先天性葡萄膜外翻与青光眼

目的 探讨先天性葡萄膜外翻的临床特点及其与青光眼的关系.方法 回顾性系列病例研究.收集2002年9月至2008年5月间共11例(16只眼)先天性葡萄膜外翻患者的临床资料,回顾性分析其临床特点和治疗效果.结果 11例(16只眼)先天性葡萄膜外翻患者中,有10例(14只眼)合并青光眼,1例(2只眼)诊断为高眼压症.有4例(5只眼)合并先天性上睑下垂,2例(3只眼)合并眼球突出,1例(1只眼)合并先天性白内障,1例(2只眼)合并先天性晶状体半脱位,1例(2只眼)合并先天性小角膜,3例合并并指畸形,1例合并Rieger综合征.9例(12只眼)青光眼患者在接受复合式小梁切除术后,10只眼的眼压得到有效控制.结论 先天性葡萄膜外翻是一种罕见的先天性异常,常伴随其他眼部畸形和全身异常;其发生青光眼的机制与虹膜前房角的发育异常有关.对先天性葡萄膜外翻合并青光眼的患者应尽早发现,早期干预和治疗.

Congenital ectropion uveae and secondary glaucoma

Objective To investigate the clinical features of congenital ectropion uveae (CEU) and the relationship between CEU and secondary glaucoma.Methods This is a retrospective case series.Clinical features and treatments of 11 patients with CEU were summarized.Combined with the literatures review of CEU, the pathogenesis and therapeutic results of secondary glaucoma were analyzed.Results Among these 11 patients, there were 10 patients with secondary glaucoma and one patient with ocular hypertension.There were 4 patients complicated with congenital ptosis, 2 patients with ocular proptosis,1 patient with congenital cataract, 1 patient with lens subluxation, 3 patients with ankylodactylia and 1 patient with Rieger's syndrome.The intraocular pressure of 9 patients was controlled after the trabeculectomy.Conclusions CEU is a rare, nonprogressive anomaly accompanied other ocular abnormalities and various systemic disorders.The pathogenesis of secondary glaucoma is related to iridotrabecular dysgenesis.It is essential to detect and to treat the secondary glaucoma as early as possible.