Complications and visual prognosis in children with aniridia

To characterize the ophthalmological findings, assess surgical outcomes, and review visual outcomes in aniridia.

A retrospective case review was performed and data were collected, including patient demographics, incidence of aniridia-associated keratopathy, glaucoma, cataract, retinal breaks or detachments, optic nerve hypoplasia, macular hypoplasia, poor vision, and nystagmus. All outcomes from surgery, including penetrating keratoplasty, trabeculectomy, Ahmed valve insertion, and cataract extraction, were recorded.

Six children (12 eyes) had corneal abnormalities, 4 had optic nerve hypoplasia, 9 had nystagmus, and 2 had retinal detachments. Four patients (7 eyes) required penetrating keratoplasty. Five patients (9 eyes) developed glaucoma and only 1 of the 4 trabeculectomies performed succeeded. Of the 6 Ahmed valve procedures performed, all succeeded in maintaining a satisfactory intraocular pressure but some required needling and 5-fluorouracil. Eight patients developed cataract and 7 required surgery. Visual outcomes were poor despite treatment. Nine patients had Snellen acuity of 6/60 or less and required low visual aids to function.

Aniridia is a disorder that requires multiple surgeries. It has a poor visual prognosis despite early diagnosis and aggressive management. Newer techniques such as Ahmed valves and Boston keratoprostheses offer hope, but its proliferative nature makes treatment difficult.