Children with Hirschsprung's disease have high morbidity in the first 5 years of life

Mads Damkjær, Joachim Tan, Joan K Morris, Maria Loane, Joanne Emma Given, Clara Cavero-Carbonell, Mika Gissler, Amanda J Neville, Anna Pierini, Anke Rissmann, David Tucker, Ester Garne*

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

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Abstract

BACKGROUND: Hirschsprung's disease is a rare congenital anomaly of the colon with absence of the ganglionic nerve cells. The treatment of the anomaly is surgical.

METHODS: This population-based data-linkage cohort study was part of the EUROlinkCAT project and investigated mortality and morbidity for the first 5 years of life for European children diagnosed with Hirschsprung's disease. Nine population-based registries in five countries from the European surveillance of congenital anomalies network (EUROCAT) participated. Data on children born 1995-2014 and diagnosed with Hirschsprung's disease were linked to hospital databases. All analyses were adjusted for region and length of follow-up, which differed by registry.

RESULTS: The study included 680 children with Hirschsprung's disease. One-year survival was 97.7% (95% CI: 96.4-98.7). Overall, 85% (82-87) had a code for a specified intestinal surgery within the first year increasing to 92% (90-94) before age 5 years. The median age at the first intestinal surgery up to 5 years was 28 days (11-46) and the median number of intestinal surgical procedures was 3.5 (3.1-3.9). Thirty days mortality after neonatal surgery (within 28 days after birth) was 0.9% (0.2-2.5) for children with a code for intestinal surgery within the first 28 days after birth and there were no deaths for children with a code for stoma surgery in the neonatal period.

CONCLUSION: Children with Hirschsprung's disease have a high morbidity in the first 5 years of life requiring more surgical procedures in addition to the initial surgery. Mortality after neonatal surgery is low.

Original languageEnglish
Article numbere2338
JournalBirth Defects Research
Volume116
Issue number5
Number of pages7
ISSN2472-1727
DOIs
Publication statusPublished - May 2024

Bibliographical note

© 2024 The Authors. Birth Defects Research published by Wiley Periodicals LLC.

Keywords

  • Humans
  • Hirschsprung Disease
  • Female
  • Male
  • Infant
  • Child, Preschool
  • Registries
  • Infant, Newborn
  • Morbidity
  • Cohort Studies
  • Europe

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