Sturge-Weber syndrome coexisting with multiple vertebral vascular malformations and hemivertebra with scoliosis and upper limb and ear hypertrophy

Histopathological examination of biopsy from the port-wine stain and nodule showed scattered dilated blood vessels with blood elements and endothelial proliferation in the dermis and fibro-collagenous tissue with adnexal structures in the intervening stroma, suggestive of cavernous hemangioma [Figure 6] and [Figure 7]. [1] The most common manifestation of this syndrome is a port-wine stain on the face, reported in 87–90%; typically located near or around an eye or on the forehead, and varies in size and color. Most patients suffer from seizures before one year of age; seen in roughly 75% of patients with unilateral brain involvement, and 95% with bilateral brain involvement. [...]8–20% of patients with port-wine stain are known to suffer from neurological symptoms and the risk is about 35% when extensive bilateral facial port-wine stains are present.