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Addressing the causes of late mortality in infants with congenital diaphragmatic hernia

Carmen Mesas Burgos1, Agnes Modée1, Elin Öst1

  • 1Department of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.

Journal of Pediatric Surgery
|September 15, 2016
PubMed

Insights

Congenital diaphragmatic hernia (CDH) patients face high mortality, primarily from respiratory issues in the first year. Later deaths in CDH survivors are often linked to gastrointestinal complications.

Area of Science:

  • Pediatric Surgery
  • Neonatal Care
  • Critical Care Medicine

Background:

  • Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal mortality despite advances in intensive care.
  • A notable proportion of CDH patients experience late mortality, indicating long-term challenges.

Purpose of the Study:

  • To investigate the causes of death in a cohort of 251 congenital diaphragmatic hernia patients over a 26-year period.
  • To identify patterns and primary drivers of mortality in CDH patients, differentiating between early and late fatalities.

Main Methods:

  • A retrospective review of a prospectively collected database of CDH patients treated between 1990 and 2015.
  • Analysis of all recorded causes of death within the CDH cohort.

Main Results:

  • Out of 251 CDH patients, 49 died between 1990 and 2015. Early mortality (before discharge or within the first year) accounted for the majority of deaths (14% before discharge, 5% after).
  • Cardio-respiratory failure was the predominant cause of early mortality (before age 1), while gastrointestinal complications were more common in late mortality cases (after age 1).
  • Most fatalities (86%) occurred within the first year of life, with over half dying before one month of age.

Conclusions:

  • Respiratory insufficiency and pulmonary hypertension are the leading causes of death in congenital diaphragmatic hernia patients.
  • Mortality is concentrated in the first year of life, but gastrointestinal morbidity becomes a significant cause of death in older CDH patients.
Abstract

Keywords:
Congenital diaphragmatic hernia (CDH)Follow-upLate mortalityMortalityOutcome

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