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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
背景：先天性巨结肠病是一种罕见的先天性结肠异常，缺乏神经节神经细胞。异常的治疗是手术。
方法：这项基于人群的数据关联队列研究是EUROlinkCAT项目的一部分，调查了被诊断为先天性巨结肠病的欧洲儿童的前5年死亡率和发病率。来自欧洲先天性异常监测网络（EUROCAT）的五个国家的9个基于人口的登记册参加了会议。1995-2014年出生并被诊断患有先天性巨结肠病的儿童的数据与医院数据库相关联。所有分析都根据随访的区域和长度进行了调整，因注册表而异。
结果：该研究包括680名患有先天性巨结肠的儿童。一年生存率为97.7%（95%CI：96.4-98.7）。总的来说,85％（82-87）在第一年内具有指定的肠道手术的代码，在5岁之前增加到92％（90-94）。首次肠道手术至5岁的中位年龄为28天（11-46），肠道外科手术的中位数量为3.5（3.1-3.9）。新生儿手术后30天（出生后28天内），出生后前28天内有肠道手术规范的儿童死亡率为0.9%（0.2-2.5），新生儿期有造口手术规范的儿童没有死亡。
结论：患有先天性巨结肠的儿童在生命的前5年发病率很高，除了最初的手术外，还需要更多的外科手术。新生儿手术后死亡率较低。
方法：这项基于人群的数据关联队列研究是EUROlinkCAT项目的一部分，调查了被诊断为先天性巨结肠病的欧洲儿童的前5年死亡率和发病率。来自欧洲先天性异常监测网络（EUROCAT）的五个国家的9个基于人口的登记册参加了会议。1995-2014年出生并被诊断患有先天性巨结肠病的儿童的数据与医院数据库相关联。所有分析都根据随访的区域和长度进行了调整，因注册表而异。
结果：该研究包括680名患有先天性巨结肠的儿童。一年生存率为97.7%（95%CI：96.4-98.7）。总的来说,85％（82-87）在第一年内具有指定的肠道手术的代码，在5岁之前增加到92％（90-94）。首次肠道手术至5岁的中位年龄为28天（11-46），肠道外科手术的中位数量为3.5（3.1-3.9）。新生儿手术后30天（出生后28天内），出生后前28天内有肠道手术规范的儿童死亡率为0.9%（0.2-2.5），新生儿期有造口手术规范的儿童没有死亡。
结论：患有先天性巨结肠的儿童在生命的前5年发病率很高，除了最初的手术外，还需要更多的外科手术。新生儿手术后死亡率较低。
