Unilateral infantile glaucoma associated with congenital iris ectropion

We report an 8-year-old female with congenital iris ectropion associated with unilateral glaucoma treated by trabeculectomy. Major features, including nonprogressive ectropion of the iris pigment epithelium, glassy smooth cryptless surface of the iris, anterior insertion of the iris root, dysgenesis of the drainage angle and mild ipsilateral blepharoptosis, were noted in the affected (left) eye, while specular microscopy revealed normal corneal endothelium. The right eye was thought to be normal. Intraocular pressure in the left eye responded poorly to antiglaucoma medications. Trabeculotomy was attempted but encountered difficulties in identifying Schlemm's canal, so was changed to trabeculectomy, which succeeded in controling the elevated pressure. Although histopathological examinations were insufficient, due to the severely damaged trabeculectomized specimens, it is suggested that glaucoma may be caused by dysgenesis of the trabeculum based on arrest of neural crest cell development.