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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 the authors' institution were reviewed to determine the incidence of ophthalmological 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 the upper and lower eyelids. Other ocular manifestations included conjunctival/episcleral hemangioma, choroidal hemangioma, iris heterochromia, retinal detachment, strabismus, and homonymous hemianopia. In seven of eight 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 four to five years was < 22 mmHg in six 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.
Dr. I. Casteels, Department of Ophthalmology, University Hospital St Raphael, Kapucijnenvoer 33, B-3000 Leuven, Belgium
9.4.9 Glaucomas associated with elevated episcleral venous pressure (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
12.10 Cyclodestruction (Part of: 12 Surgical treatment)