Objective: To assess the efficacy of goniotomy/trabeculotomy as the initial surgical procedure in early-onset glaucoma associated with Sturge-Weber syndrome.

Methods: We retrospectively analyzed 16 eyes of 14 consecutive patients with Sturge-Weber syndrome-associated glaucoma diagnosed before 4 years of age. All subjects were seen at a single institution from 1978 to 1996 and underwent goniotomy or trabeculotomy as their initial surgical procedure.

Results: Twelve eyes underwent initial goniotomy, and 4 eyes underwent initial trabeculotomy. One subject was lost to follow-up after surgery, resulting in 15 eyes for analysis. Of the initial goniotomy eyes, two thirds required a second surgical procedure. In the initial trabeculotomy eyes, half required a second procedure. Intraocular pressure was controlled (intraocular pressure < or = 22 mm Hg) in 66.7% of the eyes (10 of 15) after one or more goniotomy or trabeculotomy procedures for a median follow-up of 5.4 years (range, 1.4 to 15 years). For eyes with only one surgical procedure, 4 of 6 eyes had controlled intraocular pressure over a median follow-up of 3.4 years (range, 3 to 12 years). Seven of the 9 eyes that required more than one procedure had controlled intraocular pressure after all procedures over a median follow-up of 4.5 years (range, 1.4 to 15 years).

Conclusions: Initial or repeated goniotomy or trabeculotomy may be an effective management choice for treatment of glaucoma associated with Sturge-Weber syndrome presenting in early childhood.