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J Clin Neonatol. 2014 Jan;3(1):49-50. doi: 10.4103/2249-4847.128739.

Lethal progressive thoracic insufficiency in a neonate due to jarcho levin syndrome.

Author information

1
Department of Pediatrics, PGIMER and Ram Manohar Lohia Hospital, New Delhi, India.
2
Department of Pediatrics, Medanta Medicity Hospital, New Delhi, India.

Abstract

A rare case of Jarcho Levin syndrome (JLS) presenting as a lethal progressive respiratory insufficiency in early neonatal period is reported. The neonate had classical features of this syndrome including vertebral segmentation defects, typical costo-vertebral fusion defects and scoliosis resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of JLS with thoracic insufficiency. In addition, our case had a rare association of dextrocardia and acyanotic congenital heart disease.

KEYWORDS:

Dextrocardia; Jarcho Levin syndrome; respiratory insufficiency; ventricular septal defect; vertebral segmentation

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