Abstract
Jarcho-Levin Syndrome is first described in 1938 as crab-shaped thorax deformity and several vertebral anomalies causing respiratory distress. Later on, it is disclosed that other systems are also involved in the syndrome. There are two fenotypes of the disorder namely spondylothoracic dysplasia (STD) and spondylocostal dysostosis (SCD). The prognosis of STD is more fatal. In SCD type, which thorax deformities are milder, accompanying complex cardiac deformities make the prognosis worse. Here we report a rare case of SCD type Jarcho-Levin syndrome associated with Fallot type double outlet right ventricule defect. This is the first such case in the literature
Keywords: Jarcho-Levin syndrome, spondylocostal dysplasia, complex cardiac pathology
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