Three Cases of Simple Ectopia Lentis in One Family.

Hong, Ki Young; Joh, Sheen Chull; Chung, Young Tea

Case Report

Journal of the Korean Ophthalmological Society 1986;27(5):867-873.
Published online May 1, 1986.

Three Cases of Simple Ectopia Lentis in One Family.

Ki Young Hong, Sheen Chull Joh, Young Tea Chung

Deptartment of Ophthalmology, Presbyterian Medical Center, Chonju, Korea.

한 가족에 발생한 선천성 수정체 탈구증 3예

정영태(Young Tae Chung),홍기영(Ki Young Hong),조신철(Sin Chul Cho)

Abstract

Ectopia lentis, Synonymously with Congenital dislocated lens, is defined as a dislocation of the ocular lens, of greater or less degree, based on a developmental anomaly. It occurs in three conditions, as an isolated anomaly, in association with ocular anomaly, and as a part of a systemic syndrome with other mesodermal, particularly skeletal anomalies. Among these, simple ectopia lentis is a hereditary autosomal dominant disorders without skeletal, connective tissue or metabolic symptomatology. Except for the dislocation of the lens, these eyes are in other respects grossly normal. Regarding its management, there is a continuing debate proponents of active surgical intervention to prevent future problems to those who will not remove the lens and advocates of conservative treatment because lens surgery can be difficult and has a high rate of intraoperative and post operative complication. Recently, the authours have experienced three patients with simple ectopia lentis, which occurred in one family with autosomal dominant inheritance and achieved satisfactory result surgically. Thus these cases are presented with brief review of literatures.