Cataract in coloboma: ectopic lens lying in a retinochoroidal coloboma

Abstract

A 79-year-old-man presented for routine ophthalmological examination. He had a history of untreated amblyopia in the right eye and bilateral cataracts. His uncorrected visual acuity was light perception in the right eye and 20/160 in the left eye. Slit-lamp examination revealed typical inferonasal iris colobomas, apparent aphakia in the right eye, and cataract in the left eye (A). Funduscopic examination (B) complemented by B-scan ultrasonography of the right eye (C) revealed a luxated lens (arrow) lodged in a retinochoroidal coloboma (arrowheads), in association with proliferative vitreoretinopathy. Coloboma results from incomplete closure of the embryonic fissure. The affected structure depends on whether that failure is anterior (iris coloboma), posterior (retinochoroidal coloboma) or both, such as in this case. Involvement of the zonules or ciliary body in anterior coloboma predisposes to ectopia lentis because there is insufficient lens support.