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Massive carotid artery aneurysm presenting as an oropharyngeal swelling in a young woman

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Abstract

A 28-year-old woman presented to the emergency department with a 5-day history of a sore throat and a pharyngeal swelling that increased in size. The patient was previously investigated for Marfan's syndrome after episodes of bilateral spontaneous pneumothoraces but did not meet the criteria for diagnosis. Examination revealed a large pulsatile lesion in the oropharynx. CT angiography revealed an internal carotid artery aneurysm. The patient underwent a successful urgent open repair of the aneurysm with a primary arterio-arterial anastomosis without a patch. Histopathology of the aneurysmal sac revealed cystic medial degeneration. 2015 BMJ Publishing Group Ltd.
CASE REPORT
Massive carotid artery aneurysm presenting as
an oropharyngeal swelling in a young woman
Kah Hoong Chang, James Cotter, Gerald Thomas McGreal
Department of Surgery, Mercy
University Hospital, Cork,
Ireland
Correspondence to
Dr Kah Hoong Chang,
kahhoong_chang@yahoo.co.uk
Accepted 13 January 2015
To cite: Chang KH,
Cotter J, McGreal GT. BMJ
Case Rep Published online:
[please include Day Month
Year] doi:10.1136/bcr-2014-
206810
SUMMARY
A 28-year-old woman presented to the emergency
department with a 5-day history of a sore throat and a
pharyngeal swelling that increased in size. The patient
was previously investigated for Marfans syndrome after
episodes of bilateral spontaneous pneumothoraces but
did not meet the criteria for diagnosis. Examination
revealed a large pulsatile lesion in the oropharynx.
CT angiography revealed an internal carotid artery
aneurysm. The patient underwent a successful urgent
open repair of the aneurysm with a primary arterio-
arterial anastomosis without a patch. Histopathology of
the aneurysmal sac revealed cystic medial degeneration.
BACKGROUND
Aneurysms of the extracranial carotid arteries
(common carotid artery (CCA), external carotid
artery (ECA) and cervical part of the internal
carotid artery (ICA)) are very rare. The largest
reported series included 141 cases in a 15-year
period.
1
In addition, the diagnosis of such cases is
often missed or delayed due to its rarity, with
potentially fatal consequences. The mortality rate
in non-operated patients with carotid artery aneur-
ysm is reported at 70%.
2
CASE PRESENTATION
A 28-year-old married, nulliparous, product
builder, presented to the emergency department of
a neighbouring hospital with right-sided neck pain
and a right pharyngeal swelling associated with a
sore throat, which had been present for 5 days. She
is a non-smoker. Examination revealed a pulsatile
swelling in the right oropharynx, which was
assumed to be an abscess (gure 1A). Aspiration
was attempted but abandoned due to the patients
strong gag reex. At this point, the possibility that
it was not an abscess was considered and it was felt
prudent to obtain imaging prior to attempting any
further intervention. A CT scan was subsequently
arranged and it showed a 17×13 mm saccular
aneurysm of the right ICA. The patient was subse-
quently transferred to the on call vascular service
in the Mercy University Hospital.
The patient has no history of trauma or intraven-
ous catheterisation of the cervical jugular vein to
have caused a traumatic aneurysm. She has a history
of bilateral spontaneous pneumothoraces (left-sided
in 2007 and right-sided in 2008). She underwent an
elective right-sided limited thoracotomy and apical
Figure 1 (A) Preoperative and (B) postoperative
appearance of the oropharynx. Large pulsatile swelling in
the right oropharynx, which disappeared postoperatively
(arrow).
Chang KH, et al.BMJ Case Rep 2015. doi:10.1136/bcr-2014-206810 1
Rare disease
bullectomy followed by parietal pleurectomy. She subsequently
underwent a left-sided ligation of bullae and pleurectomy. Her
postoperative recovery for both procedures was uneventful. Her
phenotype is ectomorphic, which is the phenotype most typically
associated with Marfans syndrome.
INVESTIGATIONS
Preoperatively, a CT angiogram of the carotid arteries showed a
17×13 mm saccular aneurysm of the right ICA (gure 2A, B).
Postoperatively, the patient had MRI and MR angiography of
the brain, which were unremarkable. MR angiography of the
aorta revealed no aneurysm or dissection of the thoracic and
abdominal aorta.
The patient was previously investigated for Marfans syn-
drome in 2011, due to having presented with bilateral pneu-
mothoraces and her ectomorphic phenotype. She was evaluated
using the Ghent criteria.
3
Spontaneous pneumothorax constitu-
tes one minor criterion. She has one major and one minor skel-
etal feature. Overall, she did not meet the criteria for the
diagnosis of Marfans syndrome.
DIFFERENTIAL DIAGNOSIS
Differential diagnoses of a painful oropharyngeal swelling
include tonsillar abscess, adenoiditis, neoplasm and carotid
artery aneurysm.
TREATMENT
The decision was made to repair the aneurysm urgently in view
of risk of rupture. Under general anaesthetic, a cervical incision
was made parallel and anterior to the sternocleidomastoid
muscle and centred over the carotid bifurcation. The incision
was carried down through the platysma, and the
Figure 2 (A) Transverse plane; (B) sagittal plane; (C) three-dimensional view of the CT angiogram of the carotid artery. The right ICA aneurysm is
shown protruding into the oropharynx (yellow arrow). CCA, common carotid artery; ICA, internal carotid artery.
2 Chang KH, et al.BMJ Case Rep 2015. doi:10.1136/bcr-2014-206810
Rare disease
sternocleidomastoid muscle was retracted laterally with self-
retaining retractors.
CCA and ECA were grossly normal. The proximal ICA was tor-
tuous and diseased. The remaining ICA appeared to be unaffected.
The patient received 4000 units of unfractionated heparin intrao-
peratively. The aneurysm was embedded into the oropharynx
(gure 3A). This required extremely careful dissection of the ICA.
For this reason, the aneurysmal sac was left in situ and an inci-
sional sample of the sac was sent for histology. Owing to the tortu-
ous nature of the ICA, there was adequate length of ICA proximal
and distal to the aneurysmal sac for primary anastomosis without
theneedforapatch(gure 3B). A spatulated end-to-end anasto-
mosis of the proximal ICA was performed using 6/0 polypropylene
(gure 3C). Excellent backow was observed from the ICA prior
to closure. The patient made an uneventful postoperative recovery
and was discharged on postoperative day 3. The pharyngeal swel-
ling reduced signicantly postoperatively and completely resolved
2 weeks later (gure 1B).
OUTCOME AND FOLLOW-UP
The patient made an uneventful recovery. Further assessments
by a cardiologist and a rheumatologist refuted the diagnosis of
Marfans syndrome.
The histopathological analysis of the aneurysmal sac revealed
focal elastic fragmentation with very focal small pools of Alcian
blue-positive myxoid material, most consistent with evidence of
cystic medial degeneration. Increased brosis in the immediate
adventitial region was noted. No atheromatous change or
inammatory vasculitis was appreciated on the multiple levels
examined.
Radiological imaging of the aorta was recommended to the
patient during any future pregnancy, as pregnancy has unpre-
dictable effects on blood vessels. Cases have been reported on
patients presenting with de novo arterial dissections during
pregnancy due to the effects of progesterone on vascular tone.
4
DISCUSSION
Extracranial carotid artery aneurysms are rare and most commonly
due to artherosclerosis.
56
The literature on isolated carotid artery
aneurysm secondary to cystic medial necrosis is scarce.
78
Because of their rarity, the diagnosis of extracranial carotid
artery aneurysms is often missed or delayed, especially in the
absence of neurological symptoms. Carotid artery aneurysms in
the oropharynx or neck may be mistaken for an inammatory
or neoplastic lesion of a tonsil, peritonsilar abscess or neck
tumour.
910
Carotid artery aneurysms are associated with cata-
strophic complications if untreated. A high index of suspicion,
early recognition and prompt operative intervention is therefore
critical.
Open surgical repair is the treatment of choice in patients
with extracranial carotid aneurysms. Mortality in non-operated
extracranial aneurysms of 71% and a signicant stroke risk of
up to 50% have been reported.
1
The option of endovascular
procedure was considered in this case but deemed unsuitable in
view of several reasons: the extreme tortuosity of the diseased
vessel and the acute presentation with concern of leak or
rupture. In addition, the operator has ample experience with
open carotid operations.
Complete excision of the aneurysm is not recommended as it
is associated with a high incidence of cranial nerve injuries.
11 12
Figure 3 (A) Right neck dissection of the ICA aneurysm; (B) ICA transected proximal and distal to the aneurysmal sac; (C) end-to-end ICA
anastomosis. (A) Dissection of the right neck revealing the arterial anatomy, with the aneurysmal sac deeply embedded in the oropharynx (green
arrow). (B) Grossly normal ICA was transected proximal (green arrow) and distal (black arrow) to the aneurysmal sac. (C) Owing to the tortuosity of
the ICA, there was adequate length of the artery for the two transected ends to be approximated with an end-to-end anastomosis without a patch
(green arrow). CCA, common carotid artery; ICA, internal carotid artery; ECA, external carotid artery.
Learning points
Extracranial carotid artery aneurysm is a rare condition.
A high index of suspicion is required to diagnose this
condition.
Missed or delayed diagnosis and inappropriate management
can lead to potentially life-threatening or fatal
consequences.
Chang KH, et al.BMJ Case Rep 2015. doi:10.1136/bcr-2014-206810 3
Rare disease
In this case, complete excision of the aneurysmal sac was not
performed as it was deeply embedded in the oropharynx.
We present an extremely rare case of an ICA aneurysm pre-
senting as a painful oropharyngeal swelling. This highlights the
importance of clinical suspicion despite its rarity. An attempted
ad hoc intervention would have had life-threatening, if not fatal,
consequences.
Contributors JC and KHC prepared the manuscript. GTM supervised and approved
the nal draft for submission.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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1 Fankhauser GT, Stone WM, Fowl RJ, et al. Surgical and medical management of
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3 Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the
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4 Chang KH, et al.BMJ Case Rep 2015. doi:10.1136/bcr-2014-206810
Rare disease
... [17][18] In the case of minor vascular changes, observation is recommended, whereas major changes which may cause bleeding, airway obstruction or cosmetic defects may require more radical approaches, such as multidisciplinary surgical treatment or radiation therapy. [17][18][19] Conservative treatments include local or general administration of glucocorticoids. According to Bilewicz, the effectiveness of such treatment is about 30%. 10 The operative interventions include the use of CO 2 laser, potassium titanyl phosphate (KTP) laser, cryosurgery, harmonic scalpel, or radiotherapy. ...
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