Sturge-Weber Syndrome with Osteohypertrophy of Maxilla

Table 1

Clinical manifestations associated with Sturge-Weber syndrome [1–3, 14–20].

Sl. no	Affected area	Features
1	CNS, cranium	Mental retardation 50%, convulsion 80%, hemiplegia, hemiparesis 30%, leptomeningeal angioma, gyriform calcification (tram line), neuronal loss, cerebral cortex atrophy, cerebral ischemia, and headache
2	Development	Developmental delay
3	Eye	Glaucoma 70%, coloboma of the iris, choroidal hemangioma, buphthalmos, hemianopia, dilated blood vessels, and visual loss
4	Oral cavity	Oral manifestation 40%, port wine stain involving gingiva, buccal mucosa, palate, and floor of mouth and tongue, macroglossia, gingival hyperplasia, bleeding gums, gingival hemangioma, periodontitis, pulpal involvement, osteohypertrophy (rarely), and pyogenic granuloma
5	Skin, face	Unilateral port wine stain involving areas supplied by ophthalmic and maxillary nerves that is, check, lip, and neck.
6	Other extra oral involvement	Port wine stains on neck, chest, abdomen, back, trunk, and extremities