Hirschsprung disease (HSCR) is a congenital anomaly of the intestine caused by the developmental absence of the enteric nervous system, which results in variable lengths of intestinal dysfunction and requires surgical intervention.Due to the complex etiology, multiple surgical procedures and postoperative complications, the diagnosis and treatment strategies of HSCR have always been the focus of pediatric surgeons around the world. Seventy pediatric surgical experts with rich clinical experience from almost all national and provincial children’s medical centers in China constituted the Chinese Research Group of Hirschsprung disease (CRGHSCR). All members discussed and compiled the disagreements concerning the diagnosis, treatment, and perioperative management of HSCR based on existing evidence from previous articles, in combination with the latest high-quality evidence. The current consensus statement is aimed at standardizing the diagnostic and surgical strategies, and emphasizing postoperative rehabilitation training, to systematically improve postoperative recovery and to improve the quality of life in long-term follow-up. The consensus was reached through a total of three rounds of discussions between February 2019 and June 2022. A modified Delphi method was used for as many reiterative rounds as necessary. The quality of evidence and recommendations were evaluated according to the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system.

先天性巨结肠(HSCR)是由肠神经系统发育缺失引起的肠道先天性异常，可导致长短不一的肠道功能障碍，需要手术干预。由于病因复杂、手术操作多及术后并发症多，HSCR的诊治策略一直是世界各地小儿外科医师关注的焦点。来自我国几乎所有国家和省级儿童医学中心的70名具有丰富临床经验的小儿外科专家组成了先天性巨结肠中国研究组(CRGHSCR)。所有成员根据以往文章的现有证据，结合最新的高质量证据，讨论并汇编了关于HSCR诊断、治疗和围手术期处理的分歧。目前的共识声明旨在规范诊断和手术策略，并强调术后康复训练，系统地改善术后恢复，提高长期随访的生活质量。2019年2月至2022年6月共进行了3轮讨论，达成了共识。必要时，使用改良的Delphi方法进行尽可能多的重复轮次。根据推荐、评估、开发和评价分级(GRADE)系统评价证据和建议的质量。