Children with Hirschsprung's disease have high morbidity in the first 5 years of life.
Birth Defects Res
; 116(5): e2338, 2024 May.
Article
in En
| MEDLINE
| ID: mdl-38712918
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Registries
/
Hirschsprung Disease
Limits:
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
/
Newborn
Country/Region as subject:
Europa
Language:
En
Journal:
Birth Defects Res
/
Birth defects res
/
Birth defects research
Year:
2024
Document type:
Article
Affiliation country:
Country of publication: