先天性葡萄膜外翻

Congenital ectropion uveae

先天性葡萄膜外翻是一种罕见的眼科疾病,以虹膜基质前表面存在虹膜色素上皮、房角发育异常、进行性青光眼为特征.一般为非进行性,最主要的并发症是发展为先天性或青少年型青光眼.此病与神经嵴发育异常相关,可与某些遗传病伴发.较为特殊的临床表现是环绕瞳孔的虹膜色素膜样外翻,终止于虹膜的中周部.边界清晰,外翻的程度各方向不尽相同.几乎所有患者在确诊后都行滤过性手术治疗.

Congenital ectropion uveae （CEU） is a rare disorder characterized by overflows of the iris pigment epithelium covering the anterior iris surface, angle dysgenesis, and progressive glaucoma. The condition is generally nonprogressive but the main complication is congenital or juvenile glaucoma. This anomaly is correlated with neural crest dysplasia, and can be associated with some genetic diseases. The most typical manifestation is the pigment epithelium of the iris overflows into the pupil margin and covers a variable portion of the anterior iris surface. It may cover much of the iris, but does not extend to the iris root. Almost all patients require filtering surgery to control intraocular pressure.