Sturge Weber Syndrome on Computed Tomography: A Rare Case Report and Review of Imaging

Abstract

Sturge-Weber syndrome (SWS), also called encephalotrigeminal angiomatosis, is a neurocutaneous disorder with angiomas that involve the leptomeninges and the skin of the face. It is a rare congenital non-hereditary disorder with incidence of 1: 20,000 – 50,000. Both sexes are affected equally. Presentation is usually in childhood. Imaging is the most useful investigation in evaluation of the disease. A 28-year woman was referred for brain CT. She presented to the maxillo-facial unit with complain of right facial swelling and discoloration since birth. There was history of developmental delay, recurrent headache and weakness of the left upper and lower limbs. There were occasional episodes of seizures. No history of loss of vision. On examination, significant findings were port-wine stain, swelling on the right side of the face and reduce power on the left upper and lower limbs. Cranial CT images revealed loss of right cerebral volume with extensive tram track calcifications involving predominantly the right parietal and occipital lobes a diagnosis of SWS was made. Patient is currently on medical management.