Ocular and systemic findings in a survey of aniridia subjects.

PURPOSE: To determine the prevalence of ocular and systemic abnormalities in a group of subjects with aniridia. METHODS: Survey forms developed by Aniridia Foundation International were sent to all members prior to the 2010 AFI member conference. An additional form was provided for completion by physicians caring for patients. Forms were then collected from all members who attended the meeting. RESULTS: A total of 155 surveys were distributed, of which 83 (53%) were completed. The mean age was 25.4 ± 18.4 years, with 65% sporadic and 35% familial cases, and 2.4% with WAGR (Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation) syndrome. Ocular abnormalities included nystagmus (83%), cataract (71%), dry eye (53%), glaucoma (46%), keratopathy (45%), foveal hypoplasia (41%), strabismus (31%), and retinal disease (5%). The mean age at diagnosis of aniridia was 22.1 months (median, 1.5 months) and glaucoma was 13.6 years (median, 8.5 years). Of 38 subjects with aniridia and glaucoma, 76% were treated medically, and 58% had been treated surgically. In subjects with glaucoma, the mean number (± SD) of glaucoma medications was 1.8 ± 1.3, and number of surgical procedures was 1.7 ± 2.0. Developmental delay was reported in 17%. The mean body mass index and the prevalence of obesity in subjects with aniridia was significantly greater (P = 0.003) than in siblings without aniridia. CONCLUSIONS: In this study, aniridia was associated with nystagmus and other motility problems, cataract, glaucoma, and keratopathy. Systemic abnormalities included increased average body mass index and obesity, which appeared to occur not only in WAGR syndrome but more broadly in aniridia.

The Ahmed Glaucoma Valve in patients with and without neovascular glaucoma.

PURPOSE: To evaluate the results of Ahmed Glaucoma Valve surgery in neovascular glaucoma and control patients. PATIENTS AND METHODS: In this retrospective comparative study, we reviewed 76 eyes of 76 patients, comparing the surgical outcomes in control patients (N=38) to matched neovascular glaucoma patients (N=38). Success was defined as intraocular pressure (IOP) ≥6 mm Hg and ≤21 mm Hg, without further glaucoma surgery, and without loss of light perception. RESULTS: Average follow-up for control and neovascular glaucoma patients was 18.4 and 17.4 months, respectively (P=0.550). The mean IOPs were 17.9±8.4 mm Hg and 16.5±15.8 mm Hg at 1-year (P=0.150), and 18.4±11.2 mm Hg and 9.9±6.3 mm Hg at 2 years (P=0.057) in controls and eyes with neovascular glaucoma, respectively. Life-table analysis showed a significantly lower success for neovascular glaucoma patients compared with controls (P=0.0096), with success at 1 year=89.2% and 73.1%, 2 years=81.8% and 61.9%, and 5 years=81.8% and 20.6% for control and neovascular glaucoma eyes, respectively. Cox proportional hazard regression analysis showed neovascular glaucoma as a risk factor for surgical failure (odds ratio=5.384, 95% CI=1.22-23.84, P=0.027). Although IOP control and complications were comparable between the 2 groups, visual outcomes were worse in neovascular glaucoma patients, with 9 (23.7%) eyes with neovascular glaucoma compared with no controls losing light perception vision (P=0.002). The majority with loss of vision (5 of 9) had successful control of IOP during the postoperative period. CONCLUSIONS: Neovascular glaucoma patients have greater risk of surgical failure after Ahmed Glaucoma Valve surgery compared with controls. Despite improved IOP with drainage implants, visual outcomes may be poor, probably owing to progression of underlying disease.