RESUMO
INTRODUCTION: Aniridia, or congenital absence of the iris, is a rare and severe ocular abnormality. We report five cases diagnosed in our department over the last 5 years and describe our clinical findings and attitude. OBSERVATIONS: The patients were two adults and three children, 8-45 years old at the time of diagnosis. Visual acuity ranged from light perception to 20/50. Aniridia was associated with other abnormalities in four cases: lens ectopy in one case, ptosis in three cases, microcornea in one case, macular hypoplasia in three cases and a pterygium coli associated with a pectus excavatum in one case. All patients had complications: corneal dystrophy in three cases, lens opacities in five cases, elevated intraocular pressure (IOP) in two cases, severe amblyopia with nystagmus or strabismus in three cases. Only patients with glaucoma received IOP-lowering medications. IOP was normalized in both cases and regular controls were instituted for all the other patients. DISCUSSION: Management of aniridia is complex in our regions because patients often consult late, at a stage that involves complications and amblyopia. First-intention surgical treatment of glaucoma is not always justified. CONCLUSION: Only genetic counseling, a close follow-up after an early diagnosis and adapted surgical techniques can improve the visual prognosis of aniridia patients.
