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与甲型血友病相关的烟雾病。病例报告。

Moyamoya disease associated with hemophilia A. Case report.

作者信息

Matsuda Masahide, Enomoto Takao, Yanaka Kiyoyuki, Nose Tadao

机构信息

Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan.

出版信息

Pediatr Neurosurg. 2002 Mar;36(3):157-60. doi: 10.1159/000048372.

Abstract

A 10-year-old boy who had been diagnosed as having hemophilia A presented with episodes of transient ischemic attack. Cerebral angiography showed occlusions of the bilateral anterior cerebral arteries and the right middle cerebral artery with the development of an abnormal vascular network. The patient was diagnosed as having moyamoya disease associated with hemophilia A and subsequently underwent multiple burr hole surgery for revascularization under sufficient factor VIII supplementation. He remained asymptomatic after surgery, and follow-up cerebral angiography 5 months after the operation demonstrated significant neovascularization through the burr holes. Multiple burr hole surgery is proposed as a method of choice for the treatment of moyamoya disease associated with a bleeding tendency.

摘要

一名10岁男孩,此前被诊断为患有甲型血友病,现出现短暂性脑缺血发作。脑血管造影显示双侧大脑前动脉和右侧大脑中动脉闭塞,并伴有异常血管网形成。该患者被诊断为与甲型血友病相关的烟雾病,随后在充分补充凝血因子VIII的情况下接受了多次骨孔手术以实现血管重建。术后他一直无症状,术后5个月的随访脑血管造影显示通过骨孔有明显的新生血管形成。对于伴有出血倾向的烟雾病,建议将多次骨孔手术作为一种治疗选择方法。

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