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帕里-罗姆伯格综合征中的动脉瘤性蛛网膜下腔出血。

Aneurysmal subarachnoid haemorrhage in Parry-Rhomberg syndrome.

作者信息

Kuechler Derek, Kaliaperumal Chandrasekaran, Hassan Alfrazdaq, Fanning Noel, Wyse Gerry, O'Sullivan Michael

机构信息

Department of Neurosurgery, Cork University Hospital, Cork, Ireland.

出版信息

BMJ Case Rep. 2011 Nov 15;2011:bcr1020114920. doi: 10.1136/bcr.10.2011.4920.

Abstract

Parry-Romberg syndrome (PRS) or progressive hemi facial atrophy syndrome is a rare condition of unknown aetiology that is characterised by progressive unilateral facial and cranial atrophic changes of skin, subcutaneous tissues and bone. The authors describe a 37-year-old female with a history of PRS, who presented with a subarachnoid haemorrhage secondary to rupture of a 9 mm fusiform aneurysm of the posterior cerebral artery. There was an associated external carotid arterio-venous fistula noted with this aneurysm. The aneurysm was treated by endovascular route and was successfully coiled. Follow-up angiogram revealed spontaneous resolution of the fistula with good occlusion of the aneurysm. The aetio-pathogenesis of this rare occurrence, literature review and its management is discussed.

摘要

帕里-罗姆伯格综合征(PRS)或进行性半侧面部萎缩综合征是一种病因不明的罕见疾病,其特征为皮肤进行性单侧面部及颅骨皮肤、皮下组织和骨骼的进行性萎缩性改变。作者描述了一名37岁患有PRS病史的女性,她因大脑后动脉9毫米梭形动脉瘤破裂继发蛛网膜下腔出血。该动脉瘤伴有颈外动静脉瘘。通过血管内途径对动脉瘤进行了治疗,并成功进行了栓塞。随访血管造影显示瘘口自发闭合,动脉瘤栓塞良好。本文讨论了这种罕见情况的病因病理、文献综述及其治疗。

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