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 15.86±10.11 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.