Encephalotrigeminal angiomatosis: A Case Report and Review

Sturge Weber Syndrome is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities (glaucoma and choroidal hemangioma) and leptomeningeal angioma. Port-wine stain and abnormal findings like soft tissue calcification or bony changes in skull radiographs, mental retardation, ocular involvement and hemiplegia. Oral manifestations of the disease may vary considerably and changes in morphology and histology of gingiva, periodontium and pulp have been reported. However the most common feature is a gingival hemangiomatous lesion usually restricted to ipsilateral maxilla, mandible, floor of mouth, lips, cheeks, palate and tongue.