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 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.
van Emelen
C
C
Department of Ophthalmology, University Hospitals Leuven, Katholieke Universiteit, Belgium.
Goethals
M
M
Dralands
L
L
Casteels
I
I
D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000008:administration & dosage / Q000627:therapeutic use*; D000328:Adult; D002257:Carbonic Anhydrase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002924:Ciliary Body / Q000601:surgery*; D003452:Cryosurgery; D004359:Drug Therapy, Combination; D005128:Eye Diseases / Q000209:etiology; D005260:Female; D005901:Glaucoma / Q000209:etiology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007429:Intraocular Pressure; D008297:Male; D012189:Retrospective Studies; D013341:Sturge-Weber Syndrome / Q000150:complications*
D000319:Adrenergic beta-Antagonists; D002257:Carbonic Anhydrase Inhibitors