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 3 years of age or younger. DESIGN: Retrospective study. PARTICIPANTS: Sixty-six eyes of 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 more than 5 mmHg and less than or equal to 21 or 16 mmHg without antiglaucoma medications (AGMs), and qualified when AGMs were required. Eyes needing repeat surgery for IOP control were considered as failures. MAIN OUTCOME MEASURES: 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 eyes 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%), and foveal hypoplasia in 4 eyes. After CTT, median IOP was reduced significantly (P < 0.0001). Corneal edema cleared in 12/30 eyes and cleared with scar in 18/30 eyes. Median follow-up was 5.33 (IQR, 3.05-7.59) years. At latest follow-up, median number of AGMs was 1 (IQR, 0-2) and median visual acuity in logarithm of minimum angle of resolutions (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 and 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 years (IQR, 0.92-6.56) from first surgery. CONCLUSIONS: CTT showed good success in children with congenital aniridia with early-onset glaucoma.