Outcomes of primary combined trabeculotomy and trabeculectomy in early onset glaucoma in children with congenital aniridia.

PURPOSE To report clinical features and treatment outcomes of primary combined trabeculotomy with trabeculectomy (CTT) in congenital aniridia with glaucoma in children aged 3 years or less. DESIGN Retrospective study PARTICIPANTS: Sixty-six eyes (35 children) with congenital aniridia and early onset glaucoma who underwent CTT between May-1997 and June-2015 were included. METHODS Success was defined as complete when intraocular pressure (IOP) was >5 mm Hg and ≤21 or ≤16 mm Hg without antiglaucoma medications (AGMs), and qualified when AGMs were required. Eyes needing repeat surgery for IOP control or loss of light perception were considered failures. MAIN OUTCOME MEASURE IOP control and number of AGMs RESULTS: The median age at presentation of 35 children was 26 days (interquartile range (IQR):7, 106 days). There were 22 males (62.9%) and 13 females (37.1%). Parental consanguinity was noted in 11 children (31.4%). Aniridia was total in 52 and partial in 14 eyes. At presentation, there was corneal edema in 30 eyes (45.4%), enlarged corneal diameter in 32 eyes (48.5%), nystagmus in 15 children (23%), limbal stem cell deficiency in 1 eye, cataract in 9 eyes (13.6%), retino-choroidal coloboma in 1 eye and foveal hypoplasia in 4 eyes. Post-CTT, median IOP significantly reduced (p<0.0001), from 28 (IQR:24, 30) to 16 (IQR:14, 22) mmHg. Corneal edema cleared in 12/30 eyes, and cleared with scar in 18/30 eyes, of which 6 underwent keratoplasty later. Median follow-up was 5.33 (IQR:3.05, 7.59) years. At latest follow-up, median number of AGMs were 1 (IQR:0, 2) and median Log MAR visual acuity (n=34 eyes) was 1.35 (IQR:0.80, 2.78). The probability of complete success for IOP≤ 21 mmHg was 79.9% at 1 year and 62.3% between 2-10 years; for IOP≤16 mmHg, it was 78.6% at 1 year and decreased to 51.9% at 10 years. Qualified success for IOP ≤21 mmHg at 1 year was 82.6% and 81% over 10 years; for IOP ≤16 mmHg, it was 81.4% at 1 year and decreased to 63.1% at 10 years. Seven eyes (10.6%) required repeat intervention for IOP control with median time of 3.17 (IQR:0.92, 6.56) years from first surgery. CONCLUSION Primary combined trabeculotomy with trabeculectomy had good success rate in congenital aniridia children with early onset glaucoma.