Surgical Outcomes of Early Versus Late Onset Glaucoma Associated with Sturge-Weber Syndrome

Précis: Long-term intraocular pressure control can be difficult to achieve in eyes with Sturge-Weber syndrome glaucoma. The most commonly performed primary surgery was trabeculotomyin early onset disease and tube shunt implantation in late onset disease. Purpose: To compare long-term surgical outcomes of glaucoma associated with Sturge-Weber syndrome (SWS) in eyes with early and late-onset disease. Methods: Medical records of children with glaucoma associated with SWS who underwent surgical treatment between January 1990 and December 2018 were reviewed. Those diagnosed ≤2 years of age were categorized as early onset while those who were diagnosed >2 years of age were late onset. Failure was defined as intraocular pressure (IOP) >21 mm Hg or reduced <20% below baseline on 2 consecutive follow-up visits after 3 months, IOP ≤5 mm Hg on 2 consecutive follow-up visits, reoperation for glaucoma or a complication, or loss of light perception. Results: Forty-three eyes of 36 children were studied, including 26 eyes in the early-onset group and 17 eyes in the late-onset group. The early-onset group more frequently presented with buphthalmos, corneal edema, and Haab striae, while late-onset group had higher baseline IOP, larger cup-to-disc ratio, and longer axial length. The most commonly performed primary surgery was trabeculotomy (50%) in early-onset group and tube shunt implantation (71%) in late-onset group. The cumulative probability of failure after 5 years follow-up was 50.6% in early-onset group and 50.9% in the late-onset group ( P =0.56). Postoperative complications occurred in 3 eyes (12%) in early-onset group and 11 eyes (65%) in late-onset group ( P <0.001). Conclusions: Early and late-onset SWS glaucoma may represent 2 entities with different pathogenetic mechanisms, clinical presentations, primary surgical choices, and outcomes, though this needs corroboration in future studies.