Prevalence, Incidence, and Risk Factors for the Development of Glaucoma in Patients With Aniridia

Purpose: To assess the prevalence, incidence, and risk factors for the development of glaucoma in patients with aniridia.

Methods: Retrospective analysis of case records of patients diagnosed as having congenital aniridia between January 1986 and December 2011 was performed. Patients with a follow-up of more than 12 months were included.

Results: Ninety-one patients (180 eyes) with the diagnosis of aniridia were identified from the case records. Two eyes were excluded from the final analysis; one had developed phthisis and the other had been enucleated. Seventy-four patients (81.3%) were younger than 18 years at initial presentation. The prevalence of glaucoma at presentation was 28.8%, which could be further categorized as ocular hypertension in 19 eyes (10.5%) and glaucoma in 33 eyes (18.3%). Thirty-one eyes (28.4%) developed elevated intraocular pressure (IOP) during the follow-up period: ocular hypertension in 23 eyes (17.9%) and glaucoma in 8 eyes (6.25%). The mean IOP at the time of diagnosis was 33.9 ± 8.6 mm Hg (range: 24 to 60 mm Hg). The mean duration of follow-up was 8.1 ± 5.7 years (range: 1 to 28 years). The cumulative probability of developing elevated IOP was 4% at the end of 8 years of follow-up; this increased to 88% at the end of 28 years of follow-up. Univariate logistic regression analysis identified higher baseline IOP (odds ratio [OR]: 1.2; 95% confidence interval [CI]: 1.2 to 1.4) and limbal stem cell deficiency (OR: 2.8; 95% CI: 1.4 to 5.6) as significant risk factors for the development of elevated IOP. Higher baseline IOP remained significant on multivariate analysis (OR: 1.2; 95% CI: 1.2 to 1.4).

Conclusions: Glaucoma occurs in a substantial proportion of patients with aniridia. Eyes with increased IOP at baseline are at a higher risk. [J Pediatr Ophthalmol Strabismus. 2017;54(4):250-255.].