Outcome of Glaucoma Management in Sturge-Weber Syndrome: Case Series

Abstract

Objective: To report the clinical findings and outcome of treatment for developmental glaucoma from Sturge-Weber syndrome in four consecutive children managed at a child eye health tertiary facility in Ibadan, Nigeria. Methods: A retrospective case series of four children with Sturge-Weber syndrome (SWS) and developmental glaucoma who underwent surgical treatment for glaucoma over a 3-year period. Information on patients’ sociodemographic data, clinical features, and management are described. Results: Four male children, aged between 7 weeks and 11 years, with unilateral developmental glaucoma and varying extent of port-wine stain were included in the study. The primary surgery in three patients was trabeculectomy, while the fourth patient received a glaucoma drainage device. Shallow anterior chamber, choroidal effusion, and cataract were complications of glaucoma surgery observed. Secondary surgeries performed include anterior chamber reformation, suturing of leaking peritubular scleral channel, sclerotomy and fluid drainage, bleb needling, cataract surgery, and scleral buckle surgery. At the 1-year follow-up visit, one eye had complete success, one eye had qualified success, while the other two had treatment failure. Conclusion: The outcome of management of glaucoma associated with Sturge-Weber syndrome is modest. Though rare, SWS can cause significant ocular and systemic morbidity. Complications of therapy may be encountered, and multiple surgical interventions may be required for optimal management.