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Tex Heart Inst J. 2005; 32(1): 95–98.

Large Pseudoaneurysm of the Carotid Artery in Behcet's Disease

Hakan Posacioglu, MD, Anil Z. Apaydin, MD, Mustafa Parildar, MD, and Suat Buket, MD

Departments of Cardiovascular Surgery (Drs. Apaydin, Buket, and Posacioglu) and Radiology (Dr. Parildar), Ege University Medical Faculty, Bornova -- Izmir, Turkey

Abstract

Behcet's disease is a multisystem inflammatory disease of unknown cause. Its major pathologic feature is vasculitis. We report the occurrence of a large pseudoaneurysm of the carotid artery in a patient who had Behcet's disease. The patient underwent surgery and was discharged on the 7th postoperative day.

Ours is only the 4th such case reported in the English-language medical literature. Endovascular and surgical options are discussed herein.

Key words: Aortic valve prosthesis, durable medical equipment, endocarditis, bacterial, heart valve prosthesis, human, male, postoperative complications, reoperation, time factors

Behcet's disease is seen most often in young adults of Mediterranean or Asian descent. It is characterized by relapsing uveitis, and oral and genital ulcerations. The typical vascular feature is medium- and large-vessel involvement, which causes both arterial occlusive disease and aneurysm formation. The cessation of nutrient flow to the arterial wall due to inflammatory obliteration of the vasa vasorum can lead to perforation or pseudoaneurysm formation. The incidence of large arterial complications ranges from 2% to 6% in patients from 20 to 40 years of age with Behcet's disease.1,2

We report the case of a man with Behcet's disease who had a pseudoaneurysm of the right carotid artery.

Case Report

In June 2003, a 31-year-old man who had been experiencing neck pain, dyspnea, and hoarseness was referred to our hospital because of a rapidly expanding mass in the right side of his neck (Fig. 1). On admission, the patient was hemodynamically stable. Physical examination revealed a mass that was pulsatile and tender on palpation. A detailed medical history revealed that the patient had relapsing oral and genital ulcerations and recurrent thrombophlebitis. Results of routine laboratory tests were normal except for a high erythrocyte sedimentation rate and high C-reactive protein levels. After consultation with a rheumatologist, we established a diagnosis of Behcet's disease.

Computed tomography revealed a 75-mm-diameter pseudoaneurysm with a large mural thrombus at the bifurcation of the right common carotid artery (Fig. 2). Carotid angiography showed that the false aneurysm originated from the right carotid artery bifurcation (Fig. 3). Urgent surgery was deemed necessary to prevent fatal complications of this rapidly expanding pseudoaneurysm.

Surgical Technique

After the induction of general anesthesia, endotracheal intubation, and insertion of arterial and central venous lines, the patient was positioned for a carotid endarterectomy. A skin incision was made in the neck along the anterior border of the sternocleidomastoid muscle, and was extended toward the jugular notch of the sternum for better exposure of the proximal right common carotid artery. The pseudoaneurysm was densely adherent to the sternocleidomastoid muscle. Isolation and external clamping of the internal or external carotid artery distal to the pseudoaneurysm were impossible. After the administration of heparin, the common carotid artery was clamped just distal to the bifurcation of the brachiocephalic trunk. The pseudoaneurysm was entered, and a large mural thrombus was evacuated. Because of backbleeding from the internal and external carotid artery orifices, we occluded both arteries endoluminally by inflating a Fogarty balloon embolectomy catheter in each one. The arteries were dissected and freed from the surrounding structures to obtain a length of artery sufficient to place a clamp and perform a safe anastomosis. A Javid shunt was made in the internal carotid artery. The shunt was filled with blood in a retrograde fashion, and the proximal end of the shunt was inserted in the common carotid artery. After blood flow was restored, the external carotid artery was ligated. The gap between the internal and common carotid arteries was too long, due to the large arterial wall defect and resection of damaged tissue. Therefore, end-to-end anastomoses were used to interpose a saphenous vein between the internal and common carotid arteries (Fig. 4).

The patient had an uneventful postoperative course. Histologic examination of the false aneurysm wall showed fragmentation of the internal elastic membrane and infiltration of inflammatory cells to the outer layer of the artery. Immunosuppressive therapy was begun, and the patient was discharged on the 7th postoperative day. Four months postoperatively, follow-up angiography showed a patent vein graft (Fig. 5). When last seen in October 2004, the patient was well.

Discussion

Arterial involvement is a rare but serious complication of Behcet's disease. The inflammatory process caused by the disease can affect all vessels, regardless of size, with a wide spectrum of clinical manifestations. Occlusive arterial disease is more common in the lower limbs and may have a better prognosis than that of aneurysm formation.3 Aneurysms of the large arteries are often multiple; moreover, the vasculitis associated with Behcet's disease is also known to lead to pulmonary artery aneurysm.4,5

Aneurysms in major vessels such as the abdominal aorta and the iliac and pulmonary arteries have been reported frequently in patients with Behcet's disease; however, the incidence of carotid artery aneurysm is small ---only 3 cases of false aneurysms in the carotid arteries have been previously reported in the English-language medical literature.6 --8 This lack of data makes it difficult to predict the course and risk of rupture of these aneurysms. Therefore, aggressive treatment, which includes surgical or endovascular intervention, is recommended for the management of such aneurysms. Experience with endovascular repair of aneurysms in Behcet's disease is limited, but such repair is the only option for surgically inaccessible lesions. Only 2 cases of endovascular treatment of a carotid pseudoaneurysm in patients with Behcet's disease have been reported. In the 1st patient, a surgically inaccessible left internal carotid artery pseudoaneurysm at the base of the skull was treated by endovascular placement of an uncovered stent combined with the use of coils.6 In the 2nd case, a right common carotid artery pseudoaneurysm was treated with use of a covered stent. However, the covered stent was completely occluded within 6 months of placement.7

Complications of surgery, including graft thrombosis and pseudoaneurysm formation, are frequently reported in patients with Behcet's disease. However, suture line reinforcement with a strip of Teflon felt, surgery during the inactive stage of the disease, and the use of autografts can decrease the incidence of pseudoaneurysm formation.3 A 3rd report of carotid artery pseudoaneurysm was about a patient with a history of iatrogenic carotid artery puncture.8 This patient was surgically treated with a polytetrafluoroethylene interposition graft between the internal and common carotid arteries.

We preferred surgical intervention in our patient because of the size of the pseudoaneurysm, its compressive effect on the surrounding structures, and the unproven record of endovascular therapy. We chose an autogenous graft instead of synthetic material for our patient, because the integrity of the anastomosis of a saphenous vein autograft to the host artery becomes independent of the sutures within a few weeks. We believe that this rapid healing process decreases the incidence of subsequent pseudoaneurysm formation, especially during the active stage of the disease.

Patients who have Behcet's disease usually die of arterial complications. To prevent this outcome, such patients should receive regular immunosuppressive therapy.1,2,7,8 The 3 previously reported patients with carotid pseudoaneurysms received only intermittent immunosuppressive therapy; our patient did not receive any immunosuppressive therapy before diagnosis.

The diagnosis of Behcet's disease should always be considered in young adult patients from the Mediterranean region or Asia who present with an aneurysm or pseudoaneurysm at an unusual location. Patients with Behcet's disease should be evaluated regularly for early diagnosis of arterial complications, and surgery or endovascular intervention should be considered for any aneurysm posing a risk of rupture.

Acknowledgment

We wish to thank Kenan Aksu, MD, from the Department of Rheumatology, for his assistance in the diagnosis of Behcet's disease.

Footnotes

Address for reprints: Hakan Posacioglu, MD, Ege University Medical School, Kalp ve Damar Cerrahisi Anabilim Dali, 35100 Izmir, Turkey. E-mail: http://www.pubmedcentral.gov/redirect3.cgi?&&reftype=extlink&artid=555835&iid=18544&jid=92&&posacioglu@yahoo.com

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Kwon Koo B, Shim WH, Yoon YS, Kwon Lee B, Choi D, Jang Y, et al. Endovascular therapy combined with immunosuppressive treatment for pseudoaneurysms in patients with Behcet's disease. J Endovasc Ther 2003;10:75 --80.  .
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Figures and Tables
Fig. 1 The large and tender pulsatile mass in the right neck.
Fig. 2 Computed tomography shows a large pseudoaneurysm (about 75 mm in diameter) that originates in the bifurcation of the right carotid artery, and tracheal compression by the pseudoaneurysm.
Fig. 3 Angiography shows the pseudoaneurysm of the right common carotid artery.
Fig. 4 Intraoperative image after interposition of the reversed saphenous vein between the internal and common carotid arteries by end-to-end anastomoses.
Fig. 5 Follow-up angiography shows a patent vein graft 4 months postoperatively.