Outcome of anterior-segment surgery in Rieger's anomaly

Rieger's anomaly is characterized by a dysgenesis of the anterior ocular segment with peripheral iris strands, an abnormally prominent Schwalbe's line, and a stromal atrophy of the iris. Refractory secondary glaucoma frequently demands surgical intervention with an uncertain prognosis. The charts of 15 patients with Rieger's anomaly (11 male, 4 female; mean age 21 years) were reviewed with regard to surgical management and postoperative intraocular pressure (IOP). Micro-/sclerocornea, keratoglobus, and keratopathy, micro-/buphthalmia, glaucoma, cataract, spherophakia, retinal detachment, papillary drusen, strabismus, and amblyopia were found associated with Rieger's anomaly. Secondary glaucoma required glaucoma surgery in 12 patients. Trabeculectomy (with or without mitomycin) and cyclodestructive surgery (cyclocryotherapy or cyclophotocoagulation) yielded IOP regulation in 50% of the treated eyes at a follow-up of 1 year. Cyclodestructive interventions displayed a very limited prognosis in eyes with preoperative maximal IOP values of > 45 mmHg. An obvious decline in visual acuity was observed in all patients, depending on the duration of glaucoma.