title: Chest wall hypoplasia--principles and treatment.
abstract: The chest is a dynamic structure. For normal movement it relies on a coordinated movement of the multiple bones, joints and muscles of the respiratory system. While muscle weakness can have clear impact on respiration by decreasing respiratory motion, so can conditions that cause chest wall hypoplasia and produce an immobile chest wall. These conditions, such as Jarcho-Levin and Jeune syndrome, present significantly different challenges than those faced with early onset scoliosis in which chest wall mechanics and thoracic volume may be much closer to normal. Because of this difference more aggressive approaches to clinical and surgical management are necessary.
authors.affiliation: Associate Professor of Clinical Pediatrics, Perelman School of Medicine at The University of Pennsylvania, Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, 3501 Civic Center Boulevard, Philadelphia, PA 19104. Electronic address: mayero@email.chop.edu.
authors.forename: Oscar Henry
authors.initials: OH
authors.lastname: Mayer
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000628:therapy; D004613:Ellis-Van Creveld Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D012119:Respiration; D012120:Respiration Disorders / Q000175:diagnosis* / Q000209:etiology; D035441:Thoracic Wall / Q000002:abnormalities*
keywords: Chest wall hypoplasia; Jarcho-Levin Syndrome; Jeune Syndrome; Spondylocostal dysostosis; Spondylothoracic dysplasia