Department of Ophthalmology, Taegu Fatima Hospital

No authors listed,

Abstract

Axenfeld-Rieger syndrome is a rare autosomal dominantly inherited, developmental disorder characterized by ocular and systemic abnormalities. Ocular defects are typically bilateral, and most commonly involved structures are the peripheral cornea, anterior chamber angle, and iris.More than half of the patients develop secondary glaucoma.Common systemic anomalies of the syndrome are defects of the teeth and facial bones. We experienced a 20-year-old man who had Axenfeld-Rieger syndrome with iris and anterior chamber ang leabnormalities, anteriorly displaced Schwalbe's line and associated glaucoma in both eyes.The patient also had characteristic facial and dental abnomaities with a familial history of the disorder.We performed combined surgery of trabeculectomy, pupilloplasty and lens aspiration with posterior chamber IOL implantation in order to correct the corectopia, anisometropia and the associated glaucoma that was not contolled with medical treatment