Treatment of glaucoma in children with Sturge-Weber syndrome

J Pediatr Ophthalmol Strabismus. Jan-Feb 2000;37(1):29-34.

Abstract

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.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adrenergic beta-Antagonists / administration & dosage
  • Adrenergic beta-Antagonists / therapeutic use*
  • Adult
  • Carbonic Anhydrase Inhibitors / administration & dosage
  • Carbonic Anhydrase Inhibitors / therapeutic use*
  • Child
  • Child, Preschool
  • Ciliary Body / surgery*
  • Cryosurgery*
  • Drug Therapy, Combination
  • Eye Diseases / etiology
  • Female
  • Glaucoma / etiology
  • Glaucoma / surgery*
  • Humans
  • Infant
  • Intraocular Pressure
  • Male
  • Retrospective Studies
  • Sturge-Weber Syndrome / complications*

Substances

  • Adrenergic beta-Antagonists
  • Carbonic Anhydrase Inhibitors