Objective To explore the clinical effects of partial internal anal sphincterectomy for total co-lonic aganglionosis. Methods From Jan 2012 to July 2014,26 patients were identified TCA in our hospital. Aged from 1 month to 14 months and of a gender ratio at 1.89 ∶1 (17 male,9 female).23 of them had underg-one ileostomy surgeries before radical macrosigmoid operation.3 of them underwent radical macrosigmoid oper-ation without a stoma.through enema,anorectal manometry and laparotomy ileocolic multipoint seromuscular layer biopsy examine to diagnose,all patients underwent the colectomy,partial internal anal sphincterectomy and ileo-recto anastomosis. Results All surgeries were completed successfully.The operative time ranged from 160 to 280mins (mean 202.8mins).Blood loss during operation was 10 to 25 mL.Two of the patients were considered abdominal infection,One of them was redone the pull-through because of emblic necrosis of terminal ileum.One of them suffered from the sepsis.All the patients recovered uneventfully without anastomotic leak, cuff abscess,dehiscence,retraction and bowel obstruction.and discharged from 6 to 32 day after operation.23 patiets were followed up regularly,none of them was constipation,9 patients (64.2%)suffered from occasional enterocolitis,1 patients (7.1%)suffered from persistent enterocolitis.soiling happened in only 4 of 12 (33.3%)occasionally,one of them was mild stricture formation.2 /14 patients have the episode of failure to thrive. Conclusions TCA patiens underwent the colectomy and ileo-recto anastomosis,meanwhile partial in-ternal anal sphincterectomy in our hospital.It can prevent the postoperative constipation,control the postopera-tive enterocolitis.Defecation frequency and soiling can return normal gradually.The patients have a decent qual-ity of life.It is a alternative technique.%目的：探讨肛门内括约肌部分切除术治疗全结肠型巨结肠的临床疗效。方法自2012年1月至今我们共收治全结肠型巨结肠26例，其中男性17例，女性9例，年龄1～14个月；随访23例，失访 3例。23例先行回肠造瘘，二期根治，3例行全结肠巨结肠一期根治术，术式采用回结肠多处活检，全结肠切除，回肠肛管吻合术，全部患儿术中行肛门内括约肌部分切除。术后分别于3个月、6个月、12个月、24个月进行随访。结果患儿均手术顺利，术后感染 3例，末段回肠血栓形成、局部坏死，行二次手术1例。无直肠肌鞘感染，吻合口瘘等术后并发症。最终全部患儿治愈出院。术后随访无一例便秘复发，偶发轻微小肠结肠炎9例（64．2％），持续小肠结肠炎1例（7．1％），4例（33．3％）存在轻度污粪，无便失禁；轻度吻合口狭窄1例（7．1％）。全部患儿术后体重增加，2例生长发育落后，其余营养状况良好。结论全结肠型巨结肠采用全结肠切除，回肠肛管吻合术同时行肛门内括约肌部分切除，避免了术后便秘复发，对术后小肠结肠炎的控制效果较好，术后大便次数和控制可逐渐恢复，患儿生活质量可，是可以选择的手术方式。
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