Coexisting conditions can alter aniridia approach

Aniridia refers to partial or total lack of iris tissue. This generally is hereditary when it is bilateral and associated with other ocular defects. It may also occur after trauma or as an intraoperative complication.Congenital aniridia is rare and has an incidence of one per 50,000 to 100,000 live births. About two-thirds of cases are familial whereas one-third of cases result from a new genetic mutation in the PAX6 gene, occur without a family history and have a 30% chance of developing Wilms tumor. Serial renal ultrasound is required to rule out Wilms tumor, especially in patients with no family history. Aniridia may occur as a chromosome deletion of the short arm of chromosome 11 or as sporadic.