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

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 two consecutive follow-up visits after 3 months, IOP ≤5 mm Hg on two 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 early onset group and 17 eyes in late onset group. The early onset group more frequently presented with buphthalmos, corneal edema and Haab's 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 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). CONCLUSION:Early and late onset SWS glaucoma may represent two entities with different pathogenetic mechanisms, clinical presentations, primary surgical choices and outcomes, though this needs corroboration in future studies.