PurposeTo determine the long-term surgical and visual outcomes in Indian children with early-onset glaucoma associated with Axenfeld-Rieger anomaly (ARA).MethodsThis is a retrospective analysis of 44 eyes of 24 consecutive children with early-onset glaucoma (within 3 years of age) and ARA who underwent glaucoma surgery over a 20-year period (1991-2010) by a single surgeon. Main outcome measures were pre- and postoperative intraocular pressures (IOPs), corneal clarity, visual acuities (VAs), refractive errors, success rate, time of surgical failure, and complications.ResultsThe series consisted of 38 primary combined trabeculotomy-trabeculectomy (CTT) and 6 primary trabeculectomy procedures (Schlemm's canal could not be identified in these eyes). There was a statistically significant reduction in IOP postoperatively (27.07±4.88 vs 14.88±3.62 mm Hg; P<0.0001) with a mean reduction of 45.14%. Success probability by Kaplan-Meier survival analysis was 93% till 5 years, and then 88.1%, 82.3%, 70.5%, 56.4%, and 42.3% at year 6, 7, 8, 9, and 10, respectively. Preoperative corneal edema was present in 43/44 eyes (97.72%) and cleared in 42 eyes (97.67%). There was one case each with intraoperative hyphema and with shallow chamber postoperatively and both were successfully managed successfully. There was no incidence of endophthalmitis or any other sight-threatening complication. Data on VA were available in 34 eyes (77.3%). At final follow-up visit, 15 (44.1%) eyes had best corrected VA ⩾6/18.ConclusionsPrimary CTT is safe and effective for early-onset glaucoma associated with ARA. It leads to excellent IOP control and satisfactory visual outcome.