Eur J Cardiothorac Surg 2001;19:938-939
© 2001 Elsevier Science NL
Carotid artery pseudoaneurysm in Behcet's disease
Ahmet Özyazicio
lu,
Hikmet Koçak,
Ünsal Vural
Department of Cardiovascular Surgery, Medical School of Atatürk University, Erzurum, Turkey
Received 27 December 2000;
received in revised form 2 March 2001;
accepted 16 March 2001.
Corresponding author. Gez mah, Yasemin sok, Atmaca apt, A-Blok No:5, Erzurum, Turkey. Tel.: +90-442-2339345; fax: +90-442-3166340
e-mail: violinahmet{at}hotmail.com
 |
Abstract
|
|---|
We report a case of carotid artery pseudoaneurysm occurring in a patient with Behcet's disease for the purpose of discussing approach to this unusual complication of Behcet's disease.
Key Words: Behcet's disease Pseudoaneurysms Carotid artery
 |
1. Introduction
|
|---|
Behcet's disease is a multisystemic disease characterized by relapsing uveitis and oral and genital ulcerations. Additional manifestations consist of vascular findings in the skin joints and elsewhere. This disease appears most frequently in the Middle and Far East. The most striking vascular findings are multiple central and peripheral arterial aneurysms. Their surgical repair is an extremely hazardous undertaking.
Our patient (a female) at the age of 34 had a history of Behcet's disease. Two years ago, she began to have fever, genital and oral ulcers and lost weight as a result of anorexia. One year later, she developed abdominal pain and she was taken for exploratory laparatomi in a general surgery clinic. At laparatomy, there were both a lot of lenfadenopaty in the abdominal cavity of size 23 cm and also inflammatory intestine. Lenfoid biopsy was performed. The microscopic examination showed a non-specific acute inflammation.
She still continued to feel unwell. She had recurrent thrombophlebitis. Finally, she developed a mass on the right carotid artery trace. Symptoms were related to her mass and compression on adjacent structures. She had a iatrogenic right carotid arterial puncture history. Computerized tomography (CT) demonstrated a partly thrombosed false aneurysm both to the right common and internal carotid artery (Fig. 1). She was dispneic and oxygen dependant. We decided to perform an operation. At surgery the mass was found surrounding the carotid artery and involved the bifurcation. The patient was heparinized. The arteriotomy was performed in the common carotid artery. A balloon-tipped intraluminal carotid shunt was carefully advanced into the lumen of the internal carotid artery. The pseudoaneursym was entered sharply, and the arterial defect was identified and controlled digitally. Arteria carotid interna was fragile and delicate. A carotid internal to common carotid artery by-pass was then performed using 46 mm polytetrafluoroethylene (PTFE) graft.

View larger version (64K):
[in this window]
[in a new window]
|
Fig. 1. Computerized tomography shows shifting of the trachea away from the involved side and a partly thrombosed false aneurysm both to the right common and internal carotid artery.
|
|
Histological examination of the false aneurysm wall showed an obliterative type of arteritis. As the patient had multiple vascular component of Behcet's disease, immunosuppressive therapy began (azothioprine, corticosteroid).
 |
2. Comment
|
|---|
Pseudoaneurysms of the extracranial carotid artery are rare in comparison with pseudoaneurysms in other major arterial segments of the body [1].
For many years, the known vascular complications in Behcet's disease have been attributed to thrombophlebitis. But a considerable number of reports have been published relating to cases with aneurysm and arterial occlusion [2,3]. This disease, most frequently affecting Mediterranean and Asian populations in the third and fourth decades of life, is associated with other systemic findings including cutaneous erythematous nodules, and inflammatory processes of the gastrointestinal system. Our patient, 34 years old, had a history of oral and genital ulceration; two thirds of Behcet's triad. She had further history of cutaneous nodules and episodes of superficial thrombophlebitis. The undertaken laparatomy has been related to the enteritis. Involvement of medium or large arteries has been clearly demonstrated but infrequently reported [4,5].
Invasive percutaneous arterial procedures (diagnostic angiography) should be approached with caution as the vessel wall is likely to rupture. Angiography should be unjustified because of the increased risk of pseudoaneurysm formation at puncture sites. Noninvasive testing may allow early diagnose and early intervention for arterial complications. CT could easily be performed in this patient. CT was able to diagnose thrombosed pseudoaneurysm (Fig. 1).
Of the vascular changes in Behcet's disease, aneurysms develop at a relatively early age and are extremely prone to rupture. Because the incidence of carotid aneurysms is low and it is difficult to predict the natural history and risk of rupture of these abnormalities [6]. Therefore an aggressive surgical approach, initiated a soon as the diagnosis of this disease becomes definite, is mandatory for aneurysm. In our patient symptoms increasingly continued in the following 3 days. Since CT provided an excellent modality for evaluating pseudoaneurysm, the patient was operated on without applying any other invasive methods. When the patient was taken for operation, pseudoaneurysm had almost been fistulized on the surface of the skin.
Multiple aneurysms develop in Behcet's disease. However, Behcet's disease is the only vasculitis known to lead to pulmonary artery aneurysms [3,7]. In our patient, postoperative CT showed that right pulmonary artery aneurysms was present (Fig. 2).

View larger version (113K):
[in this window]
[in a new window]
|
Fig. 2. A postoperative CT shows that right pulmonary artery aneurysm and main pulmonary artery dilatation are present.
|
|
Most deaths from Behcet's disease stem from arterial complications resulting in ischemic bowel perforation, exanguinating hemorrhaging after aneurysm rupture, and cerebrovascular accidents. To prevent this outcome, such patients should receive regular immunosuppressive therapy. Our patient had received irregular immunosuppressive therapy before aneurysm formation.
In such patients, the approach should be focused on the early diagnosis made in that patient before the occurrence of the pseudoaneurysm and on the necessary avoidance of any diagnostic invasive arterial procedure.
 |
References
|
|---|
-
McCollum C.H., Wheeler W.G., Noon G.P., DeBakey M.E. Aneurysms of the extracranial carotid artery. Am J Surg 1979;137:196-200.[Medline]
-
Little A.G., Zarins C. Abdominal aortic aneurysm and Behcet's disease. Surgery 1982;91:359-362.[Medline]
-
Paç M, Koçak H., Ba
o
lu A., Yekeler
Femoral artery aneurysms in Behcet's disease. Vasc Surg 1991;25:719-723.
-
Enoch B.A., Castillo-Olivares J.L., Khoo T.C., Grainger R.G., Henry L. Major vascular complication in Behcet's syndrome. Postgrad Med J 1968;44:453-459.[Medline]
-
Hills E.A. Behcet's syndrome with aortic aneurysms. Br Med J 1967;4:152-154.
-
Rhodes E.L., Stanley J.C. Aneurysms of extracranial carotid arteries. Arch Surg 1976;111:339-343.[Abstract]
-
Durieux P., Bletry O., Huchon G., Whechsler B., Chretien J., Godeau P. Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-stovin syndrome. Am J Med 1981;71:736-741.[Medline]