Clinical analysis on sixteen cases of Sturge-Weber syndrome-associated glaucoma

Abstract

To investigate the clinical features of Sturge-Weber syndrome-associated glaucoma and its surgical treatment. A retrospective case series study. The general clinical data and related ocular manifestations in 16 patients (21 eyes) with Sturge-Weber syndrome-associated glaucoma in our hospital from January 2003 to December 2007 were analyzed retrospectively. Age of the patients ranged between 1 month to 31 years old, and the median age was 11 years. Bilateral facial angiomas were present in 8 patients, and two of them had extensive hemangioma. Eleven cases had unilateral glaucoma and 3 of them had bilateral facial angiomas. Five patients had bilateral glaucoma and all of them had bilateral facial angiomas. Open anterior chamber angle was found in all affected eyes by gonioscopy. B-scan ultrasonography was performed in 21 eyes and diffused occupying lesions in the choroid were found in 8 eyes (38.1%). Posterior bowing of the iris, low echo in ciliary body and shallow ciliary body detachment were found by ultrasound biomicroscopy. Anti-glaucoma surgeries including trabeculotomy, trabeculectomy, non-penetrating deep sclerectomy, valve implantation and cyclocryotherapy were performed in 18 eyes. Eighty percent of the eyes which underwent trabeculectomy developed choroidal detachment after operation. Ten patients (11 eyes) were followed-up for 22 months on average. Intraocular pressure was significantly lower than that before the operation (t = 5.3956, P < 0.01). Intraocular pressure in all followed-up eyes was controlled at < or = 21 mm Hg (1 mm Hg = 0.133 kPa). The clinical features of Sturge-Weber syndrome-associated glaucoma include facial angiomas, open anterior chamber angle and choroidal hemangioma. Anti-glaucoma surgery can reduce the intraocular pressure effectively.