title: Lethal progressive thoracic insufficiency in a neonate due to jarcho levin syndrome.
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.
authors.affiliation: Department of Pediatrics, PGIMER and Ram Manohar Lohia Hospital, New Delhi, India.
authors.forename: Euden
authors.initials: E
authors.lastname: Bhutia
authors.affiliation: Department of Pediatrics, PGIMER and Ram Manohar Lohia Hospital, New Delhi, India.
authors.forename: Arti
authors.initials: A
authors.lastname: Maria
authors.affiliation: Department of Pediatrics, PGIMER and Ram Manohar Lohia Hospital, New Delhi, India.
authors.forename: Arushi
authors.initials: A
authors.lastname: Verma
authors.affiliation: Department of Pediatrics, Medanta Medicity Hospital, New Delhi, India.
authors.forename: Sidharth Kumar
authors.initials: SK
authors.lastname: Sethi
keywords: Dextrocardia; Jarcho Levin syndrome; respiratory insufficiency; ventricular septal defect; vertebral segmentation
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