Review shows that implementing a nationwide protocol for congenital diaphragmatic hernia was a key factor in reducing mortality and morbidity

Acta Paediatr. 2018 Jul;107(7):1131-1139. doi: 10.1111/apa.14169. Epub 2018 Feb 7.

Abstract

The French Rare Disease Reference Center for congenital diaphragmatic hernia (CDH) was created in 2008, to implement a national protocol for foetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care. It has been well established that a standardised management protocol improves the prognosis of children with CDH.

Conclusion: Organising health care and implementing a nationwide French protocol were key factors for reducing mortality and morbidity from CDH.

Keywords: Congenital diaphragmatic hernia; Mortality; National protocol; Persistent pulmonary hypertension; Pulmonary hypoplasia.

Publication types

  • Review

MeSH terms

  • Clinical Protocols
  • France / epidemiology
  • Hernias, Diaphragmatic, Congenital / complications
  • Hernias, Diaphragmatic, Congenital / mortality
  • Hernias, Diaphragmatic, Congenital / therapy*
  • Humans
  • Perinatal Care
  • Prenatal Care
  • Respiratory Insufficiency / etiology