INTRODUCTION:
Aniridia is defined as Partial or complete absence of the iris. It may be associated with systemic disorders and ocular disorders. It presents as congenital aniridia which is rare and may be acquired after trauma or ocular surgery. The most common variant of congenital aniridia is a de novo mutation of the PAX6 gene. Presentation may be by poor vision since childhood. Nystagmus and strabismus are common. Iris may be complete absence. Signs of keratopathy can appear in first decade of life. The anterior chamber is open on gonioscopy, and congenital glaucoma/buphthalmos is rare in aniridia. However, within the 2nd -decade angle closure due to peripheral anterior synechia may lead to an increase in the intraocular pressure. Gonioscopy may show iris fibers which lie at the space between the rudimentary small hypoplastic iris and the trabecular meshwork. The lens in aniridia may show superior sublaxation (clear or cataractous), ectopialentis (noted in up to 56% of cases). Fovealhypoplasia are also present.
AIM OF THE WORK:
The study aim was to report on the ophthalmic eye features with aniridia in children presenting to Alexandria Main University Hospital from 2005 to 2020.