Type I Sturge-Weber Syndrome with Bilateral Megalocornea in a Three Years Old Girl

Sturge-Weber syndrome (SWS) is a sporadic condition characterized by presence of port wine vascular nevus (PWS) on the upper part of the face and ipsilateral leptomeningeal angioma. The incidence estimated at 1/50, 000 persons. Common symptoms are seizures, hemiparesis, vascular headache, developmental delay, glaucoma, and hemianopsia. We reported a three years old girl with type I SWS. She had PWS on right side of her face, leptomeningeal angiomatosis, and suspected secondary glaucoma with bilateral megalocornea. She was hospitalized because of seizures and developed transient hemiparesis. CT scan and MRI revealed abnormal calcification on asymmetric right fronto-temporo-parietal area, leptomeningeal angiomatoses and multiple small cavernous hemangioma on the tip of anterior corn of the bilateral lateral ventricle and right thalamus. EEG revealed mild general hypofunction. Patient was treated with carbamazepine and consulted to Ophthalmology, Dermatology, and Neurosurgery Departments but there were no surgery treatment at that time. Long term management was needed to prevent continuing brain damage and complications.