Author(s)

P. Polat, M. Celik , F. Alper, M. Kantarci, S. Suma

Patient

female, 27 year(s)

We presented the Color Doppler US, BT and angioraphic appearances of multiple pulmonary artery aneurysm and common carotid artery pseudoaneurysm in 25-year old woman.

A 27-year- old woman was admitted to our hospital with the complaints of gradually increasing mass lesion and pain in her right cervical region from 2 days. She had a history of recurrent oral and aphtous ulcers from 2 years. A pathergy test revealed positive findings. At US evaluation, an anechoic lesion measured 15 mm in diameter was detected with surrounding iso- hypoechoic areas consistent with hemorrhage. This lesion was connected to common carotid artery with a narrow neck. On color Doppler, the lesion showed ying yang pattern. On contrast enhanced axial computerized tomography (CT) sections, the lumen of the lesion showed extreme contrast enhancement. The surrounding hemorrhage was seen as heterogeneous densities. On 3D spiral CT angio section, the narrow neck of the lesion connected to the common carotid artery was demonstrated (Fig 1). On axial thorax CT sections, an aneurysm was detected in the right pulmonary artery and three aneurysms in the branches of the left pulmonary artery (Fig2a-b). One of them was in ascending branch, two of them were in the descending branches of the left pulmonary artery. Digital substraction angiography sections showed an aneurysm measuring approximately 3 cm in diameter in the ascending branch of the right pulmonary artery. There was one aneurysm,13 mm in diameter in ascending branch of the left pulmonary artery and two aneurysms approximately 10 mm in diameter in the descending branches of left pulmonary artery (Fig 3).

Behçet’s disease (BD) is a multisystemic disorder characterized by recurrent ulcers of the mouth and genitalia and relapsing iritis (1). The vascular lesions encountered in BD are arterial occlusions, aneurysm, venous occlusions, and variceal development (1,2). The disease affects both arteries and veins (3). The reported incidence of vascular involvement ranges 7% to 29% in the literatures. Bilateral pulmonary artery aneurysms are rare but recognized consequence of vascular involvement in BD (4). But to our knowledge there has been only one case who had pseudoaneurysm at internal carotid artery in the literature (5). BD, in general occurs more commonly in men and predominately affects people in their second and third decades. The incidence of the disease is considerably higher in Turkey, Israel, Lebanon, Iran, Japan, Korea and China (1,2). The prevalence of vascular involvement is about 25% and vascular involvement is the leading cause of death in BD(1-5). Vascular involvement is more frequent in males than in females (36% and 14%). Three forms of vascular disease (venous occlusion, arterial aneurysms and /or arterial occlusion) are found in BD. Venous lesions ( 88%) are more frequently than arterial lesions (12%). The most common site of aneurysms in BD is abdominal aorta following femoral artery, popliteal artery and pulmonary artery (1). Arterial involvement generally occurs in the late stage of the disease, usually in young men (1,3,4). Although pulmonary aneurysms are usually encountered in patients with BD, to our knowledge there has been no case reported more than 3 aneurysms with pseudoaneurysm formation in the common carotid artery. In our case, we detected total 4 aneurysms at the branches of pulmonary artery. Our case was a-27 year old woman. Her dermatological symptoms were present for 2 years. A pseudoaneurysm was detected in her right common carotid artery relatively rare site and formation of involvement. The development of aneurysm and pseudoaneurysm at arteries in BD can be explained by the pathologic process in the wall of the blood vessels. Immune complex deposition in small vessels leads to complement fixation and polymorphonuclear leukocyte activation. The neutrophil activation and perivascular infiltration in the vessel wall lead to degeneration and occlusion of the vasa vasorum. Occlusion of the vasa vasorum leads to transmural necrosis of the wall of large muscular arteries. Mild to advanced fibrinization can be seen in intima and adventitia, which may result true aneurysm formation. Ultimately perforation of the vessel wall can occur, and pseudoaneurysm formation follows(1).