Treatment of glaucoma in children with Sturge-Weber syndrome

Background: Sturge-Weber syndrome is a rare congenital neuro-oculocutaneous disorder. Ocular involvement can include glaucoma and vascular malformatio ns of the conjunctiva, episclera, choroid, and retina. Methods: The records of 19 Sturge-Weber syndrome patients (mean age 8.2 yea rs) 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. Oth er ocular manifestations included conjunctival/episcleral hemangioma, choro idal hemangioma, iris heterochromia, retinal detachment, strabismus, and ho monymous hemianopia. In 7 of 8 patients with glaucoma, topical pharmacother apy (beta-blockers and carbonic anhydrase inhibitors) alone failed to norma lize intraocular pressures. In those patients, cryocoagulation of the cilia ry 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 medi cation is an effective and safe treatment option in the management of glauc oma in children with Sturge-Weber syndrome.