Aniridic Glaucoma: Long-term Visual Outcomes and Phenotypic Associations

Purpose:The aim of this study was to evaluate long-term visual outcomes in patients with aniridic glaucoma.Design:Retrospective noncomparative observational case series.Materials and Methods:A review of medical records of 128 eyes of 64 consecutive patients with aniridic glaucoma, diagnosed after the age of 5 years was analyzed. The parameters studied included age at presentation, family history, baseline intraocular pressure (IOP), type and the number of surgical interventions, and associated comorbidities.Primary Outcome Measure:Best corrected visual acuity (BCVA) in the better eye.Results:Mean age at presentation was 15.8610.11 years (range, 5 to 47 y). The average follow-up was 7.69 +/- 4.98 years (range, 1 to 17 y). At the final follow up only 18 patients had BCVA better than 6\60 and only 5 patients had BCVA of >6/18. Seventeen of the 64 (26.5%) patients developed phthisis in 1 eye. The final visual outcomes were not associated with age at presentation (P=0.64) or sex (P=0.76); however, those with a higher baseline IOP (P=0.017), those with familial aniridia (P=0.037), and those who underwent more number of surgical interventions had poorer visual outcomes (P=0.004). Kaplan-Meier analysis demonstrated the probability of bilateral blindness to be 69.8% and 97.6% at 5 and 10 years, respectively.Conclusions:Long-term visual outcomes after therapy among aniridic glaucoma patients remain poor. Higher baseline IOP, the presence of familial aniridia, and a history of greater number of surgical interventions are associated with poorer long-term visual outcomes.