Glaucoma in Sturge-Weber syndrome

Background: In glaucoma associated with Sturge-Weber syndrome (SWS), medica l treatment often fails to control intraocular pressure, thus requiring sur gical intervention that may result in serious complications. Methods: Eight een consecutive patients with SWS were reviewed retrospectively at the King Khaled Eye Specialist Hospital. An intraocular pressure less than 20 mm Hg , plus stable optic nerve cup-to-disc ratio and corneal diameter (or visual fields where appropriate), were parameters chosen to indicate that the gla ucoma was being controlled. Results: Glaucoma was found in 15 of 18 patient s (22 eyes). The mean follow-up time was 62 months (range, 12 to 148 months ). Medical treatment alone was successful in 5 patients (7 eyes); the remai nder required surgical intervention. The initial surgical procedures includ ed cyclocryotherapy, YAG laser goniotomy, surgical goniotomy, and trabeculo tomy or trabeculectomy. Eight eyes required subsequent surgery, 5 with Molt eno or Ahmed implants. Early postoperative choroidal effusion and hemorrhag e occurred in 4 eyes and resolved spontaneously. Hemorrhagic choroidal deta chment with total retinal detachment developed in 2 patients 3 to 5 months after surgery. In 1 patient a recurrent serous choroidal detachment after s uture lysis was associated with total optic atrophy. Conclusions: Glaucoma in SWS is common. Control of glaucoma was successfully achieved with medica l treatment in 7 of 22 eyes of our 15 SWS-glaucoma patients, and we conside r it the initial treatment of choice. Of the 15 eyes that required surgery, late postoperative complications resulted in loss of vision as a result of persistent postoperative hypotony in 3 eyes that underwent surgical proced ures.