Infected cystic hygroma resulting in septic shock and respiratory failure: A case report

Abstract

Macrocystic lymphatic malformations (cystic hygromas) are a common cause of cystic neck lesions. These lesions are often diagnosed prenatally in children. In cases without airway compromise, these children are discharged from the hospital for elective treatment. Surgical excision is one treatment modality while sclerotherapy has recently shown adequate results as well. While infection is a relatively common problem for lymphatic malformations, the majority can be treated with antibiotics alone. We present a case in which septic shock and respiratory failure resulted from primary infection of a macrocystic lymphatic malformation in a term infant discharged with a lymphatic malformation of the neck. Urgent surgical drainage failed, and complete excision was ultimately required for source control due to numerous small multiloculated small cysts inaccessible via incision and drainage.

Full text

Infected cystic hygroma resulting in septic shock and respiratory
failure: A case report
Maria E. Linnaus
a
,
b
, David M. Notrica
a
,
b
,
*
a
Department of Surgery and Level I Pediatric Trauma Center, Phoenix Children’s Hospital, 1919 E Thomas Ave, Phoenix, AZ 85016, USA
b
Department of Surgery, Mayo Clinic Hospital, 5777 E Mayo Blvd, Phoenix, AZ 85054, USA
article info
Article history:
Received 17 February 2016
Received in revised form
31 March 2016
Accepted 9 April 2016
Key words:
Lymphatic malformation
Cystic hygroma
Septic shock
Neonate
abstract
Macrocystic lymphatic malformations (cystic hygromas) are a common cause of cystic neck lesions.
These lesions are often diagnosed prenatally in children. In cases without airway compromise, these
children are discharged from the hospital for elective treatment. Surgical excision is one treatment
modality while sclerotherapy has recently shown adequate results as well. While infection is a relatively
common problem for lymphatic malformations, the majority can be treated with antibiotics alone. We
present a case in which septic shock and respiratory failure resulted from primary infection of a mac-
rocystic lymphatic malformation in a term infant discharged with a lymphatic malformation of the neck.
Urgent surgical drainage failed, and complete excision was ultimately required for source control due to
numerous small multiloculated small cysts inaccessible via incision and drainage.
Ó2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Macrocystic lymphatic malformations (formerly termed “cystic
hygromas”) occur in nearly 1:2000 live-born children and are most
commonly located in the head and neck region [1e3]. With im-
provements in ultrasound imaging, these lymphatic malformations
are often seen as early as the first trimester [4]. Surgical excision is
generally regarded as definitive treatment, however, sclerotherapy
has been advocated for uncomplicated macrocystic lymphatic
malformations without evidence of airway compromise [5,6].
Several novel therapies have recently been proposed including use
of sildenafil, propranolol, and sirolimus and have shown varying
success in small case reports and case series [7e9]. Reports of
spontaneous regression of lymphatic malformations have also been
reported although rare [5]. A relatively common complication of
macrocystic lymphatic malformation is infection which may often
be treated successfully with antibiotics alone followed by elective
resection or sclerotherapy. We present a case of a large cervical
lymphatic malformation in a neonate who developed septic shock
and respiratory failure prior to treatment. The child also failed
conservative attempts at drainage, and urgent complete surgical
excision was required for source control. The operative findings
explaining failure of medical treatment and suggestions for defin-
itive management of severe infection in this situation are reviewed.
1. Case report
A 6-day-old male born at term with history of intrauterine
methamphetamine exposure and prenatally diagnosed cervical
macrocystic lymphatic malformation presented to the emergency
department (ED) with poor feeding and an increased work of
breathing associated with enlargement and discoloration of the
lymphangioma. Examination revealed sternal retractions, tachyp-
nea, hypoxia on room air, tachycardia, and a large 10 10 cm cystic
mass on the right neck with new, small areas of erythema and
ecchymosis (Fig. 1). Laboratory testing revealed severe leukopenia
and lymphocytopenia, indirect hyperbilirubinemia, and elevated
coagulation tests (PT, PTT); urinalysis was negative for infection.
Chest X-ray (CXR) performed was without evidence of tracheal
deviation and compression. Sepsis workup commenced and the
child was admitted to the Neonatal Intensive Care Unit (NICU) for
worsening respiratory distress and placed on empiric ampicillin
and gentamicin. The patient’s lymphangioma remained soft and the
patient never developed any signs of tracheal compromise or car-
bon dioxide retention. Endotracheal intubation was performed for
worsening respiratory distress without difficulty and an adequate
air leak around the endotracheal tube suggested the airway was not
compromised. Clinically, the respiratory distress, hemodynamic
Funding: N/A.
*Corresponding author. Department of Surgery, Phoenix Children’s Hospital,
1919 E Thomas Ave, Phoenix, AZ 85016, USA.
E-mail address: dnotrica@phoenixchildrens.com (D.M. Notrica).
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS
journal homepage: www.jpscasereports.com
2213-5766/Ó2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
http://dx.doi.org/10.1016/j.epsc.2016.04.005
J Ped Surg Case Reports 9 (2016) 19e22

compromise and rapid decline appeared to be from overwhelming
sepsis. Over the next few hours oxygenation became more difficult
and high-frequency oscillator ventilation was required. Blood cul-
tures became positive at 5 h for gram-negative bacteremia which
ultimately speciated into Escherichia coli resistant to ampicillin and
fluoroquinolones. Surgical consultation was obtained on the
morning of admission for the inflamed and necrotic-appearing neck
lymphangioma (Fig. 2). Urgent bedside needle decompression was
performed immediately expressing purulent fluid.
The patient continued to decline in status throughout that day
and the decision was made to perform bedside incision and
drainage with placement of a Penrose drain (Fig. 3). Care was made
to break up the septations in the wound at the time of drainage.
The patient continued intravenous (IV) antibiotic therapy for
three more days but necrosis of the wound progressed and the
patient again decompensated over 24 h requiring increased vaso-
pressor and ventilatory support. Due to the patient’s critical insta-
bility secondary to sepsis from the infected lymphatic
malformation, and failure of more conservative attempts, it was felt
that the only option for source control was complete surgical
excision. The necrotic wound was excised with electrocautery and
the spinal accessory nerve was identified and isolated. Several very
small, pus-filled lymphatic cysts had remained which contained
frank pus, and partial necrosis of the lymphangioma was noted to
extend to the base of the malformation (Fig. 4). Complete excision
was performed and the margins were locally advanced to provide a
loose, but adequate wound closure over a Penrose drain.
After the procedure, the child showed rapid improvement over
the next several hours and was weaned off vasopressors and to
conventional mechanical ventilation. The drain was removed after 2
days and the wound was loosely packed with calcium alginate. On
postoperative day three, the patient was extubated after remaining
a day on minimal ventilator settings and exhibiting a clinical air
leak. He remained on intravenous cefotaxime for a total of fourteen
days after postoperative cultures and sensitivities were obtained
and was ultimately discharged on postoperative day 15. Upon re-
turn to the surgical clinic, he was noted to have torticollis which
resolved after several weeks of physicaltherapy and had a favorable
cosmetic result (Fig. 5) without evidence of a winged scapula or any
nerve damage.
2. Discussion
To our knowledge, this is the first reported case of septic shock
and respiratory failure resulting from primary infection of a cervical
lymphatic malformation. Conservative management of the infec-
tion with systemic antibiotics in this case was initially attempted
but did not result in improvement. Neither needle decompression
nor incision and drainage of the infected cyst were adequate to
stem the clinical deterioration, and the patient progressed to
Fig. 1. The child’s macrocystic lymphangioma at the time of admission showing new
onset erythema and areas of ecchymosis.
Fig. 2. The infant has been intubated for respiratory failure. The lymphangioma has
enlarged and shows bullae and areas of necrosis.
Fig. 3. The infant has developed systemic sepsis and skin necrosis, despite incision and
drainage with care to break up septations.
M.E. Linnaus, D.M. Notrica / J Ped Surg Case Reports 9 (2016) 19e2220

wound necrosis. The persistent small purulent loculations resulting
from the multicystic lesion led to inadequate source control with
simple incision and drainage. Formal surgical exploration and
complete excision were ultimately required to obtain source control
in this patient.
Infections of macrocystic lymphatic malformations occur in
7e30% of cases in the literature and are slightly more common in
cervical lesions [10,11]. The majority of these infections and minor
inflammation can be treated with antibiotics alone [2]. In this case,
despite being a predominantly macrocystic lymphatic malforma-
tion, numerous small, loculated pus-filled cysts deep within the
neck were identified, thereby suggesting the reason intravenous
antibiotics alone were inadequate.
Other reports have noted sepsis from spontaneous infection of
these lymphatic malformations [11], but no studies reported pro-
gression to septic shock. Historically, infection was a contraindica-
tion to surgical excision and some advocated waiting to treat
surgically for 3 months after the infection had resolved [3]. More
recently, surgeons have advocated early definitive treatment of
lymphatic malformation when functional compromise is present
[12]. This approach may also help avoid sequelae of infection and
enlargement causing airway compression.
The pathogenesis of lymphatic malformations may include
aberrant venous lymphatic connections or abnormal lymphatic
development leading to stasis and enlargement of the lymphatic
malformation [13]. This child’s malformation was located on the
posterior right neck where they most commonly occur on the left.
Left-sided predominance is hypothesized to be due to the thoracic
duct entering the subclavian on the left side [14,15]. Veno-
lymphatic communications allow for bacteria to transport easier
into the circulation thus promoting a sepsis-like inflammatory
response. In this patient’s case, E. coli, a gram-negative bacillus and
producer of endotoxin, was responsible for the severe inflammatory
response and development of shock. Source control was only ob-
tained after radical skin debridement and complete excision of the
lymphatic malformation.
Airway compression from lymphatic malformations of the neck
has been the most serious reported complication previously [16,17],
but it is present in fewer than 10% of reported cases [2]. For this
patient, no airway compromise was noted based on physical exam-
ination findings and CXR imaging, and intubation was required only
for the associated respiratory failure. Computed tomography (CT) or
Magnetic Resonance Imaging (MRI) were not performed at the time
of decompensation given the patient’s instability and the belief that
drainage alone would be adequate. Once determined to be inade-
quate, the child was too unstable for transport. After definitive
resection of the lesion, the patient recovered quickly and was
extubated shortly thereafter and had an overall favorable outcome.
3. Conclusion
Newborns with lymphatic malformations of the neck may rarely
develop severe sepsis or septic shock refractory to intravenous
antibiotics or drainage alone. In the setting of septic shock from an
infected lymphatic malformation refractory to intravenous antibi-
otics and drainage, strong consideration should be given to an ur-
gent complete excision of the infected lesion for source control. A
high level of suspicion should be maintained by the treating sur-
geon that multiple smaller infected cysts may persist despite inci-
sion and drainage with breaking up septations.
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