Definition Sturge–Weber angiomatosis, or encephalotrigeminal angiomatosis, is a relatively rare, sporadic congenital capillary vascular malformation typically involving areas innervated by the trigeminal nerve.
Etiology Developmental abnormality.
Clinical features The main clinical features are characterized by unilateral hemangiomas of the facial skin, oral mucosa, and leptomeninges, brain calcification, ocular disorders, epilepsy, and occasionally mild mental handicap. Oral hemangiomas have a bright red or purple color and are usually flat but may also be raised, causing tissue enlargement. Dentists and oral surgeons must be careful during tooth extraction and periodontal surgery so as to avoid bleeding complications. Facial hemangiomas have a bright red color and are asymptomatic. Facial and oral hemangiomas are usually obvious at birth. The diagnosis is usually based on clinical criteria.
Differential diagnosis Solitary hemangioma, Klippel–Trénaunay–Weber syndrome.