Clinical presentation, diagnosis, and treatment of glaucoma associated with Sturge–Weber syndrome

BACKGROUND: The prevalence of glaucoma in Sturge–Weber syndrome ranges from 30% to 71%. AIM: The work aimed to study the clinical presentation and surgical outcomes of glaucoma in children with Sturge–Weber syndrome. METHODS: The study analyzed treatment outcomes of 34 patients (42 eyes) with glaucoma associated with Sturge–Weber syndrome. The obtained data included age, intraocular pressure, anterior-posterior axis, corneal diameter, cupping of optic discs, drug and surgical treatment. RESULTS: Age of patients at glaucoma onset was 1.8±0.5 years; corneal diameter was 12.4±0.1 mm, which exceeded the normal age range by 22.1%. The eyeball diameter exceeded the normal age range by 17.5%. Glaucoma was stabilized with drug therapy in 13 (31%) eyes. A total of 56 procedures were performed in 29 eyes, with an average of 1.93 per eye. One procedure was sufficient to compensate glaucoma in 14 (50%) eyes. An analysis of the hypotensive effect of the performed procedures showed that trabeculectomy was the most effective. The hypotensive effect was maintained in 76.2% and 50.7% of patients 1 and 5 years postoperatively, respectively. CONCLUSION: Glaucoma associated with Sturge–Weber syndrome had the same clinical presentation as primary congenital glaucoma and manifested in 69% of children under 1 year of age. Corneal and eyeball diameters were increased by an average of 22.1% and 17.4%, respectively. Surgery was required in 2/3 of cases. The most effective procedure was trabeculectomy.