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

作者：Mads Damkjær, Joachim Tan, Joan K. Morris, Maria Loane, Joanne Given, Clara Cavero‐Carbonell, Mika Gissler, Amanda J. Neville, Anna Pierini, Anke Rißmann, David Tucker, Ester Garne · 发表于：Birth Defects Research · 年份：2024 · DOI：10.1002/bdr2.2338 · 被引用次数：5 · 研究领域：Congenital gastrointestinal and neural anomalies、Congenital Anomalies and Fetal Surgery、Intestinal Malrotation and Obstruction Disorders

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...