Clinical Features and Surgical Outcomes of Sturge-Weber Syndrome with Glaucoma

ABSTRACT

PURPOSE: To report clinical manifestations including neurocutaneous and ocular findings and to evaluate outcomes of trabeculectomy in patients with Sturge-Weber syndrome. METHODS: The medical records of 10 eyes of 8 glaucoma patients with Sturge-Weber syndrome who were followed up for at least 1 year after trabeculectomy were reviewed retrospectively. We analyzed neurocutaneous and ocular findings, cumulative surgical success rates, and complications in patients with Sturge-Weber syndrome. RESULTS: The mean patient age at the time of surgery was 12.6 +/- 13.0 years and mean follow-up period was 71.6 +/- 81.8 months. All patients showed various clinical findings including facial hemangioma (8 patients), seizure (6 patients), intracranial lesion (6 patients), developmental delay (4 patients), conjunctival/episcleral hemangioma (4 eyes), and choroidal hemangioma (4 eyes). Postoperative success was achieved in 8 out of 10 eyes (80.0%). Postoperatively, serous retinal detachment occurred in 2 out of 4 eyes with preoperative diffuse choroidal hemangioma. CONCLUSIONS: Management of glaucoma associated with Sturge-Weber syndrome requires multidisciplinary treatment because of systemic involvement. Trabeculectomy appears to be an effective and relatively safe surgical option for glaucoma associated with Sturge-Weber syndrome. However, serious complications such as serous retinal detachment should be considered when planning trabeculectomy for patients with diffuse choroidal hemangioma.