Journal of Pediatric Ophthalmology & Strabismus vol:37 issue:1 pages:29-34
BACKGROUND: Sturge-Weber syndrome is a rare congenital neuro-oculocutaneous disorder. Ocular involvement can include glaucoma and vascular malformations of the conjunctiva, episclera, choroid, and retina. METHODS: The records of 19 Sturge-Weber syndrome patients (mean age 8.2 years) treated at our institution were reviewed to determine the incidence of ophthalmologic manifestations in Sturge-Weber syndrome. RESULTS: Glaucoma occurred in 42% of all patients and was more frequent in patients with a port-wine stain involving both upper and lower eyelids. Other ocular manifestations included conjunctival/episcleral hemangioma, choroidal hemangioma, iris heterochromia, retinal detachment, strabismus, and homonymous hemianopia. In 7 of 8 patients with glaucoma, topical pharmacotherapy (beta-blockers and carbonic anhydrase inhibitors) alone failed to normalize intraocular pressures. In those patients, cryocoagulation of the ciliary body was performed. Mean postoperative intraocular pressure after a mean follow-up of 4-5 years was <22 mm Hg in 6 patients. CONCLUSIONS: Cryocoagulation of the ciliary body combined with topical medication is an effective and safe treatment option in the management of glaucoma in children with Sturge-Weber syndrome.