Oda Masashi, Takahashi Jun A, Hashimoto Nobuo, Koyama Tsunemaro
Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Surg Neurol. 2004 Apr;61(4):353-6. doi: 10.1016/j.surneu.2003.05.001.
Intracranial varices are rare and most are associated with vein of Galen arteriovenous malformations (AVM) or fistulas (AVF).
A 43-year-old left-handed man presented with right hemihypesthesia and spastic gait. Neuroradiological examination revealed a spinal AVF and a giant intracerebral varix associated with a high-flow pial AVF. He had recurrent episodes of nasal bleeding, which were also confirmed in his mother's medical history, and telangiectases in the tip of his tongue and fingers. He was diagnosed with Rendu-Osler-Weber disease. After resection of the spinal AVF that produced his symptoms, we surgically exposed and obliterated the giant varix and AVF under intra- and postoperative hypotension and mild barbiturate therapy. The arteriovenous shunt was completely obliterated without hyperperfusion of the surrounding brain.
This is an extremely rare case of Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF that did not involve the vein of Galen.
颅内静脉曲张罕见，多数与大脑大静脉动静脉畸形（AVM）或瘘（AVF）相关。
一名43岁左利手男性，表现为右侧半身感觉减退和痉挛性步态。神经放射学检查发现一个脊髓AVF和一个巨大的脑内静脉曲张，伴有高流量软脑膜AVF。他有反复鼻出血发作史，其母亲的病史中也有此情况，且舌尖和手指有毛细血管扩张。他被诊断为遗传性出血性毛细血管扩张症。在切除引起其症状的脊髓AVF后，我们在术中和术后低血压及轻度巴比妥类药物治疗下，手术暴露并闭塞了巨大的静脉曲张和AVF。动静脉分流完全闭塞，周围脑组织无过度灌注。
这是一例极为罕见的遗传性出血性毛细血管扩张症，伴有继发于高流量软脑膜AVF的巨大脑内静脉曲张，且未累及大脑大静脉。
