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September 1994

Case 2

Author Affiliations

From the Department of Pediatrics, Inonu University School of Medicine, Malatya, Turkey (Dr Arisoy), and The Children's Hospital of Philadelphia (Pa) (Dr Tunnessen).

Arch Pediatr Adolesc Med. 1994;148(9):955-956. doi:10.1001/archpedi.1994.02170090069012

A 4-YEAR-OLD girl with a pink birthmark covering the right side of her face (Figure 1 and Figure 2) developed recurrent seizures. A plain roentgenogram of the skull (Figure 3) was obtained to support the suspected diagnosis.

Denouement and Discussion 

Sturge-Weber Syndrome  The most common vascular lesion associated with the Sturge-Weber syndrome is a port-wine stain or nevus flammeus, a vascular malformation present at birth and consisting of ectatic capillary to venular-sized blood vessels in the dermis. The port-wine stain does not resolve over time, unlike capillary hemangiomas. There may be associated blood vessel abnormalities involving the eye on the same side of the face, which may subsequently lead to the development of glaucoma. Leptomeningeal angiomatosis on the ipsilateral surface of the cerebral hemisphere is the other major component of the Sturge-Weber syndrome, and this malformation is responsible for the development of seizures and