Weekly Case

Title : Case 50

첨부파일 : 이연수_선생님_증례.ppt

Age / Sex : /


Age /sex : 37/M
C.C.: Low abdominal pain , Behcet’s disease history





1) What is your impression?

Courtesy : Yeon Soo Lee, MD Department of Radiology, Catholic University, College of Medicine, Daejeon Sung Mo Hospital


Diagnosis:

abdominal aortic aneurysm with intramural thrombus and several lumbar vertebral erosion



Discussion


Findings:
- Lumbar spine plain lateral film shows severe erosion of the ventral aspect of L3 body. Aortc stent is seen from L1 to L3 level.
- Sagittal T2 weighted MR image shows well defined high signal intensity mass replacing L3 body and extending disc with prevertebral space ( L2 ~ 3 level ). This lesion shows low signal intensity including some high signal focus on T1WI. Postcontrast T1WI shows enhanced aneurysmal dilatation of the abdominal aorta, just below inferior aspect of the aortic stent and nonenhanced prevertebral lesion encasing aortic lumen ( L2 ~ L3 level ). This pervertebral lesion causes marked erosion of L3 body and extends retrovertebral epidural space with L2 ~ 3 disc level.

Differential Diagnosis:
1. Neoplastic disease
2. Spondylitis

Discussion:
Behcet’s disease is a multisystem disorder that is commonly seen in Japan and the Mediterranean region. The syndrome is characterized by the triad described first by Behçet in 1937: oral ulceration, genital ulceration and uveitis. Additional features are synovitis, cutaneous vasculitis resembling erythema nodosum, meningoencephalitis and vascular involvement. The diagnosis remains clinical, as there are no pathognomonic serological or pathological tests. It is based upon five major criteria: recurrent oral ulcerations in combination with genital ulceration, eye lesions, skin lesions or a positive pathergy test. The appearance of new clinical features months or years after onset is common. The most important complications are uveitis, meningoencephalitis and large-vessel disease. Vascular involvement has been reported in 7.7-60% of cases; 25% of such lesions affect the venous system exclusively, whereas only 7% affect the arterial system. The majority of patients with vascular involvement (68%) present with both venous and arterial lesions. The vascular lesions in BD include arterial and venous occlusions and the formation of aneurysms and pseudoaneurysms, or a combination of these conditions. The most frequently reported vascular complication is thrombosis of the large veins of the limbs and superior or inferior caval vein syndrome. Arterial involvement is less common and consists mostly of aneurysm and pseudoaneurysm formation or arterial occlusion. Occlusion and aneurysms of the large systemic arteries can cause life-threatening or fatal illness from limb ischaemia, stroke, renal hypertension or aortic aneurysm. Aneurysms of the large arteries, such as the abdominal aorta and the pulmonary artery, are reported most frequently, although nearly every major artery has been found to be involved Although vertebral erosion associate with relative frequency to atherosclerotic aneurysm , it is rarely seen in BD. Vertebral collapse and lytic lesions usually are related to fracture, tumors, osteoporosis, spondylitis, or spondylodiscitis. This case shows abdominal aneurysm can cause spinal erosion and may mimic tumor or infections in BD. Treatment of BD is controversial, reconstruction with a prosthetic graft is usually unavoidable for an aneurysm or pseudoaneurysm, especially when the aorta is affected. Surgery must be performed as early as possible, because rupture of an inflammatory aneurysm can occur even when it is small. Because of the inflammation of the surrounding tissues, surgical repair is often difficult. Some authers prefer to perform only an aneurysmorraphy.

References:
1. El Maghraoui A, Tabache F, El Khattabi A, Bezza A, Abouzahir A, et al. Abdominal aortic aneurysm with lumbar vertebral erosion in Behçet's disease revealed by low back pain: a case report and review of the literature. Rheumatology (Oxford). 2001; 40(4):472-3.
2. Barros M, Lozano F, Almazan A, Arias R. Angio-Behçet with vertebral erosion: an exceptional Behçet's complication and literature review. Joint bone spine 2004 ;71(6):577-9.
3. Diekerhof CH, Reedf Dortland RW, Oner FC, Verbout AJ. Severe erosion of lumbar vertebral body because of abeominal aortic false aneurysm: report of two cases. Spine 2002; 15:27(16):E382-4



Correct Answer
Name Institution
오배근 (정답) : 일산백병원 전공의
박상현 (정답) : 순천향 천안병원 전공의

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