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A rare manifestation of Behçet’s Disease: Extracranial carotid
aneurysm
Hamit Serdar BASBUG, Yalcin GUNERHAN, Hakan GOCER, Kanat OZISIK
Department of Cardiovascular Surgery, Kafkas University Faculty of Medicine, Kars, Turkey
Behçet’s disease is a rare systemic immune vasculitis that often
presents with recurrent oral and genital mucous membrane ulcers and
ocular manifestations. It was first described by a Turkish dermatologist
Hulusi Behçet in 1937.(1) The etiology is thought to be the
autoimmunity that is triggered by a bacterial or viral infection or
another environmental factor.(2) Venous Thrombophlebitis is the most
common vascular manifestation, followed by an arterial aneurysm and
obstruction.(3) Aneurysms mostly occur in the abdominal aorta and
pulmonary arteries.(4) According to the recent literature, less than 50
cases of extracranial carotid aneurysms have been reported.(3) This
article contains the detailed vascular images of a carotid aneurysm in a
young male with Behçet’s disease.
Figure 1. Morphologic view of the patient.
Figure 2. Color Doppler ultrasound view is
showing the true carotid aneurysm (top) and
the diameters (bottom).
20-year-old male was admitted to the outpatient clinic with the
complaint of a mass on the left side of his neck. According to the
anamnesis, the aneurysm was initially appeared one year ago and
presented a slow growth after that. He had a four-year history of
relapsing oral and genital ulcers until he was diagnosed as Behçet’s
Disease two years ago. He has impaired vision in his left eye with a
minimal sense of light in his right eye. Physical examination revealed
a pulsatile mass on the left side of his neck (Figure 1). Color Doppler
ultrasound examination revealed an aneurysm of the left carotid artery
with dimensions of 27,5 mm X 20,3 mm (Figure 2). Transverse slice
of the Computerized Tomography (CT) angiography demonstrated an
aneurysm at the level of the left carotid bifurcation (Figure 3).
Figure 3. Computerized
Tomography (CT) angiography
transverse slice is showing the
carotid aneurysm.
The three-dimensional reconstruction of the CT angiography views
showed a fusiform-shaped aneurysm and its relations with the skeletal
system. (Figure 4). The three-dimensional CT angiography view after
removing the background bony structures revealed a saccular
aneurysm versus normal anatomic vasculature on the opposite side of
the neck (Figure 5). The patient was prescribed prednisone (80
mg/day), azathioprine (150 mg/day), colchicine (1 mg/day),
acetylsalicylic acid (150 mg/day) and discharged upon his rejection for
further surgical or endovascular treatment.
Figure 4. CT angiography (three-dimensional reconstruction) is showing the relationship between the carotid
aneurysm and the skeletal system (anterior and left lateral view).
Although the extracranial carotid artery involvement is a rare
manifestation of the Behçet Disease, surgical treatment is challenging
due to the frequent postoperative life-threatening complications and
the high percentage of recurrences.(5) Nevertheless, an endovascular
intervention or a surgical correction is mandatory for these patients as
they poorly response to the medical treatment.(6) However, the risk
for stent-graft restenosis due to persistent thrombophlebitis after
endovascular therapy as well as the risk for pseudoaneurysm after
surgery should always be kept in mind.(2,3)
Figure 5. CT angiography (three-dimensional vascular
extraction) showing the comparison between the two sides.
References
1. Behcet H. Uber rezidivierende aphthose durch ein Virus verursachte Geschwure am Mund, am Auge und an den Genitalien. Dermatol Wochenschr 1937;105:1152-7.
2. Hosaka A, Miyata T, Shigematsu H, Shigematsu K, Okamoto H, Ishii S, et al. Long-term outcome after surgical treatment of arterial lesions in Behcet disease. J Vasc Surg 2005;42(1):116-21.
3. Berard X, Corpataux JM, Taoufiq H, Sassoust G, Brizzi V, Midy D. (). Don't trust a vein graft to treat carotid aneurysm in patients with Behçet disease. J Vasc Surg 2010;52(2):471-4.
4. Alpagut U, Ugurlucan M, Dayıoglu E. Major arterial involvement and review of Behcet's disease. Ann Vasc Surg 2007;21(2):232-9.
5. Kalko Y, Basaran M, Aydin U, Kafa U, Basaranoglu G, Yasar T. The surgical treatment of arterial aneurysms in Behçet disease: a report of 16 patients. J Vasc Surg 2005;42(4):673-7.
6. Bouarhroum A, Sedki N, Bouziane Z, El Mahi O, El Idrissi R, Lahlou Z, et al. Extracranial carotid aneurysm in Behcet disease: report of two new cases. J Vasc Surg 2006;43(3):627-30.