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.].