An interesting case of nevus flammeus with loss of vision and hemiparesis

Abstract

Nevus flammeus is the most common benign congenital capillary malformation, often known as a port-wine stain. Sturge–Weber syndrome (SWS) is a congenital, sporadic, nonfamilial disease characterized by intracranial and ophthalmic vascular anomalies and nevus flammeus. It usually manifests as developmental delay, learning problems, paralysis, seizures, glaucoma and attention deficit, and hyperactivity disorder. A 29-year-old male patient presented with a reddish patch over the face since birth. He was found to have hemihypertrophy of face, hemiparesis of right limbs, and low intelligence quotient. On ophthalmic examination, the patient was found to have glaucoma and only perception of light in the left eye. Computed tomography brain showed atrophy of the left cerebral hemisphere and calcifications in the left frontal, parietal and occipital regions. With these findings, he was diagnosed as SWS type I. A multidisciplinary approach was followed for patient evaluation and management. This case also highlights the irreversible sequelae of this rare phacomatosis.

Sturge Weber Syndrome Roach Type I: A case report

“…10 Initially it is deep red but darkens with increasing age and may bleed on trauma. 11 Some cases present to the dentistry facilities with bleeding from gingival angiomas, and some have angiomas in the lips, palate, or beneath the tongue. The involvement of structures in the oral cavity imposes increased risk but is less frequently encountered.…”

“…Cerebral angiography is not a routine test for SWS but can be of help to exclude other forms of congenital vascular abnormalities in non-classical cases. 10,11,12…”