Sturge-Weber综合征伴发青光眼临床特征

Clinical study on Sturge-Weber syndrome complicated with glaucoma

目的 Ｓｔｕｒｇｅ－ Ｗｅｂｅｒ 综合征伴发青光眼的临床特征。方法 对１９７８ 年１ 月至１９９９年３ 月收治的１９ 例２２ 眼ＳＷＳ青光眼患者的一般状况、眼部表现、以及伴发青光眼的治疗和预后进行回顾性分析。结果 平均患病年龄１０－７ａ，１９ 例２２ 眼均伴发青光眼，以开角型青光眼为主，伴中胚叶组织残留。３ 例双眼患病，３ 例伴脉络膜血管瘤。药物降压治疗效果不佳，行手术治疗共１８ 例，手术方式有小梁切开术、小梁切除术、巩膜下灼瘘术、虹膜嵌顿术、睫状体冷冻术。眼压从术前平均３５－４５ｍ ｍＨｇ（１ｍ ｍＨｇ＝ ０ ．１３３ｋＰａ） 降至术后平均１８－２４ｍ ｍＨｇ。主要手术并发症为脉络膜上腔出血，前房出血，术后浅前房，脉络膜脱离等。结论 Ｓｔｕｒｇｅ－Ｗｅｂｅｒ 综合征伴发青光眼发生率高，青光眼治疗预后差，与诊断发现较晚有关，提示临床应注意Ｓｔｕｒｇｅ－ Ｗｅｂｅｒ 综合征中青光眼的早期诊断。

Objectives To investigate the clinical characteristics of Sturge-Weber syndrome.Methods 22 eyes of 19 cases were analyzed retrospectively,including general conditions,ocular manifestation,treatment and progmosis.Results The average of patients was 10.7 years old.All the patients were complicated with glaucoma,Biocularly involved in 3 cases.The introcular pressure(IOP)can't be controlled well by medication.Anti-glaucoma operations were done in 18 cases.The IOP was undercontrolled from 35.45mmHg to 18.24mmHg.The common complications included suprachoroidal hemorrage,hyphema,exudative choroidal detachment,shallow anterior chamber.Conclusions The incidence of glaucoma in SWS is high.The prognosis is unfavorable.It suggests that the early diagnosis in SWS complicated with glaucoma is very important.)