| CASE REPORT |
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| Year : 2017 | Volume
: 12
| Issue : 4 | Page : 386-388 |
Lipomeningomyelocele with fatty filum terminale in a patient with Jarcho–Levin syndrome: A rare association requiring special attention
Suyash Singh, Kuntal K Das, Raj Kumar
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India
Correspondence Address:
Prof. Raj Kumar
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Raebareli Road, Lucknow, Uttar Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/JPN.JPN_108_17
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.
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