脑面血管瘤病的影像特征和手术治疗

Imaging characteristics and surgical treatment of Sturge-Weber syndrome

目的总结脑面血管瘤病的治疗经验,探讨其影像学特征及手术治疗要点。方法回顾分析2009年以来手术治疗的脑面血管瘤病(Ⅰ型)患者3例(男2例,女1例),术前行CT、MRI及视频脑电图检查,手术切除病灶后随访1～2年评估疗效。结果 3例患者CT均显示钙化,强化MRI扫描显示硬脑膜和蛛网膜血管扩张、增生,脑组织萎缩,可明确病灶范围。手术疗效:颞枕叶切除2例,半球切除1例,术后均无癫痫发作。结论结合患者临床表现及CT、MRI检查可基本确诊脑面血管瘤病,对有癫痫发作的患者应早期接受手术治疗,术前和术中行脑电监测,在不增加功能损伤的前提下完全切除病灶可治疗癫痫发作。

Objective To analyze imaging findings and surgical therapy of Sturge-Weber syndrome,and to explore the effectiveness of multi-lobectomy for Sturge-Weber syndrome.Methods The data of 3 Sturge-Weber syndrome patients（type I,2 males and 1 female） were analyzed.Before surgery,all patients underwent CT and MRI scan and VEEG test and after surgery,they were followed up for 1 to 2 years.Results In CT scans,the 3 patients manifested calcification in brain tissue.Abnormal blood vessels and cerebral atrophy were displayed in MRI imaging,and the disease area could be determined.2 patients accepted tempro-occipital craniotomy,and the other patient accepted hemispherectomy.After 1 to 2 years follow-up,all patients had epilepsy release.Conclusion Sturge-Weber syndrome can be determined by combining clinical manifestation and CT/MRI imaging.Surgical therapy should be taken as early as possible in patients with epilepsy.The total removal of lesions without adding functional deficiency can release the epilepsy seizure of Sturge-Weber syndrome.