无面部血管痣Sturge-weber综合征的手术治疗及文献复习

Surgical Treatment of Sturge-Weber Syndrome without Facial Nevus

目的:提高对无面部血管痣的Sturge-weber综合征(SWS)的诊治水平。方法:总结1例无面部血管痣SWS患者的临床治疗经过,并对相关文献进行复习。结果:患者癫痫起病,无面部血管痣体征,但根据患者的临床表现、术前CT、MRI诊断为SWS,手术治疗后病理支持SWS诊断。随访2年,患者癫痫未复发。文献亦有无面部血管痣SWS患者的相同报道,多数学者认为手术对于SWS患者效果显著。结论:无面部血管痣的SWS罕见,诊断主要通过CT、MR以及病理进行诊断。早期诊断及早期手术对于控制患者癫痫发作具有明显效果。

Objective：To improve the diagnosis and early treatment of Sturge-Weber syndrome without facial nevus.Methods：To summarize the experience of surgical treatment of a patient suffer Sturge-Weber syndrome without facial nevus,and review some literature association with SWS.Results：The first clinical syndrome of the patient was epileptic seizure,although there was no facial nevus on his face,according to the clinical syndrome and results of the CT and MRI SWS was diagnosed.The patient accepted surgical treatment,and the histopathologic images of surgical resection from the patient support the preoperative diagnosis.In 2 years follow-up,the patient had no recurrence.Patient without a cutaneous nevus but with typical clinical and radiographic findings of SWS have been reported.Most author believe that surgical treatment of SWS is remarkable.Conclusion：Sturge-Weber syndrome without facial nevus is rarely.Early diagnosis and early surgical of SWS is very important for the seizure control.