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[白塞病合并多发性脑动脉瘤及上腔静脉阻塞所致下行性食管静脉曲张:一例报告]

[Behçet's disease associated with multiple cerebral aneurysms and downhill esophageal varices caused by superior vena cava obstruction: a case report].

作者信息

Tsuji S, Suzuki Y, Tomii M, Matsuoka Y, Kishimoto H, Irimajiri S

机构信息

Department of Internal Medicine, Kawasaki Municipal Hospital.

出版信息

Ryumachi. 1990 Oct;30(5):375-9, 381; discussion 379-81.

PMID:2084860
Abstract

A 62-year-old woman who had suffered from Behçet disease since 1975 was admitted to Kawasaki Municipal Hospital because of severe headache and consciousness disturbance in July 1988. The emergency brain CT scan showed subarachnoid hemorrhage. Despite clipping the neck of the cerebral aneurysm, she died of cerebral herniation. Post-mortem examination revealed four aneurysms on the anterior part of the circle of Willis: 1) at the first bifurcation of the right middle cerebral artery 2) at the bifurcation of the left internal carotid artery 3) at the anterior communicating artery 4) at the left middle cerebral artery. The complete obstruction of the superior vena cava and downhill esophageal varices with no evidence of portal hypertension were also observed. This case had downhill esophageal varices induced by vena caval obstruction as a quite rare symptom of vasculo-Behçet's disease and also complicated with congenital multiple aneurysms of cerebral arteries.

摘要

一名自1975年起患有白塞病的62岁女性,于1988年7月因严重头痛和意识障碍入住川崎市立医院。急诊脑部CT扫描显示蛛网膜下腔出血。尽管对脑动脉瘤颈部进行了夹闭,但她仍死于脑疝。尸检发现 Willis 环前部有四个动脉瘤:1)位于右大脑中动脉第一分支处;2)位于左颈内动脉分支处;3)位于前交通动脉处;4)位于左大脑中动脉处。还观察到上腔静脉完全阻塞和下行性食管静脉曲张,且无门静脉高压证据。该病例有由腔静脉阻塞引起的下行性食管静脉曲张,这是血管性白塞病极为罕见的症状,并且还合并先天性多发性脑动脉动脉瘤。

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