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Case Reports
. 2014 Sep-Dec;9(3):249-52.
doi: 10.4103/1817-1745.147580.

Spondylocostal dysostosis with lipomyelomeningocele: Case report and review of the literature

Shailendra D Anjankar  1 , Raju Subodh  1
Affiliations
Case Reports

Spondylocostal dysostosis with lipomyelomeningocele: Case report and review of the literature

Shailendra D Anjankar et al. J Pediatr Neurosci. 2014 Sep-Dec.

Abstract

Spondylocostal dysostosis (SCDO) is rare anomaly caused due to flawed embryological development of the axial skeleton during preliminary stages of gravidity, characterized by malformed vertebral column and ribs, abridged thorax and kyphoscoliosis. This entity was also reported as a "Jarcho-Levin syndrome" eponym by erstwhile authors, before the introduction of genetic based classification. A literature review showed only three cases of this clinical entity with lipomyelomeningocele. We report the fourth case report of an infant with SCDO with lipomyelomeningocele. His chest X-ray displayed absent left side 6(th)-8(th) ribs with peculiar fan like configuration, making the heart vulnerable to any direct injury. Special care has to be taken for such patients who need surgical procedure in the prone position.

Keywords: Jarcho–Levin syndrome; lipomyelomeningocele; spondylocostal dysostosis.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Chest X-ray: Absent left 6th to 8th ribs with its fan like configuration, 7th and 9th butterfly shaped thoracic vertebrae and mild scoliosis of thoracolumbar spine
Figure 2
Figure 2
Magnetic resonance imaging spine: (a) T1-weighted Sagittal image and (b) T2-weighted Sagittal image displaying lumbosacral lipomyelomeningocele with low lying cord at L4–L5 level with tethered cord and deformed 7th and 9th thoracic vertebrae (c) T2-weighted axial image at L4–L5 spinal level showing lipomyelomeningocele communicating through vertebral defect (d) short inversion time inversion recovery coronal image demonstrating deficient and malformed rib cage on left side
See this image and copyright information in PMC

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