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

Park, Jae Hwi; Lim, Su Ho; Cha, Soon Cheol

doi:2013.54.11.1737

J Korean Ophthalmol Soc > Volume 54(11); 2013 > Article

Original Article

Journal of the Korean Ophthalmological Society 2013;54(11):1737-1747.
DOI: https://doi.org/10.3341/jkos.2013.54.11.1737 Published online November 15, 2013.

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

Jae Hwi Park, Su Ho Lim, Soon Cheol Cha

Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Korea. sccha@yumail.ac.kr

녹내장이 발생한 Sturge-Weber 증후군 환자의 임상양상과 수술성적

박재휘⋅임수호⋅차순철

Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Korea

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.

Key Words: Glaucoma;Neurocutaneous findings;Sturge-Weber syndrome;Trabeculectomy