What is the prevalence of glaucoma in Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Glaucoma typically occurs in SWS only when the PWS involves the eyelids. The incidence ranges from 30-71%. Glaucoma may be present at birth but can develop at any age, even in adults. [49, 50]

Treatment includes yearly examinations to look for optic nerve damage (with measurement of IOP and visual fields) and for corneal diameter and refractive changes in children.

Glaucoma usually occurs only with an ipsilateral facial PWS, although the glaucoma may be bilateral when facial involvement is bilateral. Contralateral glaucoma may develop, although rarely. Glaucoma also may occur without neurologic involvement (Type II, Roach Scale).

Sullivan et al reviewed ocular abnormalities in 51 patients with SWS. [51] Of these, 36 patients (71%) had glaucoma, with onset before age 24 months in 26 (51%) patients; 35 (69%) patients had conjunctival or episcleral hemangiomas; and 28 (55%) patients had choroidal hemangiomas.

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