Sturge-Weber Syndrome
Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, is a phakomatosis characterized by facial port wine stains and pial angiomas. It is part of a wide spectrum of possible phenotypes included in the craniofacial arteriovenous metameric syndrome (CAMS). Radiographic features: Plain radiograph: Skull radiographs were historically useful and capable of identifying the gyriform calcification of the subcortical white matter although they no longer play a significant role in the diagnosis or management of this condition. The finding usually becomes evident between 2 and 7 years of age 2. CT: detects subcortical calcification at an earlier age than plain film and can also demonstrate associated parenchymal volume loss tram-track sign of cortical and subcortical calcification 13,14 calvarial and regional sinus enlargement may be evident ipsilateral choroid plexus may be enlarged in severe cases, a Dyke-Davidoff-Masson appearance may be seen orbital choroidal hemangiomas may be present asymmetric cavernous sinus enlargement 22.