Kuechler Derek, Kaliaperumal Chandrasekaran, Hassan Alfrazdaq, Fanning Noel, Wyse Gerry, O'Sullivan Michael
Department of Neurosurgery, Cork University Hospital, Cork, Ireland.
BMJ Case Rep. 2011 Nov 15;2011:bcr1020114920. doi: 10.1136/bcr.10.2011.4920.
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.
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。