Chest wall hypoplasia--principles and treatment

Paediatr Respir Rev. 2015 Jan;16(1):30-4. doi: 10.1016/j.prrv.2014.10.012. Epub 2014 Nov 5.

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.

Keywords: Chest wall hypoplasia; Jarcho-Levin Syndrome; Jeune Syndrome; Spondylocostal dysostosis; Spondylothoracic dysplasia.

Publication types

  • Review

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Abnormalities, Multiple / therapy
  • Ellis-Van Creveld Syndrome / complications
  • Ellis-Van Creveld Syndrome / diagnosis*
  • Ellis-Van Creveld Syndrome / therapy
  • Hernia, Diaphragmatic / complications
  • Hernia, Diaphragmatic / diagnosis*
  • Hernia, Diaphragmatic / therapy
  • Humans
  • Respiration
  • Respiration Disorders / diagnosis*
  • Respiration Disorders / etiology
  • Thoracic Wall / abnormalities*

Supplementary concepts

  • Jarcho-Levin syndrome
  • Jeune syndrome