Skeletal dysplasia: Respiratory management during infancy

Respir Med. 2017 Oct:131:18-26. doi: 10.1016/j.rmed.2017.07.063. Epub 2017 Aug 1.

Abstract

Background: Skeletal dysplasia encompasses a variety of developmental disorders of the bone and cartilage that manifest as disproportionate shortening of limbs and trunk in the neonate. Many types of skeletal dysplasia are complicated by respiratory failure at or soon after birth and require intensive care and prolonged hospitalization. Respiratory complications in these infants are complex and are characterized by airway anomalies, restrictive lung disease due to a narrow and abnormally compliant chest wall, pulmonary hypoplasia, and central apnea. Appropriate management of these unique patients requires a clear understanding of the pathophysiology and use of pulmonary function tests for early recognition and management of complications.

Conclusion: This review provides an overview of the underlying respiratory pathology and a practical guide to the newborn care provider for the diagnosis and management of respiratory complications in infants with skeletal dysplasia.

Keywords: Infants; Respiratory; Skeletal dysplasia.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Bone Diseases, Developmental / complications
  • Bone Diseases, Developmental / physiopathology*
  • Disease Management
  • Humans
  • Infant
  • Infant, Newborn
  • Lung / abnormalities
  • Lung Diseases / etiology
  • Lung Diseases / physiopathology
  • Lung Diseases / therapy*
  • Respiratory Function Tests
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / therapy
  • Sleep Apnea, Central / etiology
  • Sleep Apnea, Central / physiopathology
  • Sleep Apnea, Central / therapy
  • Thoracic Wall