Sturge-Weber Syndrome in Patients with Facial Port Wine Stain

Port wine stains (PWSs) are vascular malformations seen in 0.3% of newborns. Sturge-Weber syndrome (SWS) is a congenital neurocutaneous condition defined by facial PWS and at least one of the following abnormalities: leptomeningeal vascular anomalies, choroid plexus enlargement, cortical calcifications, cerebral atrophy, absence of superficial venous drainage, or enlarged deep hemispheric vessels. Associated seizures may induce neurologic defects such as mental retardation or hemiparesis.

To determine whether certain symptoms and cutaneous PWS distribution are associated with SWS, investigators compared 259 consecutive PWS patients with and without SWS over a 12-year period (186 younger than 15 years; median age, 8 years; 51% female). SWS wa…