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目的:探讨新生儿及3月龄内小婴儿先天性巨结肠（Hirschsprung’s disease，HD）的临床特征及根治术的疗效。方法:选择湖南省儿童医院胎儿与新生儿外科2012年6月至2020年7月收治的新生儿及3月龄内行根治术的HD患儿进行回顾性分析，通过住院及门诊电子病历系统、电话随访，记录患儿的临床特征及术后污粪、便秘、小肠结肠炎及排便功能等情况，并应用SPSS 20.0统计软件对数据进行分析。结果:共纳入340例HD患儿，男281例，女59例；早产儿15例，足月儿325例。胎便排出延迟275例（80.9%），术前小肠结肠炎140例次，术后小肠结肠炎71例次；短段型8例（2.4%），长段型40例（11.8%），常见型235例（69.1%），全结肠型36例（10.6%），全肠型21例（6.2%）。340例中33例（15例全结肠型、18例全肠型）术后病理诊断明确后家长放弃治疗，余307例中治愈304例（99.0%）。随访242例，随访率78.8%（242/307），污粪76例（31.4%，76/242），其中11例三级污粪；便秘9例（3.7%，9/242），其中3例三级便秘。结论:生后3个月内接受HD根治术总体安全可行，多数患儿术后排便功能良好，术后严重污粪、重度便秘、小肠结肠炎发生率较低。

Objective:To study the clinical characteristics and therapeutic efficacy of surgery for Hirschsprung's disease (HD) in infants 0-3 months of age.Methods:From June 2012 to July 2020, HD infants (0-3 months old) receiving surgery in the Department of Fetal and Neonatal Surgery of our hospital were retrospectively analyzed. Clinical characteristics and postoperative outcomes including fecal contamination, constipation, enterocolitis and bowel movement were recorded using electronic medical records (EMR) and telephone follow-ups. Data were analyzed using SPSS 20.0 software.Results:A total of 340 HD patients were enrolled, including 281 males and 59 females, 15 preterm infants and 325 full-term infants. Delayed meconium passage was observed in 275 patients (80.9%). 140 patients had preoperative enterocolitis and 71 had postoperative enterocolitis. The patients were classified into 5 categories: short-segment HD:8 cases (2.4%), long-segment HD:40 cases (11.8%), common HD: 235 cases (69.1%), total colonic HD: 36 cases (10.6%) and total intestinal HD:21 cases (6.2%). 33 patients (15 total colonic HD and 18 total intestinal HD) refused further treatment after a confirmed postoperative pathological diagnosis. For the remaining 307 cases, 304 (99.0%) were cured. 242 cases were followed-up, with a follow-up rate of 78.8% (242/307). 76 (31.4%) had fecal contamination, including 11 grade III cases.9 cases (3.7%) showed constipation with 3 severe cases.Conclusions:Surgery for HD within 3 months after birth is generally safe and feasible. Most infants have good postoperative bowel movement, with low incidences of severe fecal contamination, severe constipation and enterocolitis.