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April 1984

Picture of the Month

Author Affiliations

Contributed from the Tufts—New England Medical Center, Boston Floating Hospital, 171 Harrison Ave, Boston, MA 02111.

Am J Dis Child. 1984;138(4):407-408. doi:10.1001/archpedi.1984.02140420073022

Denouement and Discussion 

Sturge-Weber Syndrome 

Manifestations  Major manifestations involve the skin, brain, and eyes. A pink to purplish nonraised hemangioma (port-wine stain or nevus flammeus) is present on the face and, frequently, has a trigeminal cranial nerve distribution. The port-wine staining of the skin can be located on any part of the body, and these areas, especially the gingiva and alveolar ridges, may be hypertrophic. Glaucoma and buphthalmos commonly occur on the affected side.Central nervous system abnormalities include angiomas that usually involve the arachnoid and pia mater, cortical atrophy, cerebral calcifications, and sclerosis. The double-contoured parallel calcifications usually appear after 2 years of age. Seizures vary in severity and, on occasion, may be uncontrollable. Hemiparesis may occur contralateral to the facial nevus, and mental retardation is a frequent finding.

Genetics  Sturge-Weber syndrome does not seem to be a genetic disease, and recurrence is unlikely.

Treatment  It is difficult to