Sturge—Weber综合征并发青光眼的临床分析  

Clinical analysis on sixteen cases of Sturge-Weber syndrome-associated glaucoma

作　　者：杨强[1] 郭文毅[1] 孙兴怀[1] 孟樊荣[1] 宋月莲[1] 钱韶红[1] 王嘉健[1] 陈倩[1] 

机构地区：[1]复旦大学附属眼耳鼻喉科医院眼科,上海200031

出　　处：《中华眼科杂志》2009年第10期883-887,共5页Chinese Journal of Ophthalmology

摘　　要：目的探讨Sturge-Weber综合征并发青光眼的临床特点及手术治疗效果。方法回顾性系列病例研究。收集2003年1月至2007年12月住院治疗的16例（21只眼）Sturge-Weber综合征并发青光眼患者的临床资料，进行回顾性分析。应用Stata7．0统计学软件对数据进行分析。对有随访资料的10例（11只眼）患者术前、后眼压比较采用配对t检验。结果患者年龄为1个月至31岁，中位数年龄为11岁。颜面部血管瘤位于双侧的有8例，其中2例伴有全身皮肤广泛血管瘤。16例患者中，并发单侧青光眼11例，其中3例伴有双侧颜面部血管瘤；并发双侧青光眼5例，均伴有双侧颜面部血管瘤。16例患者前房角镜检查均为宽角。21只患眼均行B超检查，其中8只眼（38．1％）的脉络膜探及弥漫性中高回声占位性病变。超声活体显微镜检查显示有虹膜后凹、睫状体内低回声区、睫状体浅脱离等。有18只眼进行了抗青光眼手术治疗，包括小梁切开术、小梁切除术、非穿透性深层巩膜切除术、Ahmed青光眼阀植入术、睫状体冷冻术，术后发现行小梁切除术的5只眼中，有4只眼出现脉络膜脱离。术后10例（11只术眼）患者平均随访22个月，随访时患者眼压均低于其术前眼压（均≤21mmHg，1mmHg=0．133kPa），术前后眼压差异有统计学意义（t=5．3956，P〈0．01）。结论Sturge—Weber综合征并发青光眼患者的临床特征为颜面部血管瘤、宽房角、脉络膜血管瘤。抗青光眼手术治疗能有效降低眼压。Objective To investigate the clinical features of Sturge-Weber syndrome-associated glaucoma and its surgical treatment. Methods A retrospective case series study. The general clinical data and related ocular manifestations in 16 patients （21 eyes） with Sturge-Weber syndrome-associated glaucoma in our hospital from January 2003 to December 2007 were analyzed retrospectively. Results Age of the patients ranged between 1 month to 31 years old, and the median age was 11 years. Bilateral facial angiomas were present in 8 patients, and two of them had extensive hemangioma. Eleven cases had unilateral glaucoma and 3 of them had bilateral facial angiomas. Five patients had bilateral glaucoma and all of them had bilateral facial angiomas. Open anterior chamber angle was found in all affected eyes by gonioscopy. B-scan ultrasonography was performed in 21 eyes and diffused occupying lesions in the choroid were found in 8 eyes （38.1% ）. Posterior bowing of the iris, low echo in ciliary body and shallow ciliary body detachment were found by ultrasound biomicroscopy. Anti-glancoma surgeries including trabeculotomy, trabeculectomy, non- penetrating deep sclerectomy, valve implantation and cyclocryotherapy were performed in 18 eyes. Eighty percent of the eyes which underwent trabeculectomy developed choroidal detachment after operation. Ten patients （ 11 eyes） were followed-up for 22 months on average. Intraocular pressure was significantly lower than that before the operation （ t = 5. 3956, P 〈 0. 01 ）. Intraocular pressure in all fallowed-up eyes was controlled at ≤21 mm Hg （1 mm Hg = 0. 133 kPa）. Conclusions The clinical features of Sturge-Weber syndrome-associated glaucoma include facial angiomas, open anterior chamber angle and choroidal hemangioma. Anti-glaucoma surgery can reduce the intraocular pressure effectively.

关 键 词：Sturge—Weber综合征 青光眼 脉络膜肿瘤 显微镜检查 声学 眼外科手术 

分 类 号：R686[医药卫生—骨科学]