伴有前房角发育异常的先天性瞳孔-虹膜-晶状体膜（一种新的病症）

Congenital pupillary-iris-lens membrane with goniodysgenesis (a new entity).

作者信息

Cibis G W, Waeltermann J M, Hurst E, Tripathi R C, Richardson W

出版信息

Ophthalmology. 1986 Jun;93(6):847-52. doi: 10.1016/s0161-6420(86)33659-5.

Abstract

We encountered two clinically similar but genetically unrelated cases of a disorder characterized by an unusual white pupillary-iris-lens membrane with extension to a prominent Schwalbe's line, the membranes were vascularized and their appearance changed over time. In both cases the abnormality was unilateral. Anomalous chamber angles were seen on gonioscopy. The clinical appearance is similar to a combination of iridogoniodysgenesis and pupillary membrane. However, our two cases are unique and fit into neither category. We postulate that this is a new entity consisting of an anomalous chamber angle and iris-lens membrane resulting from a localized iris (pupillary membrane), infarct with secondary neovascularization, during formation of the chamber angle.

摘要

我们遇到了两例临床症状相似但基因不相关的疾病病例，其特征为一种异常的白色瞳孔 - 虹膜 - 晶状体膜，延伸至明显的施瓦贝线，这些膜有血管化现象，且外观随时间变化。两例均为单侧异常。前房角镜检查可见异常的房角。临床表现类似于虹膜前房角发育异常和瞳孔膜的组合。然而，我们的这两个病例是独特的，不属于这两类中的任何一类。我们推测这是一种新的疾病实体，由房角形成过程中局部虹膜（瞳孔膜）梗死继发新生血管形成导致的异常房角和虹膜 - 晶状体膜组成。