%0 Journal Article
%T Children with Hirschsprung's disease have high morbidity in the first 5 years of life.
%A Damkjær M
%A Tan J
%A Morris JK
%A Loane M
%A Given J
%A Cavero-Carbonell C
%A Gissler M
%A Neville AJ
%A Pierini A
%A Rissmann A
%A Tucker D
%A Garne E
%J Birth Defects Res
%V 116
%N 5
%D 2024 May
%M 38712918
暂无%R 10.1002/bdr2.2338
%X <B>BACKGROUND: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.