Linear Nevus Sebaceous Syndrome in a Child With Ocular Choristoma and Hemimegalencephaly

Abstract

Linear nevus sebaceous syndrome (LNSS) is a phakomatosis, associated with a variety of developmental abnormalities of ocular, nervous, skeletal, cardiovascular and urogenital systems. In LNSS, choristomas and colobomas are frequently seen signs, but complex choristomas are rare. We describe a case of a 3-year-old boy with a yellow alopecic plaque on his scalp, face and neck as well as a mass in the episcleral region since birth. He also had epileptic seizures since one year old. Histopathological examination of episcleral specimen revealed choristoma. Magnetic resonance imaging of the brain identified hemimegalencephaly, hemicerebral atrophy, and lateral ventricular enlargement (Turkderm 2011; 45: 155-7)