Lipomeningomyelocele (LMMC) with Jarcho-Levin syndrome (JLS) is extremely rare. The syndromic association is one of LMMC with JLS is one of phenotype of spondylocostal dysostosis (SCD). SCD is an autosomal-recessive disorder characterized by defects in the vertebrae and abnormalities of the ribs in the form of segmental fusion, malalignment, or absence of a few ribs. These patients are prone to pulmonary insufficiency and repeated infections. Close anesthetic vigil is also needed to maintain optimal ventilation during surgery. We are reporting a case of 11-month-old male child presented with LMMC swelling and absent left-side multiple ribs. On further evaluation, we found hemivertebrae and scoliosis. This report highlights the experience of operating a child with JLS in prone position.
Keywords: Fatty filum terminale; Jarcho–Levin syndrome; lipomeningomyelocele; prone positioning; spondylocostal dysostosis.