive method for detection of the embolus in the heart chambers.
Treatment is supportive. High concentration of inspired oxygen reduces the
gas embolus volume by displacing the nitrogen out of the air bubbles. Supine
position is recommended over the trendelenberg left lateral position. Increas-
ing the central venous pressure is achieved by IV fluid administration to
prevent further air entry. Hyperbaric oxygen treatment is useful in particular
with CNS involvement. Our patient had the clinical picture of acute lung
injury and capillary leak syndrome with evidence of non-cardiogenic pulmo-
nary edema. Leukocytosis and elevated hematocrit are due to severe hypox-
emia, stress and fluid shift. All of these changes normalized with supportive
care and restoration of normal oxygenation, circulation and adequate tissue
perfusion.
CONCLUSIONS: VAE is a catastrophic complication of vascular
catheter removal. It causes an acute circulatory compromise with end
organ damage. Acute lung injury with capillary leak syndrome resulting in
non-cardiogenic pulmonary edema and extravascular fluid shift could
occur as a result of this complication. Prevention of further air entry and
the institution of other supportive measures are critical in the manage-
ment of these patients.
REFERENCES:
1- Muth CM et al. Gas embolism. NEJM 2000;342(7):476-82.
2- Andrews CM et al. Preventing air embolism. AJN 2002;102(1):34-6.
DISCLOSURE: M. Shibli, None.
Wednesday, October 29, 2003
Miscellaneous Case Reports
2:00 PM - 3:30 PM
CYCLIC CUSHING’S SYNDROME CAUSED BY PERIODIC SE-
CRETION OF ACTH FROM A BRONCHIAL CARCINOID TUMOR
Douglas S. Frenia, MD*. Walter Reed Army Medical Center, Washing-
ton, DC
INTRODUCTION: Ectopic production of adrenocotropic hormone
(ACTH) accounts for approximately 15% of cases of Cushing’s syndrome.
Several types of malignant and non-malignant tumors of neuroendocrine
origin have been implicated in ectopic ACTH production. Bronchial
carcinoids account for approximately one quarter of such cases. Periodic
hormonogenesis by ectopic ACTH producing tumors other than bronchial
carcinoid is an unusual phenomenon that has been reported in the
endocrine literature. The following represents what is believed to be the
first reported case of a bronchial carcinoid with periodic ACTH produc-
tion causing cyclic Cushing’s syndrome.
CASE PRESENTATION: A 67 year old female presented with
Cushing’s syndrome manifested by generalized fatigue, weakness,
hypertension, Cushingoid appearance, thrush, hyperkalemia and hy-
perglycemia. The patient had experienced a similar episode of Cush-
ing’s syndrome four years prior to presentation. Evaluation at that time
did not reveal an etiology, and the syndrome resolved spontaneously.
In interim follow-up until the recent past, the patient remained
asymptomatic with normal ACTH and cortisol levels. On exam, the
patient showed a marked Cushingoid appearance. There were whitish
plaques on her oral mucosa, consistent with thrush. Her BP was 168/96
mm Hg. Laboratory evaluation revealed a potassium of 2.7 mmol/L,
glucose of 279 mg/dL, ACTH of 101 pg/dL (normal 5-50 pg/dL),
cortisol of 77 mcg/dL (normal 5-25 mcg/dL) and 24-hour urine
cortisol ⬎ 6000 mcg (normal 3-34 mcg/dL). An MRI of the brain was
normal. Somatostatin receptor scintigraphy with [111-In] pentetreo-
tide did not show suspicious uptake. CT of chest and abdomen
revealed an 8 mm right middle lobe nodule. Her hypokalemia was
corrected and she was begun on metyrapone and ketoconazole to
inhibit cortisol production. A cardiothoracic surgery consult was
obtained and the patient underwent resection of the right middle lobe
nodule. Pathology revealed a typical carcinoid which stained positive
for chromogranin A and ACTH. On follow-up, the patient’s symptoms
and metabolic abnormalities have resolved. ACTH and cortisol levels
have normalized.
DISCUSSION: Ectopic production of ACTH leading to hypercor-
tisolism and Cushing’s syndrome is thought to occur in approximately
1% of bronchial carcinoids. As in this patient, diagnosis of these tumors
is often delayed for years after Cushing’s syndrome becomes apparent.
There are several likely explanations for such delay. First, these tumors
are generally small, averaging about 1.1 cm at the time of diagnosis.
Conventional imaging modalities such as CT and MRI have a sensi-
tivity for these lesions which is considerably less than 100%. More
recently, attempts at localization of ACTH producing lesions with
[111-In] pentetreotide scintigraphy have proven somewhat disappoint-
ing, with sensitivities of 25-65%. In addition, up to 30% of ectopic
ACTH producing lesions will show suppression on dexamethasone
testing, thus making differentiation from pituitary lesions difficult
without petrosal venous sampling. In this case, the unusual cyclic
presentation was likely a significant contributing factor in the delay in
diagnosis. Cyclic Cushing’s syndrome is defined by a pattern of
hypercortisolism with intermittent periods of normal cortisol produc-
tion. This pattern was documented in this patient by surveillance of
ACTH and cortisol levels in the period between her episodes of
Cushing’s syndrome. Reports in the endocrine literature describe cases
of cyclic Cushing’s syndrome related to various types of ACTH
producing neuroendocrine tumors including pituitary adenoma, thy-
mic carcinoid and apudoma. However, this is thought to be the first
reported case of an ectopic ACTH producing bronchial carcinoid
causing cyclic Cushing’s syndrome. The etiology of the periodicity of
hormone production in these tumors is unknown.
CONCLUSION: Ectopic ACTH producing bronchial carcinoids can
exhibit periodicity of hormonogenesis such as that reported in other types
of ectopic ACTH producing tumors. This characteristic leads to the entity
of cyclic Cushing’s syndrome.
DISCLOSURE: D.S. Frenia, None.
HARD ROCK ARTERIES: A CASE OF A POST-OPERATIVE
COMPLICATION
Ashgan A. Elshinawy, DO*; Dorothy A. White, MD, FCCP; Diane E.
Stover, MD, FCCP. Memorial Sloan-Kettering Cancer Center, New York,
NY
INTRODUCTION: Percutaneous kyphoplasty using polymethyl-
methacrylate (PMMA) has recently become available to provide spine
stabilization and pain relief for compression fractures due to osteoporosis
or osteolytic spinal metastasis.
1
The procedure is being increasingly
utilized with good results but is associated with a rare complication of
cement embolization to the pulmonary arteries. We report such a case to
help familiarize pulmonologists with this entity.
CASE PRESENTATION: A 62-year-old man with MM and multiple
vertebral compression fractures underwent a percutaneous kyphoplasty
using PMMA under general anesthesia. He has a history of mild CAD and
a pulmonary embolism (PE). He had been taking coumadin, which was
held for the procedure.
A needle was placed transpedicularly into the vertebral bodies (L1-L3).
A total of 4cc of PMMA was injected in each pedicle at all levels, under
Tuesday, October 28, 2003
Critical Care II, continued
304S CHEST 2003—Case Reports

fluoroscopy. At the level of L2, extravasation of cement was visualized in
the paravertebral venous system, extending approximately 3-4 cm distally.
The patient remained stable.
While being monitored in the recovery unit, his vitals were: tempera-
ture ⫽ 38.4 C, HR ⫽ 100-110 (sinus tachycardia), systolic BP ⫽ 140.
Oxygen at 4 liters/min by nasal cannula was required to maintainO
2
saturation ⬎ 90%. On exam, he had bibasilar crackles. The patient had no
respiratory complaints. He received 5mg of IV Lopressor for cardiopro-
tection, which resulted in hypotension with a SBP in the 70-80 range for
several hours, despite fluid boluses.
The patient was treated with IV antibiotics, anticoagulation and incen-
tive spirometry. The fever, tachycardia, hypotension and hypoxemia all
resolved within 24 hours. He was discharged home three days after
surgery. His baseline back pain had improved.
DISCUSSION: Kyphoplasty involves the percutaneous injection of
bone cement under fluoroscopic guidance into a collapsed vertebra. Case
reports suggest at least 67% of patients experience immediate pain relief.
Slight leakage of injected cement in the paravertebral venous system can
occur in up to 65% of cases, but in a few cases cement can reach the
inferior vena cava and then, the pulmonary arteries.
1-3
Clinical impact has
ranged from asymptomatic to pulmonary infarct and hypoxemia. Risk
factors for occurrence of cement emboli include incorrect needle posi-
tioning, inadequate polymerization of the PMMA before injection, lack of
biplane fluoroscopy, degree of fracture pattern and anomalous vascular
anatomy.
1-2
In our case multiple vertebrae were involved requiring a
greater total amount of cement and the vertebral bodies were ex-
tremely disrupted.
Treatment includes supportive care and anticoagulation to prevent
clotting around the foreign material. One case of pulmonary artery
embolectomy of the material was reported in a patient with extensive
embolization with good results.
4
The differential diagnosis of post-operative distress in this patient
included atelactasis, pneumonia, pulmonary embolus and fluid overload.
These were all considered but CXR and CT suggested cement PE and
aspiration pneumonia.
CONCLUSION: With increasing use of kyphoplasty, pulmonologists
should be aware of the possibility of cement emboli. These can be
suggested by CXR and diagnosed by CT. Besides supportive care,
treatment must be individualized to the given case. It is not known
whether the presence of cement embolus will have an adverse impact on
the patient’s lung function.
REFERENCES:
1. Bernhard J, et al. Asymptomatic diffuse pulmonary embolism
caused by acrylic cement: an unusual complication of percutaneous
vertebroplasty. Ann Rheum Dis. 2003; 62(1): 85-6.
2. Padovani B, et al. Pulmonary embolism caused by acrylic cement: a
rare complication of percutaneous vertebroplasty. AJNR. 1999;
20(3): 375-7.
3. Jang JS, et al. PE of PMMA after percutaneous vertebroplasty: A
report of three cases. Spine. 2002; 27(19): E416-18.
4. Tozzi P, et al. Management of PE during acrylic vertebroplasty.
Annals of Thoracic Surgery. 2002; 74: 1706-8.
DISCLOSURE: A.A. Elshinawy, None.
INTRALOBAR BRONCHOPULMONARY SEQUESTRATIONS AS-
SOCIATED WITH BRONCHOGENIC CYSTS
Christopher J. Lettieri, MD*. Walter Reed Army Medical Center,
Washington, DC
INTRODUCTION: Extralobar bronchopulmonary sequestrations
(ELS) and bronchogenic cysts are pulmonary congenital anomalies arising
from ventral foregut malformations. Although uncommon, foregut mal-
formations frequently occur together. Intralobar bronchopulmonary se-
Wednesday, October 29, 2003
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 305S
CASE REPORTS

questrations (ILS), however, are rarely associated with bronchogenic cysts
or other congenital lesions. This phenomenon raises controversy whether
the origin of ILS is congenital or acquired. We present three cases of ILS
occurring with a bronchogenic cyst, supporting that it is a congenital, not
acquired lesion.
CASE PRESENTATION: Case 1: 47-year-old woman presents with
recurrent right lower lobe pneumonia since early adulthood. She was a
non-smoker, denied tuberculosis exposure or risk factors for HIV and
aspiration. Chest radiographs (CXR) revealed a right lower lobe opacity,
which had been present on prior images. Computed tomography (CT)
showed a right posterior basal infiltrate and 2-cm mediastinal cyst. An
aberrant aortic branch directly feeding the posterior basal segment was
identified. She was diagnosed with an ILS and underwent surgical
resection. Pathology confirmed the cyst-like structure to be a broncho-
genic cyst.
Case 2: 44-year-old, asymptomatic woman referred for a mass inciden-
tally noted on CXR. She was a non-smoker. Review of systems was
unremarkable. CXR showed a 3-cm, well-circumscribed mass in the right
lower lobe. CT revealed consolidation of the medial basal segment and
2.7-cm cystic lesion with mediastinal involvement. An aberrant branch of
the aorta supplying this segment was identified. She was diagnosed with
an ILS and underwent resection. Pathology confirmed the lesion to be a
bronchogenic cyst.
Case 3: 39-year-old, asymptomatic female referred for PPD-conver-
sion. Screening CXR revealed a 3-cm superior mediastinal mass and right
lower lobe infiltrate. Sputum was negative for acid-fast bacilli. Subsequent
CT showed a 3.2-cm mediastinal cyst and an aberrant artery branching
from the aorta, supplying a consolidated right posterior basal segment.
ILS was diagnosed with subsequent resection of the cyst and right lower
lobe. The mass was confirmed to be a bronchogenic cyst on pathology.
DISCUSSION: Bronchogenic cysts are congenital anomalies arising
from the ventral foregut. They are typically located in the mediastinum
and commonly present with other congenital malformations. Bronchopul-
monary sequestrations are congenital malformations of non-functioning
pulmonary tissue that have inadequate communication with the tracheo-
bronchial tree. They also derive from the ventral aspect of the primitive
foregut resulting in aberrant development of the tracheobronchial tree.
There are two forms of sequestration, extra and intralobar. ELS are
associated with other congenital, foregut malformations in 60% of cases,
commonly diaphragmatic hernias and bronchogenic cysts. However,
congenital anomalies are rarely associated with ILS, leading to the theory
that these may be acquired lesions, resulting from chronic inflammation
or recurrent infections. In the three presented cases, an ILS was
associated with a congenital bronchogenic cyst. Two were not associated
with prior infections, suggesting that one anomaly was not the result of the
other. The associated cyst supports that ILS is a congenital malformation
and not an acquired lesion resulting from recurrent infections.
Sequestrations are supplied by aberrant systemic arteries. Demonstra-
tion of this vessel confirms the diagnosis and guides surgical resection.
Angiography is considered the gold standard. However, newer, less-
invasive imaging techniques are equally effective; including CT angio-
gram, Doppler ultrasound and magnetic resonance angiography.
CONCLUSION: Although uncommon, ILS can be associated with
bronchogenic cysts, further supporting the theory that these are congen-
ital, foregut malformations and not acquired lesions. CT angiography is an
invaluable diagnostic tool that can both demonstrate the aberrant arterial
supply and identify other anomalous lesions.
DISCLOSURE: C.J. Lettieri, None.
NOCTURNAL HYPOGLYCEMIA ASSOCIATED WITH BIZARRE
NIGHTTIME BEHAVIORS
Anita Naik, DO*; Karl Doghramji, MD; Alan D. Haber, MD. Graduate
Hospital, Jefferson Sleep Disorders Center, Philadelphia, PA
INTRODUCTION: Parasomnias and seizures can produce physical
phenomena during sleep, but specific triggering factors are incompletely
understood. We present a case of a gentleman with agitated nighttime
behaviors apparently induced by nocturnal hypoglycemia.
CASE PRESENTATION: A 73 year-old male presented with com-
plaints of disruptive and violent movements during sleep that his wife had
noticed over the past 6 months. Sleepwalking, yelling, growling like an
animal, leaving the room in disarray and knocking down furniture marked
these bizarre behaviors. Once she had found him trying to climb out the
window. When she attempted to stop him he yelled and punched her. She
had found him naked on the floor in a fetal position on several occasions.
Episodes numbered 1-2 times weekly, usually in the first half of the night.
The patient had no recollection of these events. His medical history was
significant for insulin-requiring type II diabetes. The sleep history was
remarkable only for snoring. His wife reported that his behaviors started
at the time his nighttime insulin dosage was increased. He was usually too
violent during the episodes to obtain a finger-stick glucose reading. The
general and neurological exams were unremarkable. On the night of his
sleep study similar combative behavior, yelling, and crawling occurred in
an apparent sleep state before the recording was started. Several people
were needed to restrain him. In addition the patient had urinary
incontinence and diaphoresis. Immediate evaluation in the emergency
room revealed a serum glucose of 33 mg/dl. His symptoms promptly
resolved with administration of intravenous dextrose. He returned to the
sleep lab the same night for completion of his study, which eventually
showed frequent periodic limb movements with arousals and mild
obstructive sleep apnea (respiratory disturbance index: 10). Full montage
electroencephalogram (EEG) monitoring did not show epileptiform
discharges. Prior daytime EEG monitoring was also unremarkable. His
nighttime insulin dose was subsequently lowered with complete resolution
of nocturnal behaviors. He also received clonazepam for periodic limb
movement disorder.
DISCUSSION: The differential diagnosis for bizarre nocturnal behav-
iors includes REM sleep behavior disorder (RBD), arousal disorders,
sleep-related epilepsy and pseudoseizures. Our patient exhibited features
that overlapped among these entities. The behaviors were complex,
purposeful and elderly onset—all characteristics of RBD— but occurred
early during the sleep cycle and were not related to dream content. The
timing of the events and the lack of recall are instead consistent with
disorders of arousal such as sleepwalking and confusional states. However
these rarely produce such vigorous motor activity and typically occur in
younger individuals. Nocturnal seizures generally trigger stereotyped
movements rather than the complex actions exhibited by our patient. Yet
the occurrence of incontinence is supportive, and frontal seizures can
present with behavioral motor changes and paradoxical post-ictal agita-
tion. The absence of epileptiform discharges on nocturnal and daytime
EEG does not exclude this disorder. Unfortunately the inability to capture
the event during polysomnography precludes an EEG-based specific
diagnosis. The clinical course however strongly implicates nocturnal
hypoglycemia in connection with the abnormal behaviors. Either noctur-
nal hypoglycemia produced a wakeful episode of bizarre activity, or it
triggered a parasomnia or seizure. Since hypoglycemia alone usually
manifests as obtundation and diaphoresis rather than vigorous, purposeful
behavior, we favor the latter hypothesis. That our patient exhibited his
bizarre activity only during apparent sleep states further implicates
hypoglycemia as a trigger for a specific sleep disorder.
CONCLUSION: Nocturnal hypoglycemia should be considered in
diabetic patients who present with abnormal physical behaviors at night.
Hypoglycemia may lower the seizure threshold or unmask an underlying
predisposition for parasomias such as RBD or arousal disorders.
DISCLOSURE: A. Naik, None.
NARCOLEPSY FOLLOWING AN INSECT BITE
Shoaib Alam, MD*; Kevin Gleeson, MD; Kathleen M. Chaisson, MD.
Affiliate, Penn State University-Milton S. Hershey Medical Center,
Hershey, PA
INTRODUCTION: Narcolepsy is the second most common cause of
excessive daytime sleepiness. It affects 0.03-0.06 percent of population in
North America and Western Europe. The typical age of onset of
symptoms is second decade of life.
CASE PRESENTATION: A 52-year-old male with no history of
excessive daytime sleepiness suffered insect bite on his back during an
outdoor picnic. The bite produced an unusually large local reaction. Three
weeks later, he developed episodes of generalized weakness requiring that
he lie down, in association with strong emotions such as anger and
laughter, persisting for several minutes. In addition, he started having
severe excessive daytime sleepiness. He was evaluated by a neurologist
and a psychiatrist and underwent testing, which included MRI of brain,
EEG and a nocturnal polysomnogram. Polysomnogram revealed very
short sleep-latency and no obstructive sleep apnea. Later, a multiple sleep
latency test (MSLT) confirmed the diagnosis of narcolepsy with mean
sleep latency of 2.5 minutes and no sleep onset REM periods. Rest of his
work-up remained non-revealing. He was then treated by a sleep physi-
cian with high doses of Methylphenidate and Paroxetine, which signifi-
Wednesday, October 29, 2003
Miscellaneous Case Reports, continued
306S CHEST 2003—Case Reports

cantly improved his daytime sleepiness and cataplexy. Now, some ten
years after the onset of his symptoms he continues to have at least some
excessive daytime sleepiness and occasional cataplexy despite the above
medication.
DISCUSSION: The exact pathophysiology of narcolepsy remains
unknown. Neurochemical studies of human, canine and murine narco-
lepsy suggest that narcolepsy is associated with low levels of Hypocretin in
cerebrospinal fluid(CSF). Several genetic, neurochemical and autoim-
mune associations have been described as potential etiologies for the
characteristic pattern of neuronal destruction which results in low Hypo-
cretin levels. Possibly, narcolepsy can be a result of an autoimmune
process which develops in genetically predisposed humans. Environmen-
tal and other host factors, which contribute to the development of this
autoimmunity, are not clearly understood.
CONCLUSION: The precipitous evolution of full blown classical
narcolepsy with cataplexy in this older man, following an unusually severe
reaction to an insect bite, suggests the possibility of immunologic mech-
anism as the basis for the development of narcolepsy.
This lends support to the hypothesis that immune mediated neuronal
destruction results in the development of narcolepsy.
REFERENCES:
1. Thannickal, T.C., Moore, R.Y., Nienhuis, R., et al. Reduced number
of hypocretin neurons in human narcolepsy. Neuron 2000; 27:469.
2. Nishino, S., Ripley, B., Overeem, S., et al. Hypocretin(orexin)
deficiency in human narcolepsy. Lancet 2000; 355:39.
DISCLOSURE: S. Alam, None.
REFRACTORY WHEEZING: AN UNUSUAL PRESENTATION OF
ISOLATED UNILATERAL PULMONARY ARTERY AGENESIS
Andreea L. Antonescu-Turcu, MD*; Sangeeta Bhorade, MD. Loyola
University Medical Center, Maywood, IL
INTRODUCTION: Unilateral pulmonary artery agenesis (UPAA) is a
rare congenital anomaly, which is frequently diagnosed during childhood
due to associated cardiovascular abnormalities. Isolated UPAA is even less
frequent; usually patients are asymptomatic and are diagnosed during
adulthood after an abnormal CXR.
CASE PRESENTATION: A 41-year-old man presented with a 5-day
history of worsening shortness of breath associated with chest tightness.
He was diagnosed with Mycobacterium avium-intracellulare complex
(MAC) pneumonia 2 month prior to this episode and has been treated
with antituberculous medications. He has history of chronic bronchitis
and was diagnosed with severe asthma as an adult requiring chronic
steroids.
Physical exam revealed a comfortable looking man, tachypneic, in no
distress. Pulse oximetry revealed 72% oxygen saturation on room air.
Chest exam revealed diffuse wheezing and right basilar crackles. The rest
of his exam was unremarkable.
CXR revealed a right-sided aortic arch and hyperlucent, contracted left
lung. CT of the chest showed an absent left pulmonary artery and ground
glass infiltrate in the right base. V/Q scan showed absent perfusion to the
entire left lung in the presence of relatively normal ventilation. Echocar-
diogram did not demonstrate other cardiac anomalies.
DISCUSSION: First review of isolated UPAA cases was published in
1962. Some patients are asymptomatic that can lead to a significant delay
in the diagnosis. Recurrent pulmonary infections can be a frequent
symptom of isolated UPAA. Pathogenesis is unclear but includes impaired
mucociliary clearance and diminished delivery of inflammatory cells due
to absent pulmonary perfusion. The result is a relative immunodeficient
lung, which is susceptible even to opportunistic pathogens. Refractory
wheezing mislabeled, as severe asthma can be an unusual way of
presentation as these patients have bronchoconstriction due to alveolar
hypocapnea.
Diagnostic possibilities include CXR, echocardiogram along with high
resolution CT scan, MRI and V/Q scan. Pulmonary venous wedge
angiogram is indicated when revascularization is considered in order to
isolate the hilar arteries.
Principal differential diagnosis is Swyer-James or McLeod’s syndrome.
Diagnostic imaging of this syndrome include expiratory radiograph with
evidence of air trapping and ventilation lung scan showing a delay in the
washout phase.
CONCLUSIONS: Isolated UPAA can remain undetected until
adulthood. A high index of suspicion coupled with a thorough history,
physical examination and a set of imaging tests can lead to a correct
diagnosis.
This case illustrates that UPAA should remain in the differential
diagnosis of persistent wheezing despite the fact that this is not a usual
presentation of the disease.
REFERENCES:
1. CHEST 2002; 122:1471-1477
2. Annals of Thoracic Surgery 2002; 74: 2169-2171
DISCLOSURE: A.L. Antonescu-Turcu, None.
Upper Airway
2:00 PM - 3:30 PM
CRYPTOCOCCAL LARYNGITIS
Hassan F. Nadrous, MD*; Jay H. Ryu, MD; Jean Lewis, MD; Alain N.
Sabri, MD. Mayo Clinic, Rochester, MN
INTRODUCTION: Cryptococcal infection range from asymptomatic
pulmonary colonization to life-threatening meningitis.
1,2
. Laryngeal cryp-
tococcal infections are exceedingly rare.
CASE PRESENTATION: A 55-year-old man was evaluated for
hoarseness and worsening cough of 6 weeks’ duration. His past medical
history was notable for asthma, allergic fungal sinusitis, and sinus surgery.
His medications included inhaled albuterol, and flunisolide. He also had
stopped using fluticasone nasal spray recently. His physical examination
was unremarkable, except for a patch of leukoplakia on the right anterior
vocal cord with erythema of the entire right vocal cord noted on fiberoptic
endoscopy. Biopsy of the right vocal cord lesion was performed. GMS
stain of the biopsy specimen revealed the fungal organisms with morpho-
logic features of Cryptococcus Neoformans and the fungal culture grew
Cryptococcus Neoformans. Evaluation for cryptococcal dissemination was
negative.
Patient was treated by itraconazole 200 mg twice daily for 6 weeks
followed by fluconazole 400 mg a day for 10 additional weeks. Patient was
disease-free at the end of the treatment course and at 10-month follow-up
visit.
DISCUSSION: Our case represents the sixth case of laryngeal cryp-
tococcal infection in the literature.
The most common presenting symptom among these cases was hoarse-
ness and only one patient required tracheostomy
3
. In general, antifungal
therapy is recommended for most patients with cryptococcal infections,
especially those patients who are immunocompromised.
CONCLUSIONS: Cryptococcal laryngeal infection is rare but it
should be considered in the differential diagnosis of persistent hoarseness
occurring in an immunocompromised subject. . Inhaled corticosteroid
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 307S
CASE REPORTS

therapy may be a predisposing factor for cryptococcal laryngitis in
non-immunocompromised subjects.
REFERENCES:
1. Levitz SM. The ecology of Cryptococcus neoformans and the
epidemiology of cryptococcosis. Rev Infect Dis 1991;13:1163-9.
2. Rozenbaum R, Goncalves AJ. Clinical epidemiological study of 171
cases of cryptococcosis. Clin Infect Dis 1994;18:369-80.
3. Isaacson JE, Frable MA. Cryptococcosis of the larynx. Otolaryngol
Head Neck Surg 1996;114:106-9.
DISCLOSURE: H.F. Nadrous, None.
RESOLUTION OF SEVERE SLEEP DISORDERED BREATH-
ING BY A NASOPHARYNGEAL OBTURATOR IN A CASE OF
NASOPHARYNGEAL STENOSIS COMPLICATING UVULO-
PALATOPHARYNGOPLASTY
Alan J. DeAngelo, Pulmonary/CCM fellow*; Vincent Mysliwiec, Pulmo-
nary/CCM/Sleep Staff. Brooke Army Medical Center, Fort Sam Houston,
TX
INTRODUCTION: Obstructive sleep apnea-hypopnea syndrome
(OSAHS) is characterized by episodic decrements in airflow due to upper
airway obstruction producing arousals, oxyhemoglobin desaturation, and
daytime somnolence. Fujita et al.
1
first introduced uvulopalatopharyngo-
plasty (UPPP) as a therapy for OSAHS in 1981. Nasopharyngeal stenosis
is a rare complication of UPPP that worsens OSAHS. A paucity of
literature describing its therapy exists.
CASE: A 31-year old man was referred for OSAHS worsened by
nasopharyngeal stenosis complicating UPPP. Post-operative complaints
included velopharyngeal incompetence, distorted speech resonance, and
worsening of daytime somnolence. Polysomnography demonstrated se-
vere sleep disordered breathing with severe desaturations. He underwent
CO2 laser release of adhesions and placement of a nasopharyngeal
obturator. Follow-up polysomnogram demonstrated resolution of OSAHS
correlating with subjective resolution of daytime somnolence.
DISCUSSION: The reported efficacy of surgical therapy for OS-
AHS varies between 50-60%.
2
There are limited reports of severe
complications of UPPP with the incidence of nasopharyngeal stenosis
estimated at less than 1%.
3
Reported therapeutic modalities include
CO2 laser release of adhesions and nasopharyngeal obturator place-
ment, used in conjunction or separately.
3
Successful treatment has
been based on subjective improvement and post-operative lumen size
without mention of sleep disordered breathing. Optimal duration of
obturator placement is unknown and a post-intervention polysomno-
gram has not been reported.
CONCLUSION: Nasopharyngeal stenosis complicating UPPP can be
successfully treated with scar removal and nasopharyngeal stenting.
Polysomnographic demonstration of the effectiveness of this therapy has
not been previously reported. Important questions for future research
include duration of nasopharyngeal stenting and timing of follow-up
polysomnography.
REFERENCES:
1. Fujita S, Conway W, Zorick F, et al. Surgical correction of anatomic
abnormalities in obstructive sleep apnea syndrome: uvulopalatopharyngo-
plasty. Otolaryngol Head Neck Surg 1981;89:923-934.
2. Fujita S. UPPP for sleep apnea and snoring. Ear Nose Throat J
1984;63:227-235.
3. Krespi YP, Kacker A. Management of nasopharyngeal stenosis after
uvulopalatopharyngoplasty. Otolaryngol Head Neck Surg 2000;123:692-
695.
DISCLOSURE: A.J. DeAngelo, None.
SMALL CELL CANCER OF THE LARYNX MASQUERADING AS
BRONCHIAL ASTHMA
John Ronald, MD*; Sat Sharma, MBBS, FCCP. University of Manitoba,
Winnipeg, MB, Canada
INTRODUCTION: Upper airway obstruction is well described as a
cause of apparent asthma, unresponsive to the standard asthma therapy. A
careful clinical examination, high index of suspicion, and appropriate
analysis of spirometry is essential for the appropriate diagnosis and
management.
CASE PRESENTATION: A 25-year-old woman, previously healthy,
presented to the emergency room (ER) with dyspnea. She had smoked
cigarettes heavily for the past 10 years. Six weeks prior, she developed a
viral upper respiratory tract infection. She was diagnosed with asthma
possibly worsened by community-acquired pneumonia and was pre-
scribed antibiotics, salbutamol and fluticasone. The patient did not benefit
from this therapy and indeed felt that she had deteriorated.
She presented a second time to the ER with worsening dyspnea, where
she was found to have a room-air oxygen saturation of 95% and wheezing
that was heard throughout all lung fields. Spirometry at that time showed
a forced expiratory volume in 1 second (FEV
1.0
) of 1.6 L (47% predicted)
and a forced vital capacity (FVC) of 3.2 L (77% predicted). The ratio of
FEV
1.0
to FVC was 50%, consistent with the diagnosis of airflow obstruc-
tion. She was encouraged to continue bronchodilator therapy and received
a prescription for prednisone, and was sent home.
She returned the following day with severe dyspnea and was referred
for admission because of refractory asthma. Interestingly, inspiratory
stridor was recognized on this visit prompting a consult to the
otolaryngology service. Spirometry was repeated and considered to be
diagnostic of fixed upper airway obstruction. Endoscopy and subse-
quent computer-assisted tomography (CT) of the neck revealed a
subglottic mass approximately 3 cm in diameter. She underwent
emergent tracheostomy and examination under anesthesia. A mass
inferior to the left vocal cord was biopsied and later diagnosed as small
cell cancer of the larynx. Her post-operative course was complicated by
bilateral pneumothoraces and aspiration. She was staged as T4N0M0
and referred for chemotherapy and radiotherapy, as the tumour was
not resectable. She is in remission with combined cisplatin-based
chemotherapy and radiotherapy.
DISCUSSION: First observed in Canada in 1972, small cell cancers
of the larynx are rare. Only a few case reports are published in the
medical literature. These tumors are aggressive, classified under the
category of neuroendocrine tumors and are thought to arise from the
argyrophilic Kulchitsky cells normally found in laryngeal mucosa. The
distinct features on light microscopy suggest the diagnosis that can be
confirmed by electron microscopy. The differential diagnoses include
carcinoid, atypical carcinoid, small cell squamous carcinoma, small cell
ductal carcinoma, lymphoma, mycosis fungoides, and metastatic lung
small cell cancer. As with small cell cancers of the lung, they can be
associated with ectopic hormone production. The association with
smoking has not been definitely proven, as the incidence of this
neoplasm is so rare. Systemic chemotherapy with local radiation
therapy is the accepted therapeutic approach and may lead to long-
term remissions and even cure.
CONCLUSIONS: This case illustrates two distinct clinical features.
The previous cases of small cell laryngeal cancer have all been described
in the middle aged and elderly individuals. Our patient is the only young
adult to have developed this malignant neoplasm among all reported
cases. Upper airway obstruction is well described as a cause of apparent
asthma unresponsive to the standard asthma therapy. A careful clinical
examination and analysis of spirometry led to the appropriate diagnosis
and management. This case highlights the importance of formulating a
comprehensive differential diagnosis and following a systematic, anatom-
ical approach for work up of wheeze.
REFERENCES:
Soga J. Laryngeal endocrinomas: analysis of 278 reported cases. J
Exp Clin Cancer Res 2002; 21:5-13.
Hay JH. J Laryngol Otol 1981; 95:1081-88.
DISCLOSURE: J. Ronald, None.
VOCAL CORD AND TRACHEAL CARCINOID TUMOR
Satish C. Chada, MBBS*; Sujeeth Punnam, MD; Aparna Punnam, MBBS.
Downstate Medical Center, Brooklyn, NY
INTRODUCTION: Laryngeal neuroendocrine neoplasms are rare
neoplasms. Only 13 cases of well-differentiated carcinoid tumors of larynx
are reported so far. We present a case of elderly female who presented
with involvement of larynx, trachea and bronchi with carcinoid tumors
treated with local resection.
CASE PRESENTATION: A 69 year old black female with past
medical history of hypertension, coronary heart disease, congestive heart
failure, diabetes mellitus, 30 pack year smoking presented with 2 months
history of worsening heart failure symptoms with ocassional blood
streaked sputum, wheezing, hoarseness and an acute attack of supraven-
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308S CHEST 2003—Case Reports

tricular tachycardia.ct angiogram to rule out pulmonary embolism ex-
cluded embolism but showed nodular filling defects within the trachea
and bilateral mainstem bronchi. Bronchoscopy revealed right vocal cord
polyp and multiple polypoid lesions in trachea and bilateral bronchi.
Biopsy showed low grade, well differentiated neuroendocrine tumour
with tumor markers positive for moc 31, synaptophysin, chromogranin and
s100. Metastatic workup was negative and patient underwent sucessful
local resection of the tumors.
DISCUSSION: Carcinoids are neuroendocrine neoplasms. Most of the
carcinoids occur in appendix, rectum and ileum and they arise from
enterochromaffin cells. Pulmonary carcinoids arise from kulchitsky’s cells
and they constitute very small percentage among the carcinoid tumors.
Bronchial carcinoids usually involve the central airways and show well
defined margins. Histologically they vary from low grade typical to more
aggressive atypical ones. Similary very few typical laryngeal carcinoids
have been reported, most of them being atypical variants. Patients in 3
rd
to 7
th
decade are usually affected. Symptoms are cough, hemoptysis and
other local obstructive symptoms. Carcinoid syndrome is unusual in
laryngeal and bronchial carcinoids. Bronchoscopic biopsy is effective
usually given the central location of these tumors. Various tumor markers
like moc 31,protein s100 for sustentacular cells, chromogranin and
synaptophysin corroborate the diagnosis.
Treatment is based on the histology of the tumor and extent of
involvement. Most of them are amenable to local resection or endoscopic
ablation. Hilar and mediastinal lymphnode involvement could influence
resection procedure and long term outcome. Chemo and radiation
therapy are not effective in improving long term outcome in advanced
disease. Somatostatin analogues may be of benefit in early stages of
metastatic carcinoid tumors with carcinoid syndrome. However the
overrall 5-year survival rate in the typical variety following local resection
is around 90%.
CONCLUSION: To our knowledge this kind of a presentation of
carcinoid tumor is extremely rare. Because of the low incidence of
laryngeal and tracheal carcinoids it is important to explore the identity of
these tumors further to refine diagnostic and therauptic strategies suited
for dealing with this kind of tumors.
REFERENCES:
1) Otolaryngology Head and Neck Surgery April 1999, Vol 120 #4
2) Davila, Dg, Dum, Et Al (1993)- Bronchial Carcinoid Tumors. Mayo
Clinic Proc 68, 795-803
3) Lk Kvols, Cg Moertel, Mj O’connel Et Al (1986)- Treatment Of The
Malignant Carcinoid Syndrome: Evaluation Of A Long Acting
Somatostatin Analogue. Nejm 315, 663-666
DISCLOSURE: S.C. Chada, None.
A WOMAN WITH A PERSISTENT HOARSE VOICE AND STRI-
DOR SIX MONTHS FOLLOWING ESOPHAGOGASTRODUODE-
NOSCOPY
Samuel J. Evans, MD*; Brian M. Morrissey, MD; Ken Y. Yoneda, MD;
Division of Pulmonary and Critical Care Medicine. University of Califor-
nia, Davis, Sacramento, CA
INTRODUCTION: Trauma to the cricoarytenoid joint (CAJ) repre-
sents a potentially serious albeit rare complication of upper airway
instrumentation. Subluxation of the arytenoid cartilage has been reported
during difficult as well as uncomplicated endotracheal intubations and
extubations. To our knowledge, we report the first case of bilateral
cricoarytenoid joint subluxation following esophagogastroduodenoscopy.
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CASE PRESENTATION: A 70 year old woman was referred to us for
shortness of breath and wheezing. She had been well until five months
prior when she underwent an elective esophagogastroduodenoscopy
(EGD). For several days following the procedure the patient experienced
a hoarse voice. Subsequently she noticed an inspiratory wheeze. Mild
shortness of breath progressed to persistent dyspnea over the following
weeks and months. Trials of antibiotics and metered dose inhalers failed
to relieve her symptoms. She had a past medical history of diabetes
mellitus, hypertension, chronic renal insufficiency, and coronary artery
disease. She had never smoked. Physical exam was significant for a hoarse
voice and inspiratory stridor. Chest X-ray showed no lesions or infiltrates.
Pulmonary function testing revealed a fixed obstructive pattern with
flattening of the inspiratory and expiratory limbs of the flow volume loop.
Computerized tomography (CT) of the neck and thorax was normal except
for abnormal vocal cord position and atherosclerosis. A fiberoptic bron-
choscopy was performed which showed malpositioning of the arytenoid
cartilage bilaterally. The vocal cords appeared fixed and adducted with
minimal to no movement on vocalization. No endobronchial abnormalities
were encountered. She was referred to a laryngologist. Repeat laryngos-
copy confirmed bilateral adducted vocal cords fixed in paramedian
position. No abduction was observed with sniffing. A positive cough reflex
was present with arytenoid palpation. Attempts to endoscopically reduce
the CAJ subluxation with a spatula were unsuccessful. She ultimately
underwent laser arytenoidectomy with improvement in stridor and short-
ness of breath.
DISCUSSION: The incidence of a unilateral CAJ subluxation is
estimated to be less than 1 per 1,000 direct laryngoscopic endotracheal
intubations.
1
The incidence of bilateral CAJ subluxation is unknown.
Forces exerted via a laryngoscope blade, endotracheal tube or other
instrument have been debated to cause anterior and inferior displacement
of the arytenoid cartilage.
1,2
Systemic diseases such as rheumatoid
arthritis, sarcoidosis, Wegener’s granulomatosis and renal insufficiency
may predispose the joint to subluxation.
1,2
The diagnosis is often delayed. Bilateral arytenoid cartilage fixation
resembles vocal cord immobility due to recurrent laryngeal nerve paralysis
and thus injuries of the recurrent laryngeal nerve (RLN) are often held
responsible.
3
Both CAJ subluxation and RLN paralysis may result from
trauma to the larynx, and are difficult to differentiate clinically. The
diagnosis is established by the clinical course and laryngeal visualization.
CT, electromyography, and strobovideolaryngoscopy may help distinguish
arytenoid subluxation from RLN injury.
3
Reduction of CAJ subluxation
using a spatula under light pressure is often successful if performed in a
timely manner. However with unrecognized arytenoid dysfunction or
delayed treatment, the CAJ may undergo fibrosis and ankylosis. Patients
such as ours may ultimately require laser arytenoidectomy to relieve
stridor and respiratory distress.
CONCLUSION: Arytenoid subluxation is a rare complication of upper
airway instrumentation and should be suspected whenever persistent
hoarseness and stridor follow endolaryngeal manipulations. This is the
first report implicating EGD as a potential cause of arytenoid subluxation.
Early recognition is essential to avoid permanent vocal cord impairment.
REFERENCES:
1. Paulsen FP, Rudert HH, Tillmann BN. New Insights into the
Pathomechanism of Postintubation Arytenoid Subluxation. Anesthe-
siology. 1999; 91:659-66
2. Talmi YP, et al. Postintubation Arytenoid Subluxation. Ann Otol
Rhinol Laryngol 1996 May;105(5):384-90
3. Eckel HE, et al. Management of Bilateral Arytenoid Cartilage
Fixation Versus Recurrent Laryngeal Nerve Paralysis. Ann Otol
Rhinol Laryngol 2003 Feb;112(2):103-8
DISCLOSURE: S.J. Evans, None.
PRIMARY MALIGNANT MELANOMA OF THE TRACHEA CASE
REPORT
Cateline Sirois, MD*; Richard I. Inculet, MD; Keith Kwan, MD. London
Health Sciences Center, London, ON, Canada
INTRODUCTION: Primary malignant melanoma of the trachea is
uncommon. Only 6 cases have been reported worldwide. A case of a
patient who underwent a tracheal resection for this rare pathology is
presented.
CASE PRESENTATION: The patient is a 64 year old man who
presented with cough and hemoptysis. He did not have any symptoms of
airway obstruction. He had a 40 pack-year history of smoking.
Initial investigation was performed in a community hospital. A flexible
bronchoscopy demonstrated a lower obstructing tracheal tumor. Biopsies
showed malignant cells. A computed tomographic scan of the chest and
upper abdomen demonstrated the tracheal tumor (fig. 1). There were no
enlarged lymph nodes or evidence of metastases. A bone scan and an
abdominal ultrasound were normal. Pulmonary function test were consis-
tent with large-airway obstruction.
The patient was transfered to our institution and taken to the operating
room. Under general anesthesia the patient was intubated with a single
lumen tube and a flexible bronchoscopy was performed. The mass
occluded three quarters of the lumen of the distal trachea (fig. 2).
The tumor was polypoid and had a pedicle, which was attached about
three centimeters above the carina, on the right lateral wall. The surface
of the mass was smooth and tan in color. The tumor was removed to
provide relief of the airway obstruction. An endoscopic snare, with
electrocautery, was used to divide the pedicle. The mass was then snared
and removed together with the endotracheal tube. The patient was
discharge home the following day.
The pathology was reported as melanoma. It was a 1.9 cm polypoid
tumor. Within the subepithelial tissue were nests and sheets of epithelioid
cells. Mitotic figures were numerous. Granular brown pigment were
identified in occasional epithlioid cells and in adjacent histiocytes (fig. 3).
Immunohistochemestry showed strong positive staining of the epithelioid
cells for S 100, HMB45, and melan-A.
The patient was brought back electively to the operating room for a
tracheal resection to remove the residual tumor base. A flexible bronchos-
copy showed residual melanotic tumor at the site of the pedicle, and two
adjacent satellite nodules. A right thoracotomy was performed. Injection
of lymphazurin (isosulfan blue) into the tracheal wall was done to locate
a sentinel node. Frozen section of this node revealed no metastatic
melanoma. The tracheal resection was performed removing four tracheal
rings. The patient recovered uneventfully and was discharged home on
the seventh post-operative day.
The pathologic analysis of the resected trachea confirmed the presence of
residual melanoma. No benign junctional melanocytes were demonsrated.
DISCUSSIONS: Primary melanoma of the trachea is an extremely rare
condition. The pathological appearance may or may not suggest a primary
versus a metastatic tumor. For this reason it is mandatory to complete a
careful physical examination of all potential primary sites. In this patient,
we were satisfied no other primary site existed.
The tumor, at initial presentation, was almost completely obstructing
the distal trachea. Although it was biopsied elsewhere by flexible bron-
choscopy under mild sedation, this is not recommended because of the
potential for bleeding and ensuing complete airway obstruction. It is
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310S CHEST 2003—Case Reports

recommended that the patients with obstructing tracheal tumor be
investigated in the operating room, with rigid bronchoscope available
Sentinel node localization is the standard of practice for intermediate
risk cutaneous melanoma. This report describes the first attempt at
utilizing this for a tracheal neoplasm. It will require further study as to its
applicability.
CONCLUSION: Primary malignant melanoma of the trachea is very
rare. Tracheal resection is the treatment of choice for localized tumors.
REFERENCE:
Duarte IG, Gal AA, Mansour KA. Primary Malignant Melanoma of the
Trachea. Ann Thorac Surg 1998;65:559-60.
DISCLOSURE: C. Sirois, None.
Critical Care I
2:00 PM - 3:30 PM
VASOSPASM AND FATAL CEREBRAL INFARCTION ASSOCI-
ATED WITH SUMATRIPTAN USE IN MIGRAINE
Shoaib Alam, MD*; Yakub I. Khan, MD; Todd Gruber, MD; Shazia Riaz,
MBBS. Monmouth Medical Center, Long Branch, NJ
INTRODUCTION: Sumatriptan, a selective agonist of vascular sero-
tonin receptors(5-HT
1D
& 5-HT
1B
), is widely used in the treatment of
acute attack of migraine and cluster headaches. Current data suggests that
sumatriptan ameliorates migraine and cluster headaches through constric-
tion of certain large cranial blood vessels and/or inhibition of certain
neurochemical processes of central nervous system(CNS). Sumatriptan is
generally well tolerated and only 3.5 % patients have significant adverse
affects that warrant discontinuation of drug.
CASE PRESENTATION: A 43-year old Caucasian woman with strong
family history of migraine presented to emergency room with severe
throbbing occipital headache associated with photophobia and phonopho-
bia. Headache was partially relieved with Acetaminophen which she had
been taking at home. She had no significant past medical history except
migraine. She had no known drug allergies. Acetaminophen was her only
medication. She was a smoker with no history of alcohol or illicit drug use.
She was working as security guard at a local amusement park. Her mother
and sister had history of migraine. Physical examination was normal. Initial
laboratory data was also non-revealing, including spinal fluid analysis and
non-contrast-CT-scan of the head. She was discharged on Sumatriptan
after neurology consultation. She had never used Sumatriptan in the past.
Two weeks later, she presented with recurrent severe headache with
complete blindness for two hours. She had two prior episodes of
blindness, each lasting 20-30 minutes, within past 48 hours. She had
been using Sumatriptan for the past two weeks. A repeat CT-scan and
laboratory work including a toxicology screen was negative. She was
admitted and was started on dexamethasone, nimodipine and ketoro-
lac. Over the next twelve hours her condition worsened with develop-
ment of right-hemiparesis and later, bilateral spastic paralysis. An
emergency angiogram showed evidence of severe vasospasm of poste-
rior cerebral circulation as well as some involvement of anterior and
middle cerebral arteries. Intravenous acyclovir and cyclophosphamide
were also added for the possibility of viral encephalitis or vasculitis,
respectively. Within next 24-48 hours patient started developing signs
of raised intracranial pressure. Despite aggressive attempts, patient
could not be resuscitated. Her serum tests came back negative for
HSV, dengue fever and West Nile virus serologies. Her ESR at
presentation was 17. Blood and CSF cultures for bacteria, fungi and
AFB were also negative. Autopsy revealed acute anoxic injury in
cerebrum and marked vascular congestion of cerebrum, thalamus,
substantia nigra and basal ganglia. Pontine hemorrhage of Duret type
was also present. Post mortem cultures were also negative.
DISCUSSION: Most of the serious adverse events, such as acute
coronary syndrome, serious cardiac arrhythmias, cerebral hemorrhage,
subarachnoid hemorrhage, ischemic stroke, ischemic colitis and pe-
ripheral vascular ischemia, are considered to be related to vasocon-
strictive effects of sumatriptan. We are aware of two previous partic-
ularly relevant case reports. One is finding of segmental narrowing in
multiple cerebral vessels on digital subtraction angiography in a
43-year old male, after taking 23 tablets of sumatriptan succinate 25mg
with midrin, with no other possible explanation. Angiographic findings
were resolved upon discontinuation of the drug and administration of
nicardapine. Other is a report of multiple intracranial hemorrhages and
severe vasospasm of both anterior cerebral arteries on angiography, in
a patient taking several anti-migraine drugs with no other explanation
on extensive work-up.
CONCLUSION: The finding of any new focal neurological sign in
patients with migraine on sumatriptan should raise the possibility of
sumatriptan induced vasospasm.
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REFERENCE:
1. Meschia JF, Malkoff MD, Biller J. cerebral artery vasospasm with
excessive use of sumatriptan and midrin. Arch Neurol 1998 May;
55(5): 712-4.
2. Nighoghossian N, Derex L, Trouillas P. intracerebral hemorrhage
and vasospasm after use of antimigranous drugs. Headache 1998Jun;
38(6): 478-80
DISCLOSURE: S. Alam, None.
LEGIONELLA MICDADEI PRESENTING AS SEVERE SECRE-
TORY DIARRHEA AND SOLITARY PULMONARY MASS
Boris I. Medarov, MD*; Anita Siddiqui, MD; Tahir Mughal, MD;
Leonard J. Rossoff, MD; Mark Moshiyakhov, MD. Long Island Jewish
Medical Center, Bayside, NY
INTRODUCTION: 60% of non-pneumophila Legionella infections
are caused by L. micdadei.
CASE PRESENTATIONS: A 45-year-old obese male presented with
cough, dyspnea and high fever. Examination showed bilateral rhonchi.
Arterial blood gas revealed hypoxemia and respiratory acidosis requiring
mechanical ventilation. Presumptive diagnoses of bronchitis and obesity-
hypoventilation syndrome were made. Azithromycin and ceftriaxone were
started. He developed profound, watery, nonbloody diarrhea of up to 8
liters/day. Metronidazole was added to his regimen. Blood, sputum, urine
and stool cultures were unrevealing. Clostridium difficile A toxin assay was
repeatedly negative. A CT abdomen was unremarkable. A CT chest
demonstrated a 3.2 cm mass in the left lower lobe, hilar lymphadenopathy
and pleural effusions (Fig.1).
Sputum direct fluorescent antibody assay was positive for Legionella
micdadei. Rifampin was added and subsequently multiple sputum cul-
tures grew L. micdadei.. No stool leukocytes, ova or parasites were
identified and the osmotic gap showed secretory diarrhea. Colonoscopy
was unremarkable. The patient became afebrile after several days and his
diarrhea stopped. Follow-up CT four weeks later showed resolution of the
lung mass and lymphadenopathy (Fig.2).
DISCUSSIONS: Expanding, occasionally cavitating, pulmonary nod-
ules have been a dramatic finding in some patients infected by L.
micdadei
1
. Our patient’s pulmonary mass resolved completely after
appropriate treatment for L. micdadei.
While diarrhea is common in Legionella infections, profuse diarrhea
seems to be extremely rare. We could only find one documented case of
L. micdadei pneumonia causing massive diarrhea
2
. The etiology appears
to be secretory.
CONCLUSIONS: Legionellosis may manifest in many ways present-
ing a diagnostic challenge. Timely diagnosis and treatment require a high
degree of clinical suspicion. The recognition of uncommon presentations
with profound diarrhea and/or new onset solitary pulmonary nodule may
aid in an earlier suspicion and diagnosis of Legionella infection.
REFERENCES:
1. Ellis AR, Mayers DL et al. Rapid expanding pulmonary nodule
caused by Pittsburgh pneumonia agent. JAMA. 1981;245:1558-1559
2. Foltzer MA, Reese RE. Massive diarrhea in Legionella micdadei
pneumonitis. J Clin Gastroenterol. 1985 Dec; 7(6):525-7
DISCLOSURE: B.I. Medarov, None.
THE EFFECT OF ABDOMINAL BINDING ON WEANING A
PATIENT WITH A LARGE VENTRAL ABDOMINAL WALL HER-
NIA
C Gerard Petersen, MD*. Temple University School of Medicine,
Philadelphia, PA
INTRODUCTION: Abdominal muscle tone provides a fulcrum for
the diaphragm to contract to displace the lower thorax and decrease
intrathoracic pressure (1,2,3,4,5). Inability to generate intrabdominal
pressure during diaphragm contraction is observed in patients with
abdominal hernias, prune belly, and quadriplegia (6,7,8,9,10).
CASE PRESENTATION: A 66-year-old man was admitted after a fall.
MRI revealed cervical stenosis and edema from C
3-6
. Decompression was
performed. Postoperative course was complicated by intractable respira-
tory failure. Two years prior, the patient sustained injuries that required
an exploratory laparotomy and multiple reexplorations resulting in a large
ventral hernia. On physical exam the contour of the small intestines could
be appreciated. Ventral displacement of abdominal contents occurred
during inspiration. Manual reduction improved chest wall expansion.
We applied an elastic abdominal binder to improve respiratory mechan-
ics, but cephalad migration limited chest wall expansion. We constructed
a plaster abdominal cast kept in place by Velcro straps.
Esophageal (Pes), gastric (Pga), and transdiaphragmatic pressures (Pdi),
respiratory rate, flow, tidal volume, minute ventilation, oxygen consump-
tion (VO
2
), carbon dioxide production (VCO
2
), and arterial blood gas were
measured during tidal breathing for 5 minutes with and without the cast.
RESULTS: Measured variables are displayed in Table 1. Cast applica-
tion resulted in a 54.5% increase in end-inspiratory Pga, and a 50.8%
increase in end-inspiratory Pdi. Analysis of esophageal and gastric pres-
sure swings during spontaneous tidal breathing revealed dysynchrony
between end-inspiratory Pes (Pes
I
) and Pga (Pga
I
). Application of the cast
resulted in improved synchrony.
Pes
I
, Pga
I
, and end-expiratory Pes (Pes
E
) and Pga (Pga
E
) are plotted in
Figure 1$$. Pressure plots with the cast off demonstrate a negative
Pes:Pga slope as gastric pressure decreases during inspiration. With the
cast, the plots demonstrate a clockwise rotation with a more positive
Pes:Pga slope as gastric pressure increases during inspiration.
Prior to cast application, the patient weaned 3.9 ⫾ 3.2 hours per day for
13 days. After cast application, the patient weaned for 12.3 ⫾ 5.8 hours
per day for 3 days and was ventilator-independent on day 4.
DISCUSSION: During inspiration the patient’s abdominal contents
protruded into the hernia due to lack of an effective abdominal wall. Force
that should have contributed to Pdi generation and elevation of the lower
rib cage was spent displacing abdominal contents. The cast recreated an
effective abdominal wall preventing the displacement of abdominal
content as demonstrated a 54.5% increase in Pga.
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312S CHEST 2003—Case Reports

Improvements in Pdi were also due to more synchronous thoracic and
abdominal movements. Without cast the most negative intrapleural
pressures during inspiration (Pes
I
) were not synchronous with the most
positive gastric pressures, as should occur in normals.
Figure 1 compares the Pes:Pga slopes which demonstrate a negative
slope while the cast is off. A negative slope is associated with decreased
exercise endurance (13) and suggestive of dysynchronous contraction of
the diaphragm and rib cage muscles. The cast made the slope positive
indicating more effective contraction of the diaphragm and decreased
accessory muscle use.
An increased VCO
2
with cast application was not accompanied by a
decrease in PaCO
2
. We believe the increase in elimination of carbon
dioxide in conjunction with a decrease in PaCO
2
is reflective of increased
alveolar ventilation. The increase in CO
2
production was not reflective of
increased metabolic demands (O
2
consumption was unchanged), suggest-
ing a more efficient ventilation.
CONCLUSION: Application of an abdominal cast in a patient with an
ineffective abdominal wall contributed to increased abdominal pressure, a
more favorable fulcrum for diaphragmatic contraction, improved syn-
chrony of intrapleural and gastric pressure swings, increased Pdi genera-
tion, and increased alveolar ventilation without an increased metabolic
cost. These improvements translated to improved periods of spontaneous
breathing and weaning from prolonged mechanical ventilation.
Table 1: Cast Off versus On
Parameters Cast Off (Mean⫾SD) Cast On (Mean⫾ SD)
Pes, cm H2O -14.3 ⫾ 1.1 -15.6 ⫾ 1.0
Pga, cm H20 13.9 ⫾ 0.9 21.4 ⫾ 0.9
Pdi, cm H2O 23.8 ⫾ 1.5 35.9 ⫾ 1.0
Respiratory Rate, bpm 34.3 ⫾ 3.7 34.6 ⫾ 5.2
Tidal Volume, ml 382.6 ⫾ 65.5 404.8 ⫾ 70.6
PaCO2, mmHg 46 41
VO2, L/min 0.239 ⫾ .02 .240 ⫾ .01
VCO2, L/min 0.178 ⫾ .01 0.194 ⫾ .01
DISCLOSURE: C. Petersen, None.
HYPONATREMIC ENCEPHALOPATHY IN A MARATHON RUN-
NER
Julie-Anne Thompson, MD; Armand J. Wolff, MD*. Georgetown Uni-
versity, Washington, DC
INTRODUCTION: With increasing participation in marathons and
triathalon events, medical complications of endurance athletic events are
being increasingly reported. Hyponatremia is not uncommon in this
setting. We report a case of hyponatremia in a marathon runner that led
to encephalopathy and death.
CASE PRESENTATION: A 35 year-old 1
st
time marathoner collapsed
at mile 21 of the 2002 Marine Corps Marathon in Washington, DC. She
had complained to her family of headache and blurry vision around mile
19. She was treated in the field with 0.9% saline for presumed dehydra-
tion, but there was a 2-hour delay in transporting her. Within 30 minutes
after arrival in the ER, her mental status deteriorated quickly. She also
developed cardiac arrhythmias and her oxygenation worsened, requiring
intubation and mechanical ventilation. Her initial CXR demonstrated
pulmonary edema. By 2.5 hours after collapse, her right pupil was dilated
and fixed. She was immediately sent for computed tomography (CT) of
her brain; this demonstrated diffuse cerbral edema with mid-line shift,
effacement of the gyri/sulci, and compression of the 4
th
ventricle. On
transfer back from CT, her left pupil became dilated and fixed. She had
no gag or corneal reflexes by this point. A ventriculostomy was performed,
returning clear CSF with an elevated pressure. Her initial labs came back,
demonstrating a serum sodium of 123 meq/L. She was begun on 3% saline
for symptomatic hyponatremia, but the rate was decreased when her
repeat labs demonstrated a serum sodium of 128 meq/L. Her urine output
increased markedly, and her urine osmolarity was low. Within an hour,
repeat labs demonstrated a serum sodium of 131 meq/L and the 3% saline
was discontinued. She never regained any brain stem function, brain CT
scans showed worsening edema and shift with impending herniation, and
she failed an apnea test. She was declared brain dead within 24 hours of
admission.
DISCUSSION: Hyponatremia following endurance events is not
uncommon. 9% of participants in the 1996 New Zealand Ironman sought
medical care, as did 29% in the Hawaiian Ironman. In 1998, 605 athletes
in the New Zealand Ironman were studied. 330 finished the race and 58
had a serum sodium level ⬍ 135 meq/L, but only 18 sought medical
attention. Premenopausal women appear to be more commonly affected
perhaps because estrogen influences osmoregulation via oxytocin, aug-
menting anti-diuretic hormone (ADH) secretion. Excessive fluid intake
may contribute, but hyponatremia has resulted despite only modest fluid
intake. Exercise leads to loss of water and salt via sweat, resulting in
decreased plasma volume. Baroreceptor activity due to decreased plasma
volume results in ADH release, and effects plasma volume via thirst and
other mechanisms. Volume contraction stimulates the release of atrial
naturietic factor (ANF); causing loss of free water and sodium in the urine.
Intake of water or another hypotonic rehydration solution exacerbates the
hyponatremia. Intestinal fluid absorption increases on cessation of activity,
with redistribution of blood flow to the splanchnic circulation. Further-
more, free water clearance is limited by the maximum glomerular
filtration rate. Carbohydrate loading, common before endurance events,
may worsen the problem via increased free water production from
glycogenolysis. The quick increase in serum sodium in our patient likely
reflects diabetes insipidus following brain death.
CONCLUSIONS: Exercise induced hyponatremia is more common
than generally realized. Medical personnel staffing such events should be
aware of this potentially fatal complication of endurance exercise and
should be trained in its recognition and management. This may include
weighing participants before the event, proper equipment for detecting
hyponatremia, and appropriate fluid administration. Athletes should be
educated about this entity and be made aware of the risks of overhydration
and carbohydrate loading.
DISCLOSURE: A.J. Wolff, None.
MALIGNANT HYPERTENSION ASSOCIATED WITH PARAGAN-
GLIOMA OF THE TONSIL
Shahid Yakoob, MBBS*; Khalid Malik, MD, FCCP; Johnathan D.
Mcginn, MD; Yulin Liu, MD, PhD; Brian Carlin, MD, FCCP. Allegheny
General Hospital, Pittsburgh, PA
INTRODUCTION: Malignant hypertension (MH) is a medical emer-
gency rarely associated with paraganglioma. We present a unique case of
a MH associated with a paraganglioma of the tonsil.
CASE PRESENTATION: A 29 year-old man presented with mental
status and visual changes, hypertension (BP 220/120 torr), and respiratory
failure. During intubation for respiratory failure, a mass in the right
tonsillar region was found. The MH required intravenous labetalol and
phenoxybenzamine. A neck MRI showeda2x2.5x4cmright tonsillar
mass sparing the parapharyngeal fat. CT scans of the chest, abdomen, and
pelvis were normal. A biopsy of the mass showed prominent fibrovascular
stroma (classic “zellballen” pattern) consistent with a paraganglioma.
Plasma catecholamines were elevated and remained high after clonidine
administration. Surgical resection via transoral route with vascular control
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within the neck was performed following embolization of the tumor. The
pathology confirmed paraganglioma. His hypertension was more easily
managed following its resection. He was extubated three days after
surgery and subsequently transferred to a rehabilitation facility ten days
later. His blood pressure was 160/90 torr.
DISCUSSION: This case of malignant hypertension is unique in its
relationship to a catecholamine secreting paraganglioma. The paragangli-
oma location (tonsil) has not previously been reported in the literature.
Malignant hypertension is a medical emergency usually occurring second-
ary to uncontrolled hypertension. Rare causes of MH include pheochro-
mocytoma and paraganglioma. Paragangliomas often present as painless
upper neck masses. Hypertension, headache, palpitations and sweating
may occur; however, functional hormone secretion is uncommon when
the tumor arises in the head and neck region (2 percent). This tumor
secreted catecholamines producing MH manifested as CNS and respira-
tory dysfunction. No laboratory evidence of pheochromocytoma was
found. Resection of the paraganglioma resulted in a significant improve-
ment in blood pressure control.
CONCLUSION: This unique case shows the association of MH with a
catecholamine secreting paraganlioma of the tonsillar bed. Recognition of
this entity as a potential cause for MH is important so that appropriate
management is performed.
REFERENCES:
Jackson CG; Harris PF. Diagnosis and management of
paragangliomas of the skull base. American Journal of Surgery 1990
159(4): 389-393
Glifford, RW Jr. Management of hypertensive crises. Journal of
American Medical Association 1991; 266: 829
DISCLOSURE: S. Yakoob, None.
MEDIASTINAL HEMATOMA WITH SVC SYNDROME FROM A
CENTRAL VENOUS CATHETER INSERTION: A CASE REPORT
Hicham K. Krayem, MD*; Alvin Bowles, MD; Amr Hanbali, MD. Henry
Ford Hospital, Detroit, MI
INTRODUCTION: Superior vena cava syndrome (SVCS), mostly
secondary to malignant disease, is a rare complication of intraluminal
catheters, caused by thrombosis and obstruction to flow in the SVC. We
describe what may be the first reported case of SVC syndrome caused by
a compressing mediastinal hematoma from an attempted central line
insertion.
CASE PRESENTATION: 49-year-old male presented to the ED with
progressive dyspnea and syncope during hemodialysis. Pertinent physical
exam findings included tachypnea, tachycardia, facial and upper extrem-
ities edema. CXR revealed right mediastinal opacity. CT thorax showed a
homogeneous mass extending from apex to subcarinal level occluding the
RUL bronchus, right brachiocephalic and jugular veins, and compressing
the distal trachea and SVC.
12 days earlier, he was admitted to another hospital with anasarca, and
started on hemodialysis for ESRD. Records from that hospitalization
indicate multiple unsuccessful attempts at right subclavian and internal
jugular line placement and a negative post-procedure CXR. We diagnosed
him with a compressing mediastinal hematoma. With his symptoms
improving, he required conservative management with outpatient follow-
up. CT thorax 2 months later revealed slow resolution of the hematoma.
DISCUSSION: Subclavian line insertion carries more risk of hemo-
thorax and pneumothorax than internal jugular insertion. This latter has
higher risk of arterial puncture. The overall risk for mechanical compli-
cations for both insertion sites is similar. This risk increases significantly in
less experienced hands and with repeated attempts. Our patient’s sizable
hematoma probably resulted from an inadvertent arterial puncture/injury
or venous laceration, and aggravated by the bleeding diathesis from
uremia. This compressing hematoma led to the SVCS in our case, thus
distinguishing it from previous case reports of vascular access-induced
SVCS where the cause is intraluminal thrombosis.
CONCLUSION: To the best of our knowledge, there has not been a
reported case of hemomediastinum with SVCS from a central venous
catheter insertion. We report this case aiming to draw the attention to this
rare occurrence.
REFERENCES:
Mcgee. NEJM 2003;348:1123-1133
Madan. Am Surg 2002;68(10):904-906
Woodyard. Cancer 1993;71(8):2621-2623
DISCLOSURE: H.K. Krayem, None.
Infectious Diseases II
2:00 PM - 3:30 PM
HIGH DOSE INTRAVENOUS METHYLPREDNISOLONE IN
THE TREATMENT OF SEVERE ACUTE RESPIRATORY SYN-
DROME: A CASE REPORT
Sharmistha Mishra, MD*; Winnie M. Lee, MD, MSc; Brian Bressler,
MD; Stephen C. Juvet, MD; Denis Hadjiliadis, MD, MHS. University of
Toronto, Toronto, ON, Canada
INTRODUCTION: This is a report of a case of probable severe acute
respiratory syndrome (SARS) with use of pulse methylprednisolone as
treatment.
CASE PRESENTATION: A 72 year-old female was assessed for fever.
Her past medical history included a remote 30-pack year history of
smoking. Three weeks prior to her admission, her husband was treated in
the emergency department for atrial fibrillation. He returned nine days
later with a febrile illness and respiratory symptoms consistent with SARS.
Subsequently, it was determined that the person adjacent to his gurney
during the initial visit was a member of the SARS index family in Toronto
1
.
Our patient presented with a two week history of fever and significant
malaise.On admission, her respiratory rate was 16 breaths per minute,
oxygen saturation 91% on room air, and her temperature was 38.9°C.
Respiratory exam revealed bibasilar crackles.
Laboratory studies revealed: normal white blood cell count and differ-
ential, elevated creatine kinase (429U/L), and elevated lactate dehydro-
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Critical Care I, continued
314S CHEST 2003—Case Reports

genase (490U/L). The rest of her laboratory studies were unremarkable.
Her arterial blood gas revealed: pH 7.55, PaO
2
66mmHg, PaCO2
28mmHg. Her chest radiograph (Figure 1) was normal.
Initial therapy consisted of intravenous (IV) ribavirin and gatifloxacin.
She became afebrile on day three. However, a right mid-upper lung
infiltrate occurred on day two of admission (Figure 2).
On day six, she developed a dry cough and dyspnea. By day ten she
required 100% oxygen by face mask and her chest radiograph revealed
extension of disease to her left lung (Figure 3). Therapy with IV
methylprednisolone (60mg daily) and meropenem was initiated.
There was no improvement clinically or radiographically by day four-
teen (Figure 4). Pulse IV methylprednisolone 250mg daily for three days,
followed by 60mg IV methylprednisolone daily was given. Ribavirin was
re-initiated. Her clinical course stabilized until day 19, but she remained
on 100% FiO2.
DISCUSSION: SARS has become a global health issue. To date, there
is no known effective treatment. IV ribavirin, corticosteroids, and broad-
spectrum antibiotics have been tried
1,2
. To our knowledge, this is the first
report of high dose IV corticosteroids in the treatment of SARS outside of
Hong Kong.
Corticosteroids decrease inflammation and have been used in the
treatment of other acute severe diffuse respiratory illnesses such as acute
respiratory distress syndrome (ARDS)
3
, and bronchiolitis obliterans orga-
nizing pneumonia (BOOP)
4
with acceptable results. Some patients with
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SARS develop diffuse lung infiltrates late in their course, which may be
consistent with an inflammatory response to the original infection.
Our patient developed diffuse lung infiltrates and hypoxemia late after
admission, while remaining afebrile. Pulse IV corticosteroids were used in
an attempt to reverse the inflammatory process. This approach has been
used successfully in treating some SARS patients in Hong Kong
2
.
CONCLUSION: On day 21, our patient died from a respiratory arrest.
The autopsy findings were consistent with acute respiratory distress
syndrome (ARDS). Further investigations are needed to determine the
role of pulse dose corticosteroids in patients with SARS.
REFERENCES
1. Poutanen SM, Low DE, Henry B, et al. Identification of severe
acute respiratory syndrome in Canada. N Eng J Med 2003. Available
at http://content.nejm.org
2. Lee N, Hui D, Wu A, et al. A major outbreak of severe acute
respiratory syndrome in Hong Kong. N Eng J Med. Available at
http://content.nejm.org
3. Meduri GU, Headley AS, Golden E, et al. Effect of prolonged
methylprednisolone therapy in unresolving acute respiratory distress
syndrome: a randomized controlled trial. JAMA 1998; 280(2):159-65
4. Epler GR. Bronchiolitis obliterans organizing pneumonia. Arch
Intern Med 2001; 161:158-64
DISCLOSURE: S. Mishra, None.
PRIMARY ENDOBRONCHIAL TUBERCULOSIS AS A CAUSE
OF WHEEZING IN 16 MONTHS OLD CHILD
Rasik V. Shah, MD.*; Haesoon Lee, MD; Mary Cateletto, MD. Depart-
ment of Pediatrics, Winthrop University Hospital, SUNY Stony Brook
School of Medicine, Mineola, NY
INTRODUCTION: Primary endobronchial tuberculosis (EBTb) is a
rare Mycobacterium tuberculosis (M Tb) infection involving the bronchial
tree without any radiographic lesions in the lung parenchyme. Early
diagnosis and treatment are essential to prevent bronchial stenosis or
bronchiectasis.
CASE PRESENTATION: A 16-month-old boy with a 6-month history
of cough and wheezing not responding to albuterol and inhaled cortico-
steroids (CS) presented with a chest radiograph (CXR) demonstrating
hyperaeration of the left lung (Figure 1). Bronchoscopy revealed a
polypoid mass obstructing the left main bronchus. He had no known
exposure to M Tb and his parents were PPD negative. He had frequent
visits to Costa Rica and his PPD recently became positive. He underwent
bronchoalveolar lavage (BAL) and gastric aspirates, all negative on smear,
culture and PCR for M Tb. Biopsy of the mass on two occasions did not
show an acid-fast bacillus (AFB) but showed granulomas without necrosis
(Figure 2). CT scan of the chest after resection of the mass showed
narrowing of the entire left main stem bronchus with submucosal edema
and subcarinal lymphadenopathy without compression of bronchus (Fig-
ure 3). He was treated with 4 drugs anti-Tb therapy for 6 months, with
oral CS for the first three months under the DOT (directly observed
therapy) with an excellent outcome (Figure 4).
DISCUSSION: EBTb can cause difficult diagnostic and therapeutic
problems. The diagnosis is often missed or delayed. Late diagnosis may
contribute to a cicatricial bronchostenosis and bronchiectasis despite an
efficacious anti-Tb therapy. EBTb is usually suspected by clinical findings
and CXR evidence of bronchial obstruction. Clinical presentation of
EBTb in children is often nonspecific. Rare cases may present with
stridor, respiratory distress, asthma, or suspicion of foreign body aspira-
tion. The physical findings are related to the associated conditions such as
atelectasis of the involved lobe or airway obstruction.
Finding the cause of wheezing in an infant can be a diagnostic
challenge. It is very common but has diverse etiologies. Poor response to
the bronchodilator suggests a fixed lesion. A 2-views CXR is the first step.
Unilateral hyperinflation may be secondary to lesions causing either
intrinsic or extrinsic airway obstruction. Unilateral hyperaeration is un-
usual in EBTb, noted only in 1.5% of the cases. Direct examination with
a rigid bronchoscope is the diagnostic method of choice, when biopsies
taken and the specimen sent for pathology, cytology, and cultures for Tb.
Tuberculous granulomas may be devoid of necrosis in the early stages.
The pathogenesis of EBTb is not fully understood. Potential mecha-
nisms suggested are a direct extension from the adjacent parenchymal
infection (i.e. cavity), implantation of organisms from the infected sputum,
a hematogenous dissemination, erosion of a lymph node into the bron-
chus, and lymphatic drainage from the parenchyma to the bronchus. Our
patient probably had Tb infection from an unidentified source. It is not
clear why the inhaled AFB was able to establish in bronchus.
AFB smear and culture are positive only in 30-40% of children and 70%
in infants with pulmonary Tb (gastric washings 47%, BAL 11%, and
bronchial biopsy 84%). Recent conversion of PPD was very helpful in
establishing diagnosis of M Tb in children. Our patient met the criteria of
CDC for culture negative pulmonary Tb.
Treatment with 4 drugs regimen (isoniazid, rifampin, pyrazinamide,
and ethambutol) is indicated when the source of infection is unknown.
Addition of CS has been found to be useful only when used early in the
course of the disease.
CONCLUSION: This case highlights several clinical lessons: 1. all that
wheeze are not asthma. 2. the importance of CXR for evaluation of
persistent wheezing, 3. consideration of EBTb in differential diagnosis of
unilateral hyperaeration.
REFERENCE:
CDC Criteria for Culture Negative Pulmonary Tuberculosis
1. Positive PPD with 5 TU (5 mm or greater induration
2. Intitial AFB smears and cultures are negative
3. No other diagnosis has been established after appropriate evaluation
4. Clinical or radiological response within 2 months of intiation of
chemotherapy
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316S CHEST 2003—Case Reports

DISCLOSURE: R.V. Shah, None.
PANCOAST’S SYNDROME DUE TO CHRONIC NECROTIZING
PULMONARY ASPERGILLOSIS
Thun-How Ong, MBBS, MRCP*; Philip Eng, FCCP. Singapore General
Hospital, Singapore, Singapore
INTRODUCTION: Aspergillus can cause a wide spectrum of disease,
from an indolent aspergilloma to fulminant invasive aspergillosis. We
report here a rare case of Pancoast’s syndrome caused by chronic
necrotizing aspergillosis (CNA).
CASE PRESENTATION: A 57- year old lady presented with right
upper chest wall pain and intermittent blood-streaked sputum. Her past
history was remarkable for right upper lobe bronchiectasis, a right
mastectomy for breast cancer five years previously (with no known
recurrence of disease), as well as a right neck of femur fracture that was
complicated by prothesis infection and required several revisions.
Chest radiographs (CXR) initially showed chronic collapse/ consol-
idation of the right upper lobe. Bronchoscopy showed no endobron-
chial lesions and mycobacterial cultures were negative. Over the next
six months, the patient developed a right ptosis with weakness and
wasting of the T1 distribution of muscles. Serial CXRs and Comput-
erised Tomography (CT) of the thorax showed replacement of the right
upper lobe by thickened pleura and necrotic debris with a progres-
sively enlarging mycetoma within. Transthoracic needle aspiration of
the pleura and of the soft tissue mass yielded Aspergillus fumigatus
from both histology and cultures. She was started on oral itraconazole,
but developed severe nausea and diarrhea. Two weeks after starting
therapy, she developed signs of superior venal caval obstruction
(SVCO) as well. CT thorax confirmed this and also showed invasion of
the right internal jugular vein. She was admitted for a course of
intravenous amphotericin. However, 13 days after starting treatment,
she deteriorated suddenly with clinical evidence of pulmonary embo-
lism and succumbed.
DISCUSSION: CNA is characterised by an indolent, progressive local
invasion of lung tissue and usually occurs in patients such as ours, who
have evidence of pre-existing lung disease and are marginally immuno-
compromised eg by poor nutrition or chronic illness.
1
CNA is traditionally
differentiated from invasive aspergillosis by a more indolent course and by
lack of vascular invasion. The clinical course of this patient falls somewhat
in between - she had a rather indolent course of progression, but with
macrovascular invasion and progressive parenchymal destruction. As-
pergillus infection should perhaps be considered more of a spectrum,
from aspergilloma to CNA to invasive aspergillosis, rather than as separate
clinical entities.
Treatment with intravenous amphotericin or itraconazole syrup has
been reported to be useful.
1,2
Reported morality rates for CNA vary from
25 – 60% and may be related to the time from presentation to diagnosis;
a high index of suspicion and early treatment may improve outcome.
1
Pancoast’s syndrome is caused by involvement of the eighth cervical and
first two thoracic nerve roots by compression or invasion of a lesion at the
superior thoracic inlet. It is most commonly caused by malignancy.
Pancoast’s syndrome caused by aspergillus is rare. There are at least two
previous reports in the literature,
3,4
but both of these patients were frankly
immunocompromised and had invasive aspergillosis. To our knowledge,
this is the first report of Pancoast’s Syndrome caused by chronic necro-
tizing pulmonary aspergillosis.
CONCLUSION: In a marginally immunocompromised host, aspergil-
lus infection can be slowly yet relentlessly invasive and should be treated
with respect. Vascular invasion and invasion of the superior sulcus can
occur in chronic necrotising aspergillosis, resulting clinically in Pancoast’s
syndrome.
REFERENCES:
1. Saraceno JL, Phelp DT et al. Chronic necrotizing pulmonary
aspergillosis: approach to management. Chest 1997 112 (2) : 541-8.
2. Soubani AO, Chandrasekar PH. The clinical spectrum of
pulmonary aspergillosis. Chest 2002 121 (6) : 1988-99.
3. Collins PW, de Lord C, Newland AC. Pancoast’s tumour due to
aspergilloma Lancet. 1990 336 (8730): 1595.
4. Simpson FG, Morgan M, Cooke NJ. Pancoast’s syndrome
associated with invasive aspergillosis. Thorax 1986 41(2): 156 – 7.
DISCLOSURE: T. Ong, None.
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NOT YOUR TYPICAL STRONGYLOIDES INFECTION
P. James Abraham, MD*; Jeffery L. Garland, MD. Mayo Clinic Jackson-
ville, Jacksonville, FL
INTRODUCTION: Strongyloides hyperinfection syndrome (SHS) is
one of several clinical manifestations of Strongyloidiasis and has a
mortality rate exceeding 80%.
Herein, we describe a case of SHS with a novel feature.
CASE DESCRIPTION: A 69 year-old male from Venezuela with a
history of COPD presented to our institution for an evaluation of
progressive dyspnea not explained by his underlying lung disease. Three
weeks prior to admission, he developed episodic fevers, rigors, loose
stools, and progressive hypoxemia with worsening pulmonary infiltrates
refractory to treatment with steroids and antibiotics. Within twelve hours
of arrival at our institution, the patient’s condition acutely deteriorated
requiring transfer to the ICU. He underwent bronchoscopy with bron-
choalveolar lavage (BAL), and was empirically treated for suspected
Pneumocystis Carnii Pneumonia (PCP) with intravenous trimethoprim/
sulfamethoxazole, and for a presumed superimposed nosocomial pneu-
monia with ciprofloxacin and ticarcillin-clavulanate. The BAL specimens
subsequently revealed the presence of multiple pathogens including
Strongyloides stercoralis, Pneumocystis carnii, Pseudomonas aeruginosa,
Cytomegalovirus, herpes simplex virus, and Aspergillus species. Blood
cultures revealed Enterococcus sp., Salmonella sp., and the serum was
positive for the CMV pp65 antigen. Ivermectin, voriconazole, and gancy-
clovir were then added to his medical regimen, and a steroid taper was
instituted.
DISCUSSION: SHS is a syndrome characterized by high organism
burden due to autoinfection that most commonly occurs in immunocom-
prised hosts. The recovery of multiple pathogens is due to a “piggy back”
phenomenon that is believed to occur when enteric pathogens are
transferred to the bloodstream attached to Stongyloides larva. To our
knowledge, this is the first reported case of SHS and PCP occurring
simultaneously.
CONCLUSION: Due to its extremely high mortality rate, Strongy-
loides hyperinfection syndrome must be considered early in the differen-
tial diagnosis of respiratory failure when multiple pathogens are recovered
in a patient from an endemic area.
DISCLOSURE: P. Abraham, None.
PULMONARY ARTERY ANEURYSM CAUSED BY COCCIDIOID-
OMYCOSIS
Rade Tomic, MD*; Andres Pelaez, MD; Carmen Klass, MD; Howard
Silverboard, MD. Emory University Hospital, Atlanta, GA
INTRODUCTION: Pulmonary artery aneurysms are a very rare
vascular anomaly. Here we report a case in which cavitary lung disease
due to coccidioidomycosis led to a pulmonary arterial aneurysm.
CASE PRESENATION: A 52-year-old man with a past medical
history of coronary artery disease, hyperlipidemia was admitted in hospital
for massive hemoptysis. He reported one similar episode of hemoptysis in
1990. At that time, he was found to have a left upper lobe cavity and
mediastinal lymphadenopathy.In general, the patient appeared athletic
and well nourished. Auscultatory exam revealed crackles in the left base,
and diminished breath sounds posteriorly over the left upper lobe. The
physical examination was otherwise unremarkable. Initial laboratory tests
revealed a white blood count of 10,500 /uL with 82% neutrophils, 12%
lymphocytes, and 5% lymphocytes. The hemoglobin was 12 g/Dl, and the
platelet count was 274, 000 per mm3. Electrolytes and creatinine levels
were normal. Blood cultures were negative. Three consecutive sputum
smears were negative for AFB. Chest radiography demonstrated a thin
walled cavity in the left upper lobe. Computed tomography (CT) of the
chest with contrast revealed a pseudo aneurysm within the left upper lobe
cavity.Fiberoptic bronchoscopy found scanty blood without a source of
active bleeding. Blood was noted prominently in the posterior segment of
the left upper lobe. Initially all culture were unrevealing. The patient
continued to bleed, and therefore a repeat CT-scan of the chest was
performed. This time 3-dimensional chest pulmonary artery angiography
was utilized, revealing a (4-5 mm) pulmonary artery aneurysm within the
cavity. Thoracic surgery was consulted given the possibility of exsangui-
nation. Subsequently, an immediate left upper lobe lobectomy was
performed, and the procedure was tolerated well. Pathologic examination
revealed multiple necrotizing granulomas identified within the lung
parenchyma. In addition, a medium sized artery exhibited necrotizing
granulomatous inflammation, disrupting the wall of the vessel. After
approximately 4 weeks, Bronchoalveolar lavage (BAL) cultures revealed
coccidioidomycosis. The patient was treated for 3 months with fluconazole
400 mg a day, and had no recurrent episodes of hemoptysis.
DISCUSSION: We report a patient with cavitary lung disease and
hemoptysis caused by coccidioidomycosis. It is estimated that 60% of the
infections with coccidiidomycosis are asymptomatic. Symptomatic pa-
tients may experience symptoms ranging from a mild respiratory illness to
Wednesday, October 29, 2003
Infectious Diseases II, continued
318S CHEST 2003—Case Reports

acute pneumonia(1). In a small number of cases symptoms persist for
more than 6 weeks. Coccidioidomycosis may disseminate and present as
multisystemic disorder. Persistent pulmonary coccidiidomycosis is char-
acterized with residual pulmonary nodules, cavities, fibrosis and bronchi-
ectasis (2,3). Serology, skin testing, culture and smear and pathologic
evaluation may play an important role in diagnosis of this disorder. The
vast majority of symptomatic patients do not require specific therapy,
however patients with severe or prolonged disease should be treated(1).
Amphotericin, fluconazole and itroconazole are used for treatment for
coccidioidomycosis. Surgery may also play an important role in treatment
of this disorder. Here we were using novel radiographic technique (3D
CT pulmonary artery angiography) to better describe pulmonary vascular
anatomy.Three-dimensional computed tomographic angiography can eas-
ily and quickly be obtained and provide very useful visual information in
various diseases.
CONCLUSIONS: We demonstrated that coccidioidomycosis may
cause necrotizing granulomatous inflammation of vascular structures in
association with destruction of pulmonary parenchyma and also forming of
vascular anomaly. Finally, it is important to recognize the role of 3D CT
pulmonary artery angiography in diagnosing abnormalities of pulmonary
vasculature.
REFERENCES:
1. Einstein H.E., Johnson R.H., Coccidioidomycosis: New aspects of
epidemiology and therapy. Clin. Infect. Dis. 16: 349-356, 1993
2. Stevens D.A.,: Coccidioidomycosis, N.Engl. J. Med., 332: 1077-
1082, 1995
3. Batra P., : Pulmonary Coccidioidomycosis, J. Thoracic Imag. 7:
29-38, 1992
DISCLOSURE: R. Tomic, None.
CAVITARY LUNG DISEASE IN A DRY-ROT WORKER
Samuel J. Evans, MD*; Nicholas J. Kenyon, MD; Eva Y. Lew, MD; Brian
M. Morrissey, MD; Division of Pulmonary and Critical Care Medicine,;
University of California Davis. University of California, Davis, Sacra-
mento, CA
INTRODUCTION: Pulmonary infection with Sporothrix schenckii (S.
schenckii) may mimic tuberculosis with an insidious progression of upper
lobe predominant fibrocavitation. We describe a young man with severe
primary pulmonary sporotrichosis in whom the definitive diagnosis was
not established for five years after the onset of symptoms.
CASE PRESENTATION: A 40 year old carpenter and dry-rot
repairman with a five-year history of cough complained of worsening
dyspnea on exertion and infrequent night sweats. He produced
1
⁄
2
cup of
sputum daily. He had seen numerous physicians. Extensive evaluations for
tuberculosis had been negative. Therapeutic attempts with antibiotics had
failed. He smoked 2 packs of tobacco daily for 28 years. He drank 6 to10
beers daily. Physical exam was significant for hyperresonance on chest
percussion and bronchial breath sounds loudest on the right. Digital
clubbing was present. Chest X-ray showed hyperlucent regions in both
upper lobes and loss of volume on the right. Thoracic computerized
tomography confirmed bilateral bronchiectasis and thick walled irregular
cavities. Coarse nodules with a ‘tree and bud’ appearance were seen
throughout both lungs. A bronchoalveolar lavage (BAL) specimen was
obtained from the right middle lobe. Fungal culture from the BAL grew
S. schenckii. Therapy with oral itraconazole was initiated with improve-
ment in symptoms
DISCUSSION: S. schenckii is a dimorphic fungus commonly found
in soil and plant matter. Lung infection is thought to be caused by
inhalation or aspiration of S. schenckii spores.
1,2
An occupational
association is suggested as the disease has been reported in florists,
construction and agricultural workers, and lumber salesmen. Men
appear to be affected more often than women. Most cases present
between the ages of 30 and 60.
1,2
Impaired host defense is frequently
implicated as an etiological factor. Alcohol abuse is a prominent
association. The disease is difficult to recognize and confirm. The
differential diagnosis includes other fungal diseases, mycobaterial
infection, and malignancy. Tuberculosis is the most frequently sus-
pected diagnosis before confirmation of sporotrichosis.
1,2
Chest imag-
ing often shows cavitary lesions and parenchymal densities with an
upper lobe predominance. Noncavitary presentations have been de-
scribed.
3
The optimal therapy is unknown. Amphotericin B has been
used to treat cavitary disease, however overall cure rates with the sole
use of this agent are poor. Itraconazole is also active against S.
schenckii and should be considered.
4
The newer antifungal agent
voriconazole has not been shown to have enhanced activity against S.
schenckii.
5
Surgical resection alone has been successful in a limited
number of cases. The highest cure rates appear to result from the
combined use of antifungals with surgical resection. Intracavitary
antifungal treatment may be a consideration in the poor surgical
candidate.
CONCLUSION: Pulmonary infection with S. schenckii is often insid-
ious and the diagnosis is frequently delayed as our case illustrates.
Tuberculosis is most often suspected before S. schenckii is identified. In
young male patients with upper lobe predominant fibrocavitary lung
disease and a history of alcohol abuse, pulmonary sporotrichosis must be
considered within the differential diagnosis; especially after a thorough
mycobacterial work-up has proven negative. Management recommenda-
tions involve the combined use of antifungals with surgical resection.
REFERENCES:
1. England DM, Hochholzer L. Primary pulmonary sporotrichosis.
Report of Eight Cases with Clinicopathologic Review. Am J Surg
Pathol 1985 Mar;9(3):193-204
2. Pluss JL, Opal SM. Pulmonary Sporotrichosis: Review of Treatment
and Outcome. Medicine 1986 May;65(3):143-53
3. Comstock C, Wolson AH. Roentgenology of Sporotrichosis. Am J
Roentgenol Radium Ther Nucl Med 1975 Nov;125(3):651-5
4. Kauffman CA, Hajjeh R, Chapman SW. Practice Guidelines for the
Management of Patients with Sporotrichosis. Clinical Infectious
Diseases 2000:30:684-687
Wednesday, October 29, 2003
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 319S
CASE REPORTS

5. McGinnis MR, et al. Sporothrix schenckii Sensitivity to Voricon-
azole, Itraconazole and Amphotericin B. Med Mycol 2001 Aug;
39(4):369-71
DISCLOSURE: S.J. Evans, None.
Tracheal and Endobronchial Diseases
2:00 PM - 3:30 PM
GLOMUS TUMOR OF THE TRACHEA
Hassan F. Nadrous, MD*; Mark S. Allen, MD; Jim Jett, MD. Mayo Clinic,
Rochester, MN
INTRODUCTION: Glomus tumors are derived form specialized cells
surrounding arteriovenous anastomosis. This is the 16th reported case
involving the trachea and the first where three-dimensional reconstruction of
computed tomography was used to clearly define extraluminal extension.
CASE PRESENTATION: A 39 nine years old male had intermittent
hemoptysis for two and a half years. His hemoptysis was attributed to
gastroesophageal reflex. A screening computed tomography (CT) of the
chest showed a tracheal mass. A bronchoscopy and biopsy of the mass
showed it was a glomus tumor. The axial and reformatted images
computed tomography (CT) of the chest with virtual bronchoscopic
images were performed and clearly demonstrated extraluminal extension.
A bronchoscopic-assisted sleeve resection with end to end anastomosis
was carried out without complication and pathologic evaluation confirmed
the extraluminal extension of tumor.
DISCUSION: Glomus tumor originates in the modified smooth
muscle cells of the glomus body. Four distinctive subtypes have been
classified and these are classic glomus tumor, glomangioma, glomangio-
myoma, and oncocytic glomus tumor.
1
Glomus tumor of the trachea is
very rare with only 16 cases including our case have been described.
2,3
.
None of the tumors was locally invasive or had metastasis, however
extratracheal extension was seen in four cases including our patient.
CONCLUSIONS: Glomus tumor of the trachea is rare. Sleeve resec-
tion of the trachea and primary reconstruction is the treatment of choice.
CLINICAL IMPLICATIONS: Three dimensional reconstruction CT
is helpful to evaluate extraluminal extension of a tracheal glomus tumor
prior to surgery.
REFERENCES:
1. Shin DH, Park SS, Lee JH, et al. Oncocytic glomus tumor of the
trachea. Chest. 1990;98:1021-1023.
2. Gowan RT, Shamji FM, Perkins DG, et al. Glomus tumor of the trachea.
Ann Thorac Surg. 2001;72:598-600. 3. Menaissy YM, Gal AAMansour KA
Glomus tumor of the trachea. Ann Thorac Surg. 2000;70:295-297.
3. Menaissy YM, Gal AAMansour KA Glomus tumor of the trachea.
Ann Thorac Surg. 2000;70:295-297.
DISCLOSURE: H.F. Nadrous, None.
CHURG-STRAUSS SYNDROME PRESENTING AS VOCAL
CORD AND ENDOBRONCHIAL NODULES
Steve K. Mashour, MD*; Samuel A. Allen, DO; Chad Stone, MD; Alan D.
Betensley, MD. Henry Ford Hospital, Detroit, MI
INTRODUCTION: Churg-Strauss syndrome (CSS) is an uncommon
disease entity first described in 1951.
1
Only one previous case has
documented endobronchial nodules, which were shown to be granulo-
mas.
2
The following case is the first known description of CSS presenting
with both vocal cord and endobronchial nodules due to eosinophilic tissue
infiltration without granulomas.
CASE PRESENTATION: A 48-year-old male with asthma presented
with hemoptysis and weight loss. Exam revealed both wheezes and crackles.
His white blood cell count was 13,500/mm
3
with 5,400/mm
3
eosinophils.
ANA and rheumatoid factor were negative and p-ANCA titer was 1:80.
Computed tomography of the chest showed tracheal wall thickening,
multiple pulmonary infiltrates, and scattered nodular areas of ground glass.
Bronchoscopy showed multiple raised white lesions on the vocal cords,
trachea, and the left and right main stem bronchi.
Endobronchial biopsies revealed interstitial eosinophilia and capillaritis.
Fungal cultures and acid-fast bacilli cultures were all negative. Prednisone
was started; the patient noted marked improvement in his symptoms, and
peripheral blood eosinophilia decreased to 1% after two days.
DISCUSSION: CSS has the classical triad of allergic rhinitis, asthma,
and peripheral blood eosinophilia. Approximately 10% of patients known
to have one of the major vascultides has CSS. The ACR V diagnostic
criteria include asthma, eosinophilia, mono- or polyneuropathy, non-fixed
pulmonary “infiltrates,” paranasal sinus abnormalities, and extravascular
eosinophils. Presence of four of these criteria is diagnostic of CSS with a
sensitivity of 85% and specificity of 100%.
CONCLUSIONS: This is an unusual case of CSS presenting with vocal
cord and endobronchial nodules, highlighting the fact that the eosino-
philic tissue infiltration can appear anywhere along the respiratory tract
and can appear as nodular lesions on bronchoscopic visualization. CSS
should be considered in the differential diagnosis of vocal cord or
endobronchial nodules.
Wednesday, October 29, 2003
Infectious Diseases II, continued
320S CHEST 2003—Case Reports

REFERENCES:
1. Churg J Strauss L. Allergic granulomatosis, allergic rhinitis, and
periarteritis nodosa. AmJPathol1951;27:277-301.
2. Alvarez-Sala R, et al. Congestive cardiomyopathy and endobronchial
granulomas as manifestations of Churg-Strauss syndrome.
PostgradMedJ1995Jun;71(836):365-6.
DISCLOSURE: S.K. Mashour, None.
Wednesday, October 29, 2003
Tracheal and Endobronchial Diseases,
continued
CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 321S
CASE REPORTS

NOT A BRONCHOLITH: AN UNEXPECTED CAUSE OF BRON-
CHIAL OBSTRUCTION
Ziad C. Boujaoude, MD*; Sajjad Shah, MD; Hikmat N. Dagher, MD;
Robin Gross, MD; Melvin R. Pratter, MD; Thaddeus Bartter, MD.
Robert Wood Johnson Medical School at Camden, Camden, NJ
INTRODUCTION: Airway obstruction from proximal tracheobron-
chial lesions is well-described, but for a projectile to obstruct a main
bronchus is almost unheard of.
CASE PRESENTATION: A 30-year-old man presented to the
pulmonary clinic with dyspnea on exertion, stridor, and intermittent
wheezing. He had a past history of gunshot wound to the chest ten
months prior associated with tracheostomy and mechanical ventilation.
He had cough intermittently productive of purulent sputum without
systemic symptoms.
Physical examination revealed a tracheal scar, inspiratory stridor, and
expiratory wheezing heard mostly over the anterior chest. Spirometry with
lung volumes revealed mixed restrictive and obstructive physiology, with
FEV1 35% predicted and TLC 69% of predicted. The FEV1 increased
35% after bronchodilators. Chest x-ray (Graphic 1) demonstrated a
mediastinal bullet.
The clinical diagnosis was asthma and unexplained restriction. He
underwent flexible fiberoptic bronchoscopy to rule out tracheal steno-
sis. Bronchoscopy revealed a bullet causing 95% obstruction of the left
mainstem bronchus (Graphic 2). Follow-up CT scan revealed left
mainstem location of the bullet with left volume loss and hypoperfu-
sion on the left. The patient was referred to surgery for sleeve
resection.
DISCUSSION: Broncholiths are now rare. Aspiration of foreign bodies
is relatively common, but to our knowledge this is the first reported case
of a foreign body traversing the thorax to lodge in the bronchial tree. In
this case, near-complete left bronchial obstruction appears to have caused
hypoventilation of the left lung, hypoperfusion, and volume loss on that
side. This resulted in restrictive physiology. The most plausible explana-
tion for the bronchodilator response is concomitant asthma.
CONCLUSION: The implausible can occur. This is an unusual and
interesting case of proximal airway obstruction and its possible physiologic
profile.
DISCLOSURE: Z.C. Boujaoude, None.
ENDOBRONCHIAL STRICTURE FROM TUBERCULOSIS
TREATED WITH LOCAL STEROID INJECTION AND BRON-
CHOPLASTY
Cristina A. Reichner, MD*; Michael N. Solomon, MD; Julie-Anne
Thompson, MD; Charles A. Read, MD FCCP; Eric D. Anderson, MD
FCCP. Georgetown University Medical Center, Washington, DC
INTRODUCTION: Endobronchial tuberculosis (EBTB), defined as
tuberculous infection of the tracheobronchial tree, is a rare manifestation
of a common disease. As a complication of pulmonary tuberculosis, EBTB
can result in endobronchial stenosis, atelectasis, bronchiectasis and ob-
structive pneumonia. The treatment of EBTB beyond antituberculous
therapy remains uncertain.
CASE PRESENTATION: We report a case of a 39 year old Chinese
female with recurrent endobronchial tuberculosis who presented with
worsening dyspnea, wheezing and developed complete obstruction of the
left mainstem bronchus 3 months after initiating antituberculous therapy.
Admission chest X-ray (figure 1) showed significant air-space disease and
volume loss in the left lung, mediastinal shift to the left and over expansion
of the right lung. CT scan of the chest demonstrateda1cmendobronchial
occlusion of the left mainstem bronchus (figure 2). Bronchoscopically, the
left mainstem bronchus was occluded by a fibrotic web (figure 3). After
excluding active tuberculosis, she underwent flexible bronchoscopy with
endobronchial steroid injection of 3cc of 40mg/ml triamcinolone ace-
tonide at the site of obstruction (figure 4). She was also given 60mg of oral
prednisone daily. Two weeks later, repeat bronchoscopy demonstrated a
small opening at the site of the left mainstem bronchus obstruction (figure
5). Successful balloon dilatation (figures 6 and 7) and endobronchial stent
placement (figure 8) were then performed with marked improvement in
left lung volume (figure 9).
DISCUSSION: The pathogenesis of EBTB is thought to be initi-
ated when Mycobacteria bacilli directly implant from the lung paren-
chyma, infiltrate the airway from mediastinal lymph nodes or spread
Wednesday, October 29, 2003
Tracheal and Endobronchial Diseases,
continued
322S CHEST 2003—Case Reports

hematogenously. The incidence ranges from 10 to 39% and is higher in
females in most series. The clinical features of EBTB are variable.
Cough and fever are the most common symptoms and are often
associated on physical exam with decreased breath sounds, localized
wheeze and rhonchi. Chest X-rays show consolidation or volume loss.
The main differential diagnoses include asthma, foreign body aspira-
tion, bronchial carcinoma, inflammation, bacterial and fungal infec-
tions and sarcoidosis. The optimal treatment other than antitubercu-
lous therapy remains controversial. Systemic steroids have shown
inconsistent responses. One previous report by Verhaege et al. showed
resolution of EBTB with submucosal injection of methylprednisolone.
As in our patient, endobronchial steroid injection offers an alternative
to surgical resection which had been established as the treatment for
strictures secondary to EBTB.
CONCLUSION: For stenotic EBTB, balloon dilatation with endo-
bronchial stent placement may be feasible if local steroid injection therapy
is able to open an area of complete stenosis.
REFERENCES:
1. Shim YS. Endobronchial tuberculosis. Respirology 1996; 1:95-106.
2. Lee JH, Park SS, Lee DH, et al. Endobronchial Tuberculosis
revisited: Clinical and bronchoscopic features in 121 cases. Chest
1992; 102: 990-994.
3. Verhaege W, Noppen M, Meysman M, et al. Rapid healing of
endobronchial tuberculosis by local endoscopic injection of cortico-
steroids. Monaldi Arch Chest Dis 1996; 5: 391-393.
4. Wan IYP, Lee TW, Lam HCK, et al. Tracheobronchial stenting for
Tuberculosis airway stenosis. Chest 2002; 122: 370-374.
Wednesday, October 29, 2003
Tracheal and Endobronchial Diseases,
continued
CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 323S
CASE REPORTS

DISCLOSURE: C.A. Reichner, None.
SUPRAGLOTTIC HEMANGIOMA, BRONCHOSCOPY DIAG-
NOSTIC OF AN ATYPICAL PRESENTATION OF LARYNGEAL
STRIDOR, ASSOCIATED WITH DOUBLE VASCULAR RING IN
AN INFANT
Erick W. Bringas, Resident*; G. Teresita Gutie´rrez, MD; M. Jose Cota,
MD; Daniel Carrasco, MD; M. Silvia Lule, MD. INER, Mexico D.F.,
Mexico
INTRODUCTION: Hemangioma represent only the 10% of the
laryngeal pathology usually in the subglottic space, been the cavernous
and capillary histologic types the most common, and the laryngeal stridor
the mainly sing of clinic suspicion.
We present an extremely rare case of Supraglottic hemangioma asso-
ciated to extrinsic compression in a pediatric patient.
CASE PRESENTATION: Male infant of eight month of age, without
family history of cardiovascular disease, Delay on grown-up and ponderal
deficit of 25%, with a history of laryngeal stridor since born, and remission
Wednesday, October 29, 2003
Tracheal and Endobronchial Diseases,
continued
324S CHEST 2003—Case Reports

of multiple exacerbation , until it became continuous; a diagnostic of
laringotracheitis and recurrent neumonia was make, requiring hospitallary
treatment in four times. Eigth days before admition present with biphasic
stridor, without modification on postural changes, a treatment for larin-
gotracheitis was stared for five days without respond and respiratory
distress that required ventilatory support, and was send from a pediatric
hospital to our institution (Instituto Nacional de Enfermedades Respira-
torias of Me´xico D.F.) The Chest X ray showed complete intermitent left
lung atelectasi and dynamic hyperinsuflation of contralateral lung.The
whole bioquimical laboratory were in normal limits , arterial gas presented
respiratory acidosis.
A supraglottic exofitic red lesion of soft surface was present in
bronchoscopy, just on the rigth ventricular band, occluding 90% of the
hypofaring and the glottic and supraglottc lumen by a valvular efect, then
was postulated the clinic suspection of hemangioma and extrinsic com-
pression of the main rigth bronchus.
The echocardiography reported moderate to severe pulmonary arterial
hypertension with structural hart between normal limits; The helicoidal
computed tomography suggested the posibility of a vascular ring, without
the posibility of performed cardiac catheterization due to an a Strepto-
coccus pyogenes bacteremia.
A tracheotomy after lesio´n diagnostic was performed, with a satisfactory
evolution weaning of mechanical ventilation around ten days; nevertheless
he presented a sudden cardiorespiratory arrest due to plugging in the
canul.
The necropsy reported: supraglottic cavernous hemangioma and double
vascular ring. Sling kind of the left pulmonary artery and right aortic arc.
DISCUSSION: This is an unusual case presentation of supraglottic
hemagioma due to the mayority of cases have a subglottic presentation
and the association with a congenital vascular malformation of the left
pulmonary artery and rigth aortic arc makes the case of interest due to the
few cases reported in the literature and the posibility of a new multiple
syndrome reported previously.
CONCLUSIONS: The evaluation of stridor in pediatric population
with bronchoscopy permit not only the valoration of the airway and to
make some diferential diagnostic like foreing body, moreover it is an
impotant diagnostic tool in this lesion that maybe evolve a special
syndrome in this group of patient.
DISCLOSURE: E.W. Bringas, None.
PRIMARY MELANOMA OF THE TRACHEA
Cateline Sirois, MD*; Richard I. Inculet, MD; Keith Kwan, MD. London
Health Sciences Center, London, ON, Canada
INTRODUCTION: Primary malignant melanoma of the trachea is
uncommon. Only six cases have been reported worldwide. A patient who
underwent a tracheal resection for this rare pathology is presented.
CASE PRESENTATION: The patient is a 64-year-old male smoker
who presented with cough and hemoptysis. He did not have any
symptoms of airway obstruction. Initial investigation was performed in a
community hospital. A flexible bronchoscopy demonstrated a lower
obstructing tracheal tumor. Biopsies showed malignant cells. Computed
tomographic scan of the chest and upper abdomen demonstrated the
tracheal tumor .
There were no enlarged lymph nodes or evidence of metastases. A bone
scan and an abdominal ultrasound were normal. Pulmonary function test
were consistent with large-airway obstruction.
The patient was transfered to our institution and taken to the operating
room. Under general anesthesia the patient was intubated with a single
lumen tube and a flexible bronchoscopy was performed. The mass was
seen to occlude three quarters of the lumen of the distal trachea. The
tumor was polypoid and had a pedicle, which was attached about three
centimeters above the carina, on the right lateral wall. The surface of the
mass was very smooth and pink in color. The tumor was removed to
provide relief of the airway obstruction. An endoscopic snare, with
electrocautery, was used to divide the pedicle. The mass was then snared
and removed together with the endotracheal tube. The patient was
discharge home the following day.
Wednesday, October 29, 2003
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CHEST / 124/4/OCTOBER, 2003 SUPPLEMENT 325S
CASE REPORTS

The pathology was reported as melanoma. It was a 1.9 cm polypoid
tumor. Within the subepithelial tissue were nests and sheets of epithelioid
cells. Mitotic figures were numerous. Granular brown pigment were
identified in occasional epithelioid cells and in adjacent histiocytes .
Immunohistochemestry showed strong positive staining of the epithelioid
cells for S 100, HMB45, and melan-A.
The patient was brought back electively to the operating room for a
tracheal resection to remove the residual tumor base. A flexible bronchos-
copy showed residual melanotic tumor at the site of the pedicle, and two
adjacent satellite nodules. A right thoracotomy was performed. Injection
of lymphazurin (isosulfan blue) into the tracheal wall was done to locate
a sentinel node. Frozen section of this node revealed no metastatic
melanoma. The tracheal resection was performed removing four tracheal
rings. The patient was discharged home, uneventfully, on the seventh
post-operative day.
The pathologic analysis of the resected trachea confirmed the presence
of residual melanoma. No benign junctional melanocytes were demon-
strated.
DISCUSSION: Primary melanoma of the trachea is an extremely rare
condition. The pathological appearance may or may not suggest a primary
versus a metastatic tumor. Therefore, it is mandatory to complete a careful
physical examination of all potential primary sites. We were satisfied no
other primary site existed in this patient.
The tumor, at initial presentation, was almost completely obstructing
the distal trachea. Although it was first biopsied by flexible bronchoscopy
under mild sedation, this is not recommended because of the potential for
bleeding and ensuing complete airway obstruction. It is recommended
that the patients with an obstructing tracheal tumor be managed in the
operating room, with a rigid bronchoscope available.
Sentinel node localization is the standard of practice for intermediate
risk cutaneous melanoma. This report describes the first attempt at
utilizing this for a tracheal neoplasm. It will require further study as to its
applicability.
CONCLUSION: Primary malignant melanoma of the trachea is very
rare. Tracheal resection is the treatment of choice for localized tumors.
REFERENCES:
Duarte IG, Gal AA, Mansour KA. Primary malignant melanoma of the
trachea. Ann Thorac Surg 1998;65(2):559-60.
Li W, Ellerbroek NA, Libshitz HI. Primary malignant tumors of the
trachea. Cancer 1990;894-9.
DISCLOSURE: C. Sirois, None.
Cancer I
2:00 PM - 3:30 PM
MICROANGIOPATHIC HEMOLYTIC ANEMIA AS THE FIRST
MANIFESTATION OF LUNG ADENOCARCINOMA
Pablo E. Molina, MD*; Jay I. Peters, MD. University of Texas Health
Science Center at San Antonio, San Antonio, TX
INTRODUCTION: Microangiopathic hemolytic anemia (MAHA) is
an uncommon complication of mucin-producing adenocarcinomas usually
of the stomach or breast. We present a case of MAHA as the first
manifestation of bronchoalveolar carcinoma (BAC). This is only the third
reported case of MAHA associated with lung cancer.
CASE PRESENTATION: A 55 year-old never smoking female
presented with fatigue and dyspnea on exertion for 4 months. Evalu-
ation revealed Coombs negative MAHA. Serologies for collagen
vascular diseases were negative. A bone marrow biopsy revealed
erythroid hyperplasia. Her therapy included plasmapheresis, two
cycles of cyclosphosphamide, prednisone, 20 units of packed red blood
cells and a splenectomy without improvement. On arrival to our
tertiary care center, laboratory revealed a hematocrit of 33.7%,
hemoglobin 10.4 g/dL, platelet count of 228,000/mm3, INR 1.1, PTT
29 seconds, reticulocyte count of 16%, LDH 1219 units/mL and a
haptoglobin ⬍10 mg/dL. The peripheral blood smear revealed many
schistocytes and microspherocytes. Mammogram and abdominal/pelvic
computed tomography (CT) were unremarkable. Chest CT revealed a
left lower lobe consolidation and subcarinal and left hilar lymphade-
nopathy suggestive of lymphoma. The patient underwent bronchos-
copy with transbronchial biopsy and transbronchial needle aspirate
which was sent for flow cytometry. Both revealed mucin-producing
adenocarcinoma consistent with BAC. She received chemoradiation
with carboplatinum and taxol with partial remission of her MAHA.
DISCUSSION: MAHA is a well-documented but rare complication of
disseminated adenocarcinomas, usually of the stomach and breast, but has
been reported with other organ malignancies including pancreas, colon
and lung. MAHA is a marker for poor prognosis, and survival in
non-treated patients has ranged from 3 to 61 days. Rapid initiation of
chemotherapy usually controls the microangiopathic process.
CONCLUSION: MAHA is an infrequent manifestation of lung ade-
nocarcinoma of the lung. Extensive evaluation for malignancy, including
lung cancer, should be performed in patients presenting with Coombs
negative MAHA.
DISCLOSURE: P.E. Molina, None.
PULMONARY CRYPTOCOCCOMA MIMICKING MALIGNANCY
IN A NORMAL HOST
Hikmat N. Dagher, MD*; Thaddeus Bartter, MD; Wajdi S. Kfoury, MD;
Ziad C. Boujaoude, MD; Melvin R. Pratter, MD. Robert Wood Johnson
Medical School at Camden, Camden, NJ
INTRODUCTION: We report a cryptococcoma in a normal host
presenting as a pulmonary mass with a “false positive” 99mTc depreotide
scan.
CASE PRESENTATION: A 48-year-old man presented with a 4x3 cm
mass not present 16 months earlier. Computed tomographic scans
confirmed the presence of an isolated left lower lobe mass surrounded by
normal parenchyma. The patient denied all respiratory and systemic
symptoms. Past medical history was significant only for “walking pneu-
monia” years earlier. He denied human immunodeficiency virus risk
factors, tuberculosis exposure, significant travel history, and use of
tobacco, alcohol, and recreational drugs. Physical examination was unre-
markable.
A 99mTc depreotide scan (Graphic 1) showed intense left lower lobe
uptake corresponding with the roentgenographic abnormality. Bronchos-
copy was unremarkable, with negative smears, cultures, acid fast stains,
fungal stains, and cytology.
The mass was resected. Pathology revealed a solitary peripheral mass.
Histologic section (Graphic 2) demonstrated extensive granulomatous
involvement of the pulmonary parenchyma with multinucleated giant cells
containing abundant GMS-positive yeast forms. Their capsules were
focally but not uniformly positive for mucicarmine (Graphic 3). The
staining is characteristic for Cryptococcus neoformans.
The patient completed six weeks of oral Fluconazole.(1) He remains
asymptomatic.
DISCUSSION: To our knowledge, this is the first reported case of
a depreotide-positive cryptococcoma. Cryptococcoma in a normal host
is rare, though reported.(1) This patient was a non-smoker, and the
mass had an apparent rapid growth rate which made malignancy less
likely, but the lack of an alternative benign diagnosis after bronchos-
copy mandated further evaluation. The depreotide study was done with
the thought that a negative study would markedly decrease the
possibility of cancer and might allow conservative management. As the
case evolved, the markedly positive study (coupled with patient
preference) led to a decision to resect.
Wednesday, October 29, 2003
Tracheal and Endobronchial Diseases,
continued
326S CHEST 2003—Case Reports

In a recent multicenter study using depreotide, sensitivity and
specificity for malignancy were 96.6% and 73% respectively,(2) similar
to those of fluorine-18-fluorodeoxyglucose positron emission tomogra-
phy (PET scanning).(3) Both modalities do have “false positives” in the
sense that a positive scan can represent a non-malignant biological
process. Reported false positives for depreotide include granulomas,
hamartomas, and rounded atelectasis.(3,4,5) Reported false positives
for PET include caseating granuloma, histoplasma, cryptococcoma,
schwannoma, fibrous mesothelioma, tuberculoma, rheumatoid nodule,
and “inflammatory lesion.”(6,7,8) The two modalities are similar not
only in their diagnostic accuracy but also in the type of biological
process which will create a false positive. One PET-positive crypto-
coccoma has been reported; this depreotide-positive cryptococcoma is
an example of the tendency of both modalities to be positive in areas
of granulomatous inflammation.
CONCLUSION: Depreotide and PET scanning can at times be very
helpful in the diagnostic work-up of solitary pulmonary masses. Both can
be false-positive in cases of granulomatous disease.
REFERENCES:
1. Aberg JA, Mundy LM, Powderly WG. Pulmonary cryptococcosis in
patients without HIV infection. Chest 1999;115:734-40
2. Leef JL, III, Klein JS. The solitary pulmonary nodule. Radiol Clin
North Am 2002;40:123-43
3. Blum J, Handmaker H, Lister-James J et al. A multicenter trial with
a somatostatin analog (99m)Tc depreotide in the evaluation of
solitary pulmonary nodules. Chest 2000;117:1232-38
4. Blum J, Handmaker H, Rinne NA. The utility of a somatostatin-type
receptor binding peptide radiopharmaceutical (P829) in the evalu-
ation of solitary pulmonary nodules. Chest 1999;115:224-32
5. Thomas LP, Balingit AG, Morison DS et al. False-Positive techne-
tium 99m depreotide scintigraphy resulting from round atelectasis.
J Thorac Imaging 2003;18:97-99
6. Patz EF, Jr. Evaluation of focal pulmonary abnormalities with FDG
PET. Radiographics 2000;20:1182-85
7. Dewan NA, Shehan CJ, Reeb SD et. al. Likelihood of malignancy in
a solitary pulmonary nodule. Chest 1997;112:416-22
8. Knight SB, Delbeke D, Stewart JR et al. Evaluation of pulmonary
lesions with FDG-PET. Chest 1996;109:982-88
DISCLOSURE: H.N. Dagher, None.
PULMONARY HYPERTENSION IN A PATIENT WITH MYELO-
FIBROSIS
Joe G. Zein, MD*; Tina R. Chou, MD; Patricia A. Tietjen, MD, FCCP.
Saint Vincent Hospital and Medical Center, New York, NY
INTRODUCTION: Chronic myeloproliferative disorders (CMD) are
characterized by clonal expansion of a multipotent hematopoietic progen-
itor cell with the overproduction of one or more of the formed elements
of the blood. Pulmonary complications are usually infectious or thrombo-
embolic. Pulmonary hypertension (PH) is higher than expected in patients
with CMD (1). The contributing role of chemotherapy, mainly Anagrelide
and Busulfan may be underestimated. We are reporting a case of PH in a
patient with idiopathic myelofibrosis.
CASE PRESENTATION: 78 year-old man, former smoker (20 pack
years, quit 40 years prior) with CMD (diagnosed 1
1
⁄
2
years PTA),
presented with a history of increasing fatigue and dyspnea on exertion for
6 months. He denied chest pain, cough or palpitations. He denied any
other constitutional symptoms or toxic habits. His past medical history was
also significant for bleeding gastric ulcers treated with partial gastrectomy
(1960), HTN and hypercholesterolemia. He had been on lisinopril,
Simvastatin and Aspirin. On physical exam he was afebrile, P⫽90,
BP⫽150/62, RR⫽18. The remainder of his physical exam was normal
except for a grade II/VI systolic murmur that radiated to the left sternal
border, and hepatosplenomegaly. Laboratory data: WBC⫽ 21 K, Hct⫽ 26
%, platelet count (plt)⫽ 521 K, LDH⫽2557 U/L, normal liver function
tests. Chest roentgenogram showed blunting of the of the right costo-
phrenic angle. The remainder of the lung fields appeared well aerated.
The cardiac silhouette was unremarkable. Transthoracic echocardiogra-
phy revealed peak RVSP⫽ 55-63 mmHg, mild MR and mild TR. The LV
and RV functions were normal. . Pulmonary function tests (PFTs) showed
an FEV1⫽3.29 liters (157% of predicted), a TLC⫽7.94 (133%of pre-
dicted) and DLCO⫽16.6(74% of predicted). A perfusion lung scan in the
presence of a normal chest roentgenogram, and a helical chest CT were
negative for pulmonary embolism.
DISCUSSION: The etiology of the pulmonary hypertension was
unclear in this patient who denied using any anorectic drugs and was
never treated with any chemotherapeutic agents with direct effect on
pulmonary vasculature or parenchyma. Thromboemblic disorders were
extremely unlikely given the results of the lung scan and the helical chest
CT. The TTE failed to demonstrate any evidence of congenital heart
disease. Other etiologies for secondary pulmonary hypertension, such as
connective tissue disorders, HIV infection, carcinoid syndrome, or chronic
liver disease were unlikely (serologies were negative- ANA, RH factor,
anti-cardiolipin Ab).
The exact mechanism for PH in patients with CMD is still unknown.
Infiltration of the lung parenchyma by hematopoietic cells, thromboem-
bolic disorders and Left ventricular failure have all been incriminated (2).
Plts and plts derived growth factors have been advocated to play a central
role (1,2). Lowering plt counts may improve symptoms or delays the onset
of PH. Plt count was persistently elevated in this patient. Anagrelide, a
medication usually used to control plt count, is occasionally associated
with many cardiovascular and pulmonary side effects including PH. Its use
may be attempted mainly in patients with thrombocytosis, considering the
potential benefit in this serious condition. Anticoagulation and antiplatelet
therapy should be tried in the absence of bleeding diathesis and could be
beneficial.
CONCLUSION: PH associated with CMD is a serious condition,
associated with considerable morbidity and mortality and without proven
effective therapy. It should be suspected in patients with MDS presenting
with dyspnea. An attempt to lower platelets count could be helpful.
REFERENCES:
1. Dingli D; UtzJP; et al. Unexplained Pulmonary Hypertension in
Chronic Myeloproliferative Disorders. Chest 2001;120(3):801-8
2. Garcia-Manero G; Schuster SJ; et al. Pulmonary Hypertension in
Patients with Myelofibrosis Secondary to Myeloproliferative Dis-
eases. Am J Hematol 1999;60:130-135.
DISCLOSURE: J.G. Zein, None.
Wednesday, October 29, 2003
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75 YEAR OLD MALE WITH FATIGUE AND WEIGHT GAIN
Jameel F. Durrani, MBBS*, UMDNJ- New Jersey Medical School,
Wallington, NJ; James Donahoo, Professor of Cardiothoracic Surgery -
UMDNJ; Walter Strauss, Attending Pulmonologist, VA Medical Center,
NJ; Jin K. Choe, Attending Pathologist - VA Medical Center, NJ.
INTRODUCTION: A 75 year old male presenting with fatigue and
weight gain for 4-6 weeks.
CASE PRESENTATION: 75 year old Caucasian male with history of
spinal cord injury and paraplegia, presented with fatigue and weight gain
for 4-6 weeks. Examination showed blood pressure of 160/105, easy
bruising, paraplegia and a normal cardiorespiratory examination.
Initial potassium level was 2.3 mg/dl. It remained low despite potassium
replacement. Random serum cortisol level was 112 mcg/dl. Serum
aldosterone level was 4 ng/dl (N⫽⬍16). The plasma renin level mea-
surement was 0.39 ng/ml/hr (N⫽ 0.5-1.5 ). A low dose and high dose
Dexamethasone suppression test failed to suppress the cortisol level that
remained above 90 mcg/dl. Serum ACTH level was 1227 pg/ml (N⫽9-52
pg/ml. A chest, abdominal and pelvic CT revealed a 1.5 cm diameter
subpleural density noted at the base of right middle lobe. Bilateral adrenal
gland hyperplasia was noted. Pt underwent right middle lobe resection. A
postoperative whole body octreotide scan was negative for abnormal
uptake. A diagnosis was ectopic ACTH secreting subpleural carcinoid
tumor was made.
DISCUSSION: Pulmonary Carcinoid is a low-grade malignant neo-
plasm. Incidence is only 0.6- 2.4 % of all lung cancers. Mean age at
diagnosis is 50. Incidence is higher in caucasians. The lesions can be
central (80%) or peripheral (20%) including subpleural location. The
etiology and pathogenesis of carcinoid tumors are unclear. There is no link
with cigarette smoking or with most other agents known to be associated
with pulmonary carcinoma. The initial presentation can be vague. Histo-
pathology is similar in central and peripheral tumors showing neoplastic
cells that are small, argyrophilic, often distinctly spindled, grouped in
small nests with a somewhat whorled appearance.
Treatment includes surgical resection of the lesion.
CONCLUSION: Pulmonary subpleural carcinoid is a rare tumor
requiring a high index of suspicion for diagnosis.
DISCLOSURE: J.F. Durrani, None.
Figure1: Baseline Chest Xray and CT scan
Fig.2: H&E stain typical carcinoid tumor. 100X
Fig 3: ACTH positive cells (brown) on immunohistochemical staining.
100X
Wednesday, October 29, 2003
Cancer I, continued
328S CHEST 2003—Case Reports

PRIMARY MUCINOUS CYSTADENOCARCINOMA OF THE
LUNG
Khaleel R. Salahudeen, MD*; Brian Carlin, MD, FCCP; Peter Kaplan,
MD, FCCP; Karen Clarey, MD; Jason Robke, MD. Divisions of Pulmo-
nary and Critical care Medicine, Cardiothoracic Surgery and Pathology,
Drexel University School of Medicine, Allegheny General Hospital.,
Pittsburgh, PA
INTRODUCTION: Mucinous cystadenocarcinoma (MCAC)is an ep-
ithelial malignancy of the ovary. This is the first reported case of MCAC
presenting as a lung primary.
CASE PRESENTATION: This 49-year-old woman (25 pack year
smoker) presented with a dry cough and shortness of breath onexertion
over six months. She was without constitutional symptoms, weight loss or
abdominal pain. She had decreased breath sounds over the right chest. A
chest xray showed a large mass (ill defined borders) occupying the right
upper and mid lung fields.
Chest CT scan showed a large cystic lesion encompassing entire right
upper lobe extending to the posterior mediastinum and right middle lobe
collapse. Bronchoscopy showed extrinsic compression of the posterior
trachea and right mainstem bronchus. A right thoracotomy with right
upper and middle lobectomy was performed. The pathology showed a
mucinous cyst adeno carcinoma with peribronchial lymph node involve-
ment No distant spread of the tumor was found Chemotherapy was
started and clinical followup is ongoing.
DISCUSSION: This woman presented with a unique case of MCAC of
the lung. The differential diagnosis of the cystic lesion included broncho-
genic or pericardial cyst, esophageal duplication cyst, neurenteric cyst,
and Echinococcus cyst. While usually asymptomatic in nature, each of
these may present with symptoms similar to this patient (namely, cough).
MCAC has not previously been reported to present with the lung as a
primary site. It is an epithelial ovarian malignancy and can be of a serous
or mucinous variety. Spread of the tumor to the abdominal cavity,
diaphragmatic surface and pleura has been reported.
CONCLUSION: This is the first reported case of MCAC presenting as
a lung primary. Such an entity should be considered in a patient
presenting with a cystic lesion of the lung.
DISCLOSURE: K.R. Salahudeen, None.
PRIMARY MUCOSA-ASSOCIATED LYMPHOID TISSUE (MALT)
LYMPHOMA OF THE BRONCHUS PRESENTING WITH UNI-
LATERAL WHEEZING
Khaleel R. Salahudeen, MD*; Brian Carlin, MD, FCCP; Peter Kaplan,
MD, FCCP; Karen Clarey, MD; Imran Bajwa, MD. Division of Pulmo-
nary and Critical Care Medicine and Pathology, Drexel University School
of Medicine, Allegheny General Hospital., Pittsburgh, PA
INTRODUCTION: Primary B cell lymphoma of the bronchial mucosa
is a rare entity. We present a case manifest initially with shortness of
breath and unilateral wheezing.
CASE PRESENTATION: A 74 y.o. woman (25 pack year former
smoker) presented with gradual onset of shortness of breath on exertion
and a nonproductive cough over a few months. She had an episode of
hemoptysis but denied chest pain, fever, night sweats, or weight loss. On
examination she was found to have wheezing noted only over the left
hemithorax. A chest xray was normal and a CT scan (chest) showed
circumferential narrowing of left mainstem bronchus. Bronchoscopy
revealed a nodular appearance of the mucosa with narrowing of the distal
left mainstem, left upper, and lower lobe bronchi. A similar nodular
appearance without luminal narrowing was seen over the right upper lobe.
Biopsies obtained from both sites showed mucosal-associated lymphoid
tissue lymphoma (MALT) of the B cell type. CT scans of the abdomen and
pelvis were normal.
DISCUSSION: Primary MALT lymphoma of the bronchus, a rare
entity, presented in this patient with unilateral wheezing. Initially the
differential diagnosis included airway/endobronchial lesions such as neo-
plasm, foreign body, or fibrosis from TB or histoplasmosis. MALT
lymphoma of the lung (one of the primary pulmonary lymphomas) is
defined as clonal lymphoid proliferation affecting one or both lungs
(parenchyma and/or bronchus) in a patient with no detectable extra
pulmonary involvement at diagnosis or during the subsequent 3 months.
Our patient’s presentation is unusual given the onset of unilateral
wheezing in the presence of a normal chest xray (most patients with
MALT lymphoma are asymptomatic with pulmonary parenchymal abnor-
malities noted on chest xray at presentation).
CONCLUSION: Primary MALT lymphoma of the lung is a rare entity.
We present this unusual MALT lymphoma with unique presenting feature
of unilateral wheeze.
DISCLOSURE: K.R. Salahudeen, None.
Tuberculosis
2:00 PM - 3:30 PM
CASE OF MILIARY TUBERCULOSIS WITH CYSTIC CT SCAN
CHANGES
Mamoun I. Najjar, MD*; Boris Medarov, MD; Anita K. Siddiqui, MD;
Eric Gottesman, MD; Brian Benoff, MD. Long Island Medical Center,
New Hydepark, NY
INTRODUCTION: In 2-6% of primary tuberculosis, massive,
lymphohematogenous dissemination of tubercle bacilli results in mil-
iary tuberculosis (MTB). Secondary activation of chronic tuberculosis
can also present as MTB. The characteristic radiological findings on
chest radiography (CXR) and computed tomography imaging (CT) are
innumerable fine nodules. Associated cystic changes are extremely
rare.
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CASE REPORT: An 88-year-old female patient from home presented
with cough, fever, lethargy progressing over 2 weeks. She was cachectic,
had low-grade fever, and bilateral basal crackles. CXR showed bilateral
diffuse nodular opacities and left apical large cavity. Chest CT showed
diffuse micronodular lesions, diffuse small cystic changes (image I & II).
The picture was consistent with miliary disease. Bronchoalveolar lavage
(BAL) was positive for Acid Fast Bacilli staining and transbronchilal
biopsy revealed necrotizing granulomatous inflammation. The patient was
started on isoniazide, rifampin, ethambutol and pyrazinamide. In three
weeks the BAL culture grew Mycobacterium tuberculosis sensitive to all
anti-TB agents. A follow up CT scan in 6 weeks showed improvement in
the miliary infiltrates, resolution of cystic changes (image III).
DISCUSSION: MTB has a wide range of CT findings. In addition to
diffuse 1-3 mm micronodules, it also can present as diffuse or localized
reticular opacities, ground glass attenuation, air trapping, consolidation,
cavitation, fibrosis, lymph nodes enlargement, and septal thickening. MTB
is a rare cause of cystic parenchymal changes. We report here an unusual
presentation of miliary tuberculosis associated with diffuse cystic changes
that were reversible and resolved after six weeks of antituberculous
therapy. The differential diagnosis of multiple cystic changes is extensive
and includes Pneumocystis carinii infection, lymphangioleiomyomatosis,
sarcoidosis, and Langerhans cell histiocytosis among others.
CONCLUSION: Pulmonary TB in adults is associated with unusual
radiographic manifestations for tuberculosis in up to one third of cases.
This report further supports atypical radiographic presentation of tuber-
culosis. In the proper clinical context, clinicians should have a high index
of suspicion for tuberculosis in the presence of atypical CT chest findings.
DISCLOSURE: M.I. Najjar, None.
CRYPTIC MILIARY TUBERCULOSIS WITH A PRODROME RE-
SEMBLING PANCREATITIS
Nicos P. Hadjiangelis, MD*; Doreen J. Addrizzo-Harris, MD, FCCP.
NYU Medical Center, New York, NY
INTRODUCTION: Miliary tuberculosis (TB) is a diagnostic challenge
for clinicians. Pancreatitis as a presenting symptom is an extremely rare
manifestation of miliary TB.
CASE PRESENTATION: A previously healthy 31-year-old male was
admitted because of severe nausea, malaise and night sweats for four days.
He denied other constitutional symptoms. He did not smoke, use alcohol
or drugs. He was born in the United States; lived in Manhattan and
worked for a computer company. He kept no pets and had no recent
travel. He took no medications and had no allergies.
He was febrile (103° F); heart rate was 79; and respirations were 17.
The blood pressure was 100/60 mm Hg, and the oxygen saturation was
100% on room air. The physical examination was unremarkable. The
admission labs were signficant for leukopenia(2.7 x 10^9/L), thrombocy-
topenia(118 x 10^9/L), mild transaminemia and high lipase (⫽260 U/L;
normal 66). He was HIV negative and PPD positive (12 mm).
The admission chest x-ray (CXR) was normal (below):
Computed tomographic (CT) evaluation of the abdomen was consistent
with pancreatitis. On the 12
th
hospital day the patient complained of
dyspnea. CXR and the CT scan images are shown:
Patient underwent bronchoalveolar lavage (BAL) and transbronchial
biopsy (TBBx) which showed alveolar macrophages without granulomas,
vasculitis or acid fast bacilli (AFB). Open lung biopsy (OLBx) revealed
multiple necrotizing granulomas:
The patient showed remarkable improvement on antituberculous ther-
apy and was discharged home three weeks latter on direct observed
therapy.
DISCUSSION: TB pancreatitis has a wide spectrum of non specific
clinical presentations. Rarely pancreatitis may be the only manifestation of
unrecognized miliary TB. The only case report of pancreatitis occuring
before miliary TB was puplished in 1978. Our case is unusual, because the
admission CXR was normal, sputum smears, BAL and TBBx were
negative for AFB. The diagnosis was confirmed with OLBx.
CONCLUSION: Miliary TB is generally a curable disease, which takes
many forms. A high index of suspicion and diagnostic persistence are
required for diagnosis.
Wednesday, October 29, 2003
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330S CHEST 2003—Case Reports

DISCLOSURE: N.P. Hadjiangelis, None.
AN UNUSUAL PRESENTATION OF “POST-TUBERCULOUS
BRONCHIECTASIS”
Saulat H. Fatimi, MBBS; Muhammad Irfan, MBBS; Ali B. Zubairi,
MBBS*. The Aga Khan University Hospital, Karachi, Pakistan
INTRODUCTION: Bronchiectasis is the permanent dilatation and
destruction of bronchi with potential to cause devastating illness.We
report a case of huge bronchiectatic cavity causing massive hemoptysis .
CASE PRESENTATION: A 45 year-old Afghani gentleman presented
to our hospital with massive hemoptysis of about 1 liter of bright red blood
over 2 days. He denied having fever,chest pain,night sweats or weight
loss.He had a history of Pulmonary tuberculosis 4 years back.For the past
1 year he had productive cough.He had a 40 pack-year history of smoking.
Clinical examination revealed a cachectic gentleman with heart rate of
110,BP 80/60,respiratory rate 26 and finger clubbing.
Chest examination revealed hyperresonance with diminished breath
sounds over the left anterior side.Rest of the physical examination was
unremarkable.
Laboratory investigation showed hemoglobin 10.8,WBC count 23000
with neutrophilia, platelet count 595,000,normal PT and PTT,and PaO2 of
53.3mmHg on room air.
Chest Radiograph (Figure 1) on admission showed large cavities with
volume loss in the left lung.CT Scan revealed extensive fibrosis and
bronchiectasis in the left lung.Right lung showed less severe fibrosis(Fig-
ure 2,3).Fiberoptic bronchoscopy revealed two large ’holes’ in the left
upper lobe bronchus communicating with a cavity (Figure 4).Bleeding
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vessels were identified in the cavity.Epinehrine was used for local control
of bleeding followed by left bronchial artery embolization (Figure 6,7,8).
DISCUSSION: Bronchiectasis is a disease of bronchi and bronchioles
involving a vicious cycle of transmural infection and inflammation.Post-
inflammatory bronchiectasis remains very common in developing coun-
tries as a sequelae to pulmonary tuberculosis.Anatomic changes occur in
bronchial vessels adjacent to bronchiectatic segments, such as bronchial
artery hypertrophy, expansion of peribronchial and submucosal bronchial
arteriolar plexus and anastomoses with the pulmonary artery bed. Bleed-
ing can originate from either enlarged, tortous bronchial vessels or rich
submucosal plexus in the wall of bronchiectatic segments.Rupture of such
a vessel can result in massive hemoptysis.
CLINICAL IMPLICATIONS: This represents a case of huge bron-
chiectatic cavities resulting in lifethreatening hemoptysis.Post tuberculous
bronchiectasis remains very common cause of massive hemoptysis in
developing countries like Pakistan.
REFERENCES:
1. Indian J.Pediatrics 2000 Feb;67(2):133-9
2. Singapore Med J 1999;Vol 40(02)
DISCLOSURE: A.B. Zubairi, None.
Wednesday, October 29, 2003
Tuberculosis, continued
332S CHEST 2003—Case Reports

ACUTE CEREBRAL EDEMA AS A MANIFESTATION OF PARA-
DOXICAL REACTION(PD) IN T.B MENINGITIS: EXCELLENT
RESPONSE TO STEROIDS
Vijay Baimeedi, MD*; Padmanabhan Krishnan, MBBS; Santi Dhar,
MBBS. Coney Island Hospital, Brooklyn, NY
INTRODUCTION: Paradoxical reaction is defined as documented wors-
ening of signs or symptoms of TB at pulmonary or extrapulmonary sites
during appropriate anti TB treatment after initial response to treatment.Para-
doxical reaction has been described in HIV negative and HIV positive
patients. Use of HAART has increased its frequency. We describe a patient
on treatment for TB meningitis who developed acute cerebral edema on the
addition of HAART that responded dramatically to steroids.
CASE PRESENTATION: 36 year old male with AIDS on treatment
for 7 weeks for TB meningitis and miliary TB with good response, was
admitted with worsening mental status. Viral load at initial presentation
was 522,000, CD4⫽31. CT head on diagnosis revealed cerebral edema
that resolved by discharge. 10 days before readmission pt was started on
HAART. On examination pt was lethargic without focal neurologic deficit
but did exhibit bilateral Babinski. CT head showed multiple areas of high
density in left thalamus, left frontal lobe with surrounding edema with
impending herniation. The patient was mechanically ventilated and was
kept on Mannitol, steroids, anti TB, and empiric anti Toxoplasma
treatment. At this time viral load had decreased to ⬍400 and CD4
increased to 91. Patient’s neurological status improved dramatically within
24 hours and was weaned off the ventilator. No focal neurological deficits
were seen except for old right hemiparesis. MRI done one week later
showed meningeal enhancement, no midline shift, no cerebral edema.
DISCUSSION: Paradoxical reaction has been described in CNS
Tuberculosis especially in patients with low CD4 and high viral load.In
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patients with CNS Tuberculosis worsening of mental status due to
cerebral edema secondary to paradoxical reaction exists especially when
HAART is added too early in the course in the course of treatment. The
paradoxical reaction is believed to be related to the development of
improved Mycobacterium tuberoculosis specific immune responses dur-
ing the course of anti TB treatment. There is increased proliferation of
peripheral blood mononuclear cells, and gamma interferon in response to
Mycobacterium tuberculosis specific antigens following HAART in HIV
infected patients.
CONCLUSION: In patients with TB meningitis the possibility of
cerebral edema developing due to paradoxical reaction must be consid-
ered when deciding the timing of HAART. Recognition of this phenom-
enon will allow for timely adition of steroids in patients with TB meningitis
who exhibit rapid deterioration of CNS function following HAART.
DISCLOSURE: V. Baimeedi, None.
EXTRAPULMONARY MYCOBACTERIUM TUBERCULOSIS: A
CHALLENGING PURSUIT
Elizabeth U. Bollenbacher, DO*; Sanjay Chawla, Pulmonary Critical Care
fellow; Patricia A. Tietjen, Chief Pulmonary Medicine. St Vincent’s
Hospital, New York, NY
INTRODUCTION: In 1994, 16% of newly reported cases of Myco-
bacterium tuberculosis involved extrapulmonary sites. Often, these cases
represent a diagnostic challenge. Many sites are not easily accessible and
require invasive procedures for diagnosis.
CASE PRESENTATION: A 27 year old Mexican male presented to
the ED with progressive shortness of breath, fever, night sweats, and a 10
pound weight loss over 1 month. He also had abdominal and chest pain of
5 months duration. Oral temperature was 103, other vitals were unre-
markable. Labs were remarkable for anemia and elevated liver function
tests. A chest roentgenogram revealed a small left pleural effusion and
mediastinal collection. He was placed in respiratory isolation. Sputa were
negative for AFB x 3. A body CT revealed multiple cystic collections along
the left pleura from the apex to the diaphragm, anterior mediastinum,
abdomen, and pelvis. Empiric treatment was started for echinococcus and
bacterial superinfection with albendazole, ciprofloxacin, and metronizad-
ole. Serum echinococcus Ab was negative. A 6cm x 6cm parahepatic cyst
was drained by CVIR. The cultures grew Streptococcus intermedius/
milleri. Ampicillin-sulbactam was started, but fever persisted. Endocardi-
tis was suspect, but there was no evidence of vegetation on echocardio-
gram. An exploratory laporoscopy was then required which revealed liver
cystic structures, intraabdominal adhesions, and plaques along all perito-
neal surfaces. The largest of the cystic structures was excised and sent for
frozen section. A necrotizing granuloma was identified. AFB smears were
negative. Because of a high index of suspicion, treatment with isoniazid,
rifampin, ethambutol, pyrazinamide, and B6 was started. CVIR drained
the pleural fluid, also AFB smear negative. Eventually, AFB cultures of
the peritoneal biopsy and pleural fluid grew Mycobacterium tuberculosis.
An immunocompromised state was suspect. HIV test was negative. After
two weeks of treatment, the patient was discharged on antituberculosis
treatment and amoxicillin-clavulanate.
DISCUSSION: Before the recognition of HIV, more than 80% of all
cases of M. tuberculosis were limited to the lungs. Up to 2/3 of HIV
infected patients with tuberculosis have both pulmonary and extrapulmo-
nary or extrapulmonary tuberculosis alone. Extrapulmonary tuberculosis
presents more of a diagnostic challenge than pulmonary tuberculosis,
especially in those that do not carry a diagnosis of HIV. The presenting
symptoms and signs are nonspecific: fever, weight loss, night sweats,
anorexia, and weakness. Those patients with the highest risk include: HIV
infected, foreign born, household contacts of the infected, low income
populations, homeless, alcoholics, injection drug users, residents of
nursing homes or prisons, and those with medical conditions that
predispose to immunosuppression. The patients that offer no medical
history may go undiagnosed. In adults, infections may be due to new
infection, reactivation of previously latent infection, or very rarely rein-
fection. Common sites of reactivation are the apices of the lungs,
vertebrae, and kidneys. In order of frequency, the extrapulmonary sites
commonly involved are the lymph nodes, pleura, bones, joints, meninges,
and peritoneum. These sites require invasive procedures for diagnosis.
The specimens should be sent for AFB smear, mycobacterial culture, and
histology. The main histologic finding is the necrotizing granuloma. M.
tuberculosis is slow growing and may require 4 to 8 weeks for growth, as
in this case. An intermediate strength PPD test is a helpful adjunct for
diagnosing exposure to or infection with M. tuberculosis. However, it may
be falsely negative in those with active disease and overwhelming
infection. When considering extrapulmonary tuberculosis, clinicians must
maintain a high index of suspicion to pursue the diagnosis.
CONCLUSION: Extrapulmonary findings and persistent fever should
prompt a continued search for M. tuberculosis, especially in a patient
coming from an area where M.tuberculosis is prevalent.
DISCLOSURE: E.U. Bollenbacher, None.
MILIARY TUBERCULOSIS WITH AN UNUSUAL PRESENTA-
TION AND DELAYED DIAGNOSIS
Eiad A. Kseibi, MD*; Peter P. Pace, MD, FCCP; Alan D. Haber, MD,
FCCP. Graduate Hospital, Philadelphia, PA
INTRODUCTION: Miliary tuberculosis (TB) occurs following sys-
temic dissemination of the Mycobacterium tubercle (M.Tb). Though
common worldwide, it is infrequently seen in the United States. Delays in
diagnosis contribute to poor outcome. We present a case of miliary TB in
which an unusual presentation and empiric community-acquired pneu-
monia (CAP) therapy contributed to a late diagnosis and fatal result.
CASE PRESENTATION: An 83-year old female presented with 5
weeks of dry cough, dyspnea, weakness and weight loss. There was no
history for immune compromise or tuberculosis exposure. The physical
examination revealed an afebrile, tachypneic woman with diffuse inspira-
tory crackles, without jugular venous distension or peripheral edema.
Laboratory studies confirmed severe hypoxemia (PaO
2
/FiO
2
ratio 169),
and a transaminitis. Chest radiograph (CXR) revealed bilateral symmetric
infiltrates, consistent with acute respiratory distress syndrome (ARDS).
Levofloxacin was started for presumed CAP. An echocardiogram demon-
strated normal cardiac function. The patient clinically and radiographically
improved except for a lingering reticulonodular infiltrate. All routine
cultures were negative, but an angiotensin converting enzyme level was
elevated. Fiberoptic bronchoscopy was performed 7 days into the hospital
antibiotic course. Transbronchial biopsies revealed non-caseating granu-
lomas, with negative acid-fast bacillus (AFB) stains of lung tissue and
bronchoscopy wash. Prednisone was started for presumed sarcoidosis, and
she was discharged with outpatient follow-up. Four weeks later she
returned with confusion and fever. CXR was unchanged. A lumbar
puncture revealed a cerebrospinal fluid (CSF) pleocytosis (total white
blood cell count 476 cells/mm
2
, 48% lymphocytes), elevated protein (617
mg/dl) and depressed glucose (18 mg/dl). The CSF gram stain, AFB
smear, fungal stain, bacterial antigens, fungal and bacterial cultures were
all negative. Antituberculous medications were started empirically, but the
patient died four days later. CSF acid-fast cultures ultimately revealed M.
Tb growth.
DISCUSSION: Worldwide, tuberculosis occurs frequently in its miliary
form and is among the most common causes of death by infectious disease.
In the United States however, the incidence of miliary TB is low, resulting in
a reduced vigilance for this disease. Unlike cavitary tuberculosis, miliary
disease is less easily diagnosed by sputum analysis, and carries a broader
differential diagnosis including sarcoidosis, hypersensitivity pneumonitis, and
other granulomatous infections. This case highlights several difficulties in
establishing an accurate diagnosis. First the patient presented with ARDS. In
developing nations miliary TB is a reported trigger for ARDS, possibly from
activation of an inflammatory cascade due to hematogenous dissemination of
the organism. In the United States however, TB is rarely encountered as a
cause of acute lung injury. That the patient improved early in the hospital
course probably reflects a partial response to levofloxacin. Fluoroquinolones
have proven activity against M. Tb. However the expanding use of fluoro-
quinolones as empiric treatment for CAP may limit the diagnostic yield of
acid-fast cultures. Though respiratory cultures are positive in miliary TB in 55
- 75 % of cases, this patient’s bronchoscopy culture was negative, perhaps due
to levofloxacin pretreatment. Finally, unlike cavitary tuberculosis the granu-
lomas of miliary disease are more frequently smear-negative and non-
caseating. In such instances, PCR amplification on formalin-fixed tissue with
subsequent DNA probe-hybridization has recently been shown to provide an
accurate early diagnosis of tuberculosis, and may have proved useful in this
case.
CONCLUSION: This case represents an unusual presentation of
miliary TB with ARDS, and a misleadingly negative AFB culture possibly
due to treatment with levofloxacin. Given their proven activity against M.
Tb. and their wide usage as empiric CAP treatment, fluoroquinolones may
delay the diagnosis of tuberculosis. In cases where fluoroquinolones have
been given, negative respiratory cultures do not exclude TB, and further
diagnostic work-up such as PCR amplification may be warranted.
DISCLOSURE: E.A. Kseibi, None.
Wednesday, October 29, 2003
Tuberculosis, continued
334S CHEST 2003—Case Reports