A Rare Presentation of Orbital Cellulitis in a Child with Sturge-Weber Syndrome

Abstract

Sturge - Weber Syndrome (SWS) is an uncommon neuro-oculocutaneous disorder. It is, also known as encephalotrigeminal angiomatosis, which is characterized by angiomas involving the face, choroid, and leptomeninges. Vascular malformation in conjunctiva, choroid, episclera, retina leading to glaucoma is the common ocular manifestation. It is one of the phakomatoses and is often associated with ocular problem, seizures, intellectual disability, and angioma. Here we report a child who has orbital cellulitis of the left eye in a SWS patient. Rare disorder like SWS, is a congenital neurocutaneous disorder. It is characterised classically by facial capillary haemangioma that is the port wine stain (PWS), which involves the face, forehead and/or scalp along with a choroidal angioma and a venous angioma of the leptomeninges. The incidence of SWS is 1 in 20,000 - 50,000 live births. The systemic implications of SWS are vast and include ophthalmic manifestations, dermatologic, neurologic, and oral manifestations. 1-3 Here, we are presenting a rare case of Sturge - Weber Syndrome in an eight-year-old male child who presented with orbital cellulitis of the left eye.