Treatment Outcomes of Primary Combined Trabeculotomy With Trabeculectomy in Early Onset Glaucoma With Sturge-Weber Syndrome

Abstract

Précis: In eyes with Sturge-Weber syndrome (SWS) with early onset glaucoma, primary combined trabeculotomy with trabeculectomy (CTT) had a good intermediate-term success rate and minimal postoperative complications. Design: Retrospective cohort study. Objective: To report the intermediate-term outcomes of primary CTT in early onset glaucoma with SWS. Patients and Methods: This study included 49 eyes of 49 children (1997–2020) with SWS and early onset glaucoma who underwent primary CTT with at least 1-year postoperative follow-up. Success was defined as complete when intraocular pressure was >5 mm Hg and ≤16 mm Hg under general anesthesia or ≤21 mm Hg in the clinic without antiglaucoma medications (AGMs) and as qualified with AGM. Results: The median age (interquartile range) at CTT was 0.58 (0.19, 8.3) years. The median postoperative follow-up was 4.7 years (2.5, 9). The majority were male children (31/49, 63%). Epilepsy was noted in 7 (14.3%) and diffuse choroidal hemangioma in 17 children (35%). At diagnosis, 37 eyes(75%), had corneal edema, and the mean (±SD) horizontal corneal diameter was 12.8 ± 0.7 mm. Postoperatively, the median intraocular pressure decreased from 26 (22, 30) mm Hg to 16 (12, 20) mm Hg (P < 0.0001) and the median number of AGM reduced from 1 (0,1) to 0 (0,1; P < 0.01). Complete success probability of CTT was 86% (76, 96) at 1 year and 64% (49, 84) at 5 years. Qualified success was 98% (94, 100) at 1 year and 89% (78, 100) at 5 years. A larger cup-to-disk ratio (P < 0.005) was associated with a higher risk of surgical failure. Postoperative complications were noted in 9 eyes (18%), all resolved with conservative management except one eye with a retinal detachment that ended in phthisis bulbi. Conclusion: CTT as a primary procedure showed good long-term efficacy and safety in SWS with early onset glaucoma.