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.