At what age do the signs and symptoms of Sturge-Weber syndrome (SWS) typically present?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

The typical patient presents at birth with facial angiomas; however, not all children with facial angiomas and PWS have SWS, which raises certain diagnostic and prognostic concerns. [32]

In incomplete SWS (type III, Roach Scale), CNS angiomas occur without cutaneous features; therefore, no suspicion of SWS arises until a seizure or other neurologic problem develops. Thus, the diagnosis of SWS is not always straightforward.

Secondary glaucoma may present at any age, although early onset is the rule, with approximately 60% of glaucomas presenting at birth or in early infancy and another 30% presenting during childhood. The median ages reported for onset of visual symptoms related to secondary retinal changes range from age 8-20 years.


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