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Bahar KAYAHAN, MD; Nilda SÜSLÜ, MD; Cavid CABBARZADE, MD; Gamze ATAY, MD; Gözde YAZICI, MD; Ali
Şefik HOŞAL, MD
Carotid Blowout Syndrome During Radiotherapy: A Case Presentation and Review of A Rare Entity
KBB-Forum
2013;12(3)
www.KBB-Forum.net
CASE REPORT
CAROTID BLOWOUT SYNDROME DURING RADIOTHERAPY: A CASE
PRESENTATION AND REVIEW OF A RARE ENTITY
Bahar KAYAHAN, MD1; Nilda SÜSLÜ, MD1; Cavid CABBARZADE, MD1; Gamze ATAY, MD1;
Gözde YAZICI, MD2; Ali Şefik HOŞAL, MD1
1Hacettepe, Kulak Burun Boğaz ve Baş Boyun Cerrahisi, Ankara, Türkiye 2Hacettepe, Radyasyon Onkolojisi, Ankara, Türkiye
SUMMARY
Carotid blowout syndrome (CBS) is an uncommon but a potentially lethal complication with 40% mortality and 60% morbidity,
encountered in head and neck cancer patients. CBS has three subtypes; threatened, impending, acute. Impending form is characterized by
spontaneously resolving recurrent epistaxis attacs. Radical neck dissection, pharyngocutaneous fistulae, recurrent/ residual tumor and
irradiation have been determined as potential risk factors of CBS. Nasopharyngeal carcinoma is highly radiosensitive and its primary
treatment is radiotherapy. We report a 77-year-old male patient with T4N2cM0 nasopharyngeal cancer with impending form of CBS
diagnosed during the initial days of radiation.
Keywords: Nasopharyngeal cancer, Radiotherapy
RADYOTERAPİ SIRASINDA ‘‘CAROTİD BLOWOUT'' SENDROMU: OLGU SUNUMU VE BU NADİR DURUMUN
GÖZDEN GEÇİRİLMESİ
ÖZET
‘‘Carotid blowout'' sendromu (CBS), baş boyun kanserli hastalarda görülen, sık rastlanmayan ancak %40 mortalite ve %60 morbidite
oranları ile yüksek riske sahip bir komplikasyondur. CBS üç alt gruptan oluşmaktadır; akut, subakut-yaklaşan ve yavaş-gizli ilerleyen.
Subakut-yaklaşan tip CBS'de tekrarlayan ve kendiliğinden duran diffüz kanamalar görülmektedir. Radikal boyun diseksiyonu,
faringokutanöz fistül, nüks / rezidü tümör varlığı ve radyoterapi hikayesi CBS için potansiyel risk faktörleridir. Nazofarinks kanserinin
oldukça radyosensitif olması nedeniyle primer tedavisi radyoterapidir. Bu yazıda T4N2cM0 evreli nazofarinks kanseri olan, primer
radyoteraipinin erken günlerinde subakut - yaklaşan tip CBS gelişerek exitus olan 77 yaşında bir erkek hasta sunulmuştur.
Anahtar Sözcükler: Nazofarenks kanseri, Radyoterapi
INTRODUCTION
Carotid blowout syndrome (CBS) is a life-
threatening condition which is defined as rupture or
exposure of carotid artery.1,2 It is a potentially lethal
complication in patients with head and neck cancer.
CBS has an average mortality rate of 40 % and
morbidity rate of 60%.3,4 Radical neck dissection,
pharyngocutaneous fistulae, irradiation, recurrent or
residual tumor have been implicated as potential
causes of CBS.3-6 Nasopharyngeal cancer is a
radiosensitive tumor and the primary treatment is
radiotherapy with or without chemotherapy
depending on the stage.5,7 Although radiation induced
CBS with pseudoaneurysm formation is an
uncommon complication in nasopharyngeal cancer,
several reports had declared cases with
pseudoaneurysm of petrous segment of internal
carotid artery.1,5,7-10
Corresponding Author: Cavid Cabbarzade MD; Hacettepe,
Kulak Burun Boğaz ve Baş Boyun Cerrahisi, Ankara, Türkiye, E-mail:
drcavidcab@yahoo.com
Received: 17 June 2013, revised for: 26 July 2013, accepted
for publication: 28 July 2013
In this paper, an adult patient who had been on
radiotherapy due to nasopharyngeal cancer and
developed CBS during initialdays of treatement is
presented and literature regarded CBS is reviewed.
CASE PRESENTATION
A 77-year old male patient admitted to our clinic with
bilateral neck masses. Otolaryngological examination
revealed an ulcerative mass on postero-superior wall
of nasopharynx, several conglomerated
lymphadenopathies on bilateral level II and V and
right vocal cord paralysis. From his past medical
history, it was learned that he had an ischemic
cerebrovascular attack twenty years ago and he has
diabetes mellitus type II. Magnetic Resonance
Imaging revealed a mass in nasopharynx which was
invading cavernous sinus and prevertebral muscles;
and there were metastatic cervical
lymphadenopathies in level II-III-V bilaterally.
Histopathological diagnosis of the nasopharyngeal
mass was non-keratinized undifferentiated
nasopharyngeal cancer. Patient was diagnosed as
T4N2cM0 nasopharyngeal cancer, and concomittant
chemoradiotherapy was planned. Seventy Gy with a
104
Bahar KAYAHAN, MD; Nilda SÜSLÜ, MD; Cavid CABBARZADE, MD; Gamze ATAY, MD; Gözde YAZICI, MD; Ali
Şefik HOŞAL, MD
Carotid Blowout Syndrome During Radiotherapy: A Case Presentation and Review of A Rare Entity
KBB-Forum
2013;12(3)
www.KBB-Forum.net
fraction dose of 2 Gy to gross tumor volume, 66 Gy
to whole nasopharynx and positive lymph nodes was
planned with intensity modulated radiotherapy
technique. Cisplatinium, with a dose of 35 mg/m2/
week, was planned concomitantly with radiotherapy.
On the 18th day of radiotherapy, patient admitted to
the emergency clinic with epistaxis. He stated that he
had several episodes of bleeding all of which
resolved spontaneously during the last three days. In
the emergency clinic, hemoglobin level was detected
to be 8.9 mg/dl. On otolaryngological examination an
oozing type epistaxis originating from the
nasopharyngeal mass was noticed. The bleeding was
controlled with simple transnasal packing. He was
hospitalized and two unites of red blood cells were
transfused. After the patient was stabilized in
hemodynamic means, an angiography with
computerized tomography was performed with
suspicion of pseudoaneurysm on internal carotid
artery wall. It was detected that a 9 mm left internal
carotid artery pseudoaneurysm was present with
certain amount of extravasation (Figure 1, 2). The
patient was consulted with interventional
neuroradiology and stenting of the internal carotid
artery segment with pseudoaneurysm was planned.
Just after the radiological imaging was performed;
patient had a profuse epistaxis and he deteriorated
rapidly. He had a filliform pulse with remarkable
hypotension. He was intubated and resuscitation was
started immediately. He was given proper fluid
support intravenously and atropine, adrenaline,
bicarbonate by close monitoring. Bleeding continued
during resuscitation and at the end of an hour patient
was accepted as exitus.
DISCUSSION
Carotid blowout syndrome might be
observed due to head and neck tumor itself or the
treatment modality. Not only a part of carotid artery
could be infiltrated by tumor or its neck metastasis;
but also it could be nuded or damaged by treatment
modalities such as neck dissection or irradiation.4,11
In the treatment of nasopharyngeal cancer,
radiotherapy is being administered to the primary site
and neck bilaterally. Large arteries are relatively
resistant to radiotherapy however stenosis may be
observed even in large vessels following
radiotherapy. This side effect is a delayed
complication of radiotherapy and is observed years
after treatement. Carotid blowout syndrome is a rare
complication of radiotherapy and usually occurs after
re-irradiation. In a review which was published
recently, the rate of CBS was 2.6% for patients
receiving salvage head and neck reirradiation.12 The
median time to CBS, which was measured from the
begining of reirradiation was 7.5 months. In this
review, patients receiving reirradiation with
stereotactic body radiotherapy (SBRT) were
excluded. The rate of CBS in patients receiving
salvage radiotherapy with SBRT is reported to be in
the range of 10% to 17%.13,14 It is observed during 5
to 24 months following SBRT.
Figure 1: Pseudoaneurysm formation on left internal
carotid artery, petrocavernous junction (Computed
Tomography Angiography).
Figure 2: Pseudoaneurysm formation on left internal
carotid artery, petrocavernous junction (Computed
Tomography Angiography).
105
Bahar KAYAHAN, MD; Nilda SÜSLÜ, MD; Cavid CABBARZADE, MD; Gamze ATAY, MD; Gözde YAZICI, MD; Ali
Şefik HOŞAL, MD
Carotid Blowout Syndrome During Radiotherapy: A Case Presentation and Review of A Rare Entity
KBB-Forum
2013;12(3)
www.KBB-Forum.net
Radiotherapy is involved in pathophysiology
of CBS as it can lead to vascular pathologies such as
obliteration of vaso vasorum, adventitial fibrosis,
atherosclerosis, weakening of arterial wall,
subendothelial vacoulization, fragmentation of tunica
media elastic fibers of the carotid artery.6,7,10
Irradiation may also cause osteonecrosis of petrosal
bone which is critical in formation of
pseudoaneurysm at petrosal segment of carotid artery
which can trigger CBS.7,8 Recurrent cases require
booster doses of radiotherapy which makes carotid
artery, particularly the petrous segment, more
vulnerable to pseudoaneurysm formation and further
bleeding.5,7 Our case is unusual, as the damage to the
large vessel was seen during the first line
radiotherapy, whereas in literature CBS is usually
reported with reirradiation or after the conclusion of
therapy.1,2,5,8
Neck dissection is known as another
etiologic factor for CBS. Postoperative complications
after neck dissection, such as flap necrosis, wound
infection, pharyngocutaneous fistula formation may
cause arterial wall damage and injuries of carotid
artery.5,6,15
Carotid blowout syndrome is classified into
three subtypes; threatened, impending and acute.
Threatened CBS is defined as exposure of the carotid
artery due to wound breakdown or neoplastic
invasion of carotid system although hemorrhage has
not occured yet. If the carotid artery is not promptly
covered with healthy tissue, the rupture is inevitable.
Impending CBS is defined as short episodes of
bleeding which can resolve spontaneously or can be
controlled by simple packing. However complete
rupture is certain; only the time is unknown. Acute
CBS is defined as profuse bleeding which cannot be
controlled by simple packing. As there is a complete
and sudden rupture of the carotid artery, patient
deteriorates rapidly. Unless the vessel repair is
provided immediately, resuscitation would not help
for long term stabilization.1,4,8,16 Regarding the
spontaneous recovering recurrent epistxis attacks, our
case should be considered as an impending type of
CBS with pseudoaneurysm formation.
Management of CBS should depend on some
principles; the initial and cardinal priority is to
provide both respiratory and cardiac stabilization.
The next step should be identification of the site and
extent of the pathology via diagnostic angiography. 4
In case of carotid system involvement (internal or
common); temporary balloon occlusion test (BTO)
should be performed to determine intracranial
collateral circulation. The purpose of BTO is to
assess the availability of collateral circulation if the
ruptured carotid artery is permanently occluded.2,4
Balloon occlusion test is a decision making step not
only for endovascular treatment modalities but also
for the open surgery techniques. Emergent surgical
ligation of carotid system without BTO has average
neurologic morbidity of 60%.17 Prior to the neck
dissection procedure or irradiation of an extended
tumor invading carotid system; result of the BTO
may help to decide whether to ligate the vessel or to
construct a bypass.6 In literature; endovascular
modalities are more preferrable compared to surgical
management of CBS. Surgical management is usually
difficult as the exploration and repair of previously
irradiated fields is annoying.2,6 On the other hand,
endovascular techniques are less invasive. They can
be performed with local anesthesia and do not
necessitate any tissue harvesting.3,4,6,16
Based on the BTO result there are two main
types of endovascular treatment; de-constructive
versus constructive. Deconstructive management is
designed for the patients who can tolerate BTO and
are suitable for the permanent occlusion of carotid
artery by coil or glue embolization. Constructive
management is designed for the patients who can not
tolerate BTO therefore for patent and safe arterial
flow is provided by endovascular stents.2,4,11,15
Despite the BTO results, there are certain reports in
the literature stating cases who had passed the test
however developed ischemic complications including
cerebral infarction due to deconstructive
embolization procedure. The reason is thought to be
that BTO is a short timed- test (20-30 minutes) and
sometimes collateral circulation insufficency may be
seen in longer period.8
In case of nasopharyngeal cancer which is
irradiated, CBS is seen as a result of pseudoaneurysm
formation mainly in medium to large sized vessels on
a basis of atherosclerosis.7,9,10 Our patient had history
of ischemic cerebrovascular attack which gives
suspicion of tendency to thrombosis in a retrospective
fashion. As sson as pseudoaneurysm is detected on
angiography both deconstructive and constructive
endovascular modalities can be considered, however
each has its own limitations. Pseudoaneurysm is an
arterial bulging which is not baring normal vessel
wall structure. Besides surrounding bone is usually
ostoenecrotic which lacks any support mechanisms.
There can be acute rupture of pseudoaneurysm during
inflation of BTO balloon or stent replacement.11 Also
after embolization of pseudoaneurysm extravasation
of coils has been seen in some cases.7 To find a
solution; coil embolization with stent placement has
been worked on, but is been still controversial.8,18
106
Bahar KAYAHAN, MD; Nilda SÜSLÜ, MD; Cavid CABBARZADE, MD; Gamze ATAY, MD; Gözde YAZICI, MD; Ali
Şefik HOŞAL, MD
Carotid Blowout Syndrome During Radiotherapy: A Case Presentation and Review of A Rare Entity
KBB-Forum
2013;12(3)
www.KBB-Forum.net
Following endovascular procedures, there
might be recurrent hemorrhages which are found to
be correlated either with infection or anticoagulant
therapy. Therefore appropiate antibiotic treatment
should be administered after both deconctructive and
constructive procedures.2,15 Anticoagulant therapy is
generally started just before stent placement and
continued lifelong but much more intensive in the
first week. As a precaution, bleeding parameters
should be followed intimately, unnecessary
medication should be avoided when the optimum
level is reached.2,16
Carotid blowout syndrome might cause lethal
bleeding and it is better to realize risk factors in the
earlier period and to intervene if possible. It has been
noticed that focal soft tissue necrosis on Magnetic
Resonance Imaging of patients with nasopharyngeal
cancer is a predictor of CBS.9 Also both the
radiologist and the head neck cancer team (surgeon,
oncologist and radiation oncologist) should be alert
for pseudoaneurysm formation, vessel wall damages
and the degree of adjacent osteonecrosis to predict
any possible form of CBS.1,5
In conclusion; CBS is an uncommon but
lethal complication of head and neck cancer patients.
Not only the profuse hemorrhage, but also minimal
recurrent bleedings should be examined intimately.
Once it occurs, patient should be evaluated
immediately and proper treatment should be
administered quickly. As it is more important to
avoid such a catastrophic complication; predictors on
routine imaging techniques should not be missed and
fundamental precautions should be applied.
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