Glaucoma in Sturge-Weber syndrome.

Background: In glaucoma associated with Sturge-Weber syndrome (SWS), medical treatment often fails to control intraocular pressure, thus requiring surgical intervention that may result in serious complications.

Methods: Eighteen 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 glaucoma was being controlled.

Results: Glaucoma was found in 15 of 18 patients (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 remainder required surgical intervention. The initial surgical procedures included cyclocryotherapy, YAG laser goniotomy, surgical goniotomy, and trabeculotomy or trabeculectomy. Eight eyes required subsequent surgery, 5 with Molteno or Ahmed implants. Early postoperative choroidal effusion and hemorrhage occurred in 4 eyes and resolved spontaneously. Hemorrhagic choroidal detachment with total retinal detachment developed in 2 patients 3 to 5 months after surgery. In 1 patient a recurrent serous choroidal detachment after suture lysis was associated with total optic atrophy.

Conclusions: Glaucoma in SWS is common. Control of glaucoma was successfully achieved with medical treatment in 7 of 22 eyes of our 15 SWS-glaucoma patients, and we consider 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 procedures.