结直肠重复畸形七例诊治经验

Management experiences of colorectal duplication in children： a report of 7 cases

目的探讨儿童结直肠重复畸形的诊断和治疗经验，以提高认识，避免诊疗失误。方法回顾性分析2001年1月至2017年6月收治的术后病理均确诊为结直肠/直肠重复畸形7例患儿的临床资料。本组男4例，女3例；临床症状出现时间为出生至11月龄；确诊时间为生后2 d至11岁；管状3例，囊肿型3例，管状＋囊肿型1例。本组初诊为先天性直肠肛管畸形3例、Currarino综合征1例、直肠膀胱瘘1例、直肠舟状窝瘘1例、直肠尿道瘘并直肠黏膜脱垂1例，仅2例患儿首次手术前诊断准确。合并先天性直肠肛管畸形4例，合并Currarino综合征1例，合并直肠尿道（膀胱）瘘3例，合并后尿道瓣膜1例。结果本组全结直肠重复畸形2例，1例剔除重复畸形病灶并保留正常结直肠肠管；1例行全结直肠及病变全切，小肠拖出吻合。长段型结直肠重复畸形2例，切断保留肠段共壁，切除部分重复畸形和毗邻肠管后吻合。直肠重复畸形3例，1例完整切除囊肿型重复畸形；另2例切除重复畸形和毗邻直肠，拖下和肛门行吻合。本组平均接受3.57次手术（1～7次）。仅全结直肠及病变全切和小肠拖出吻合患儿围手术期出现吻合口瘘，被迫行再次小肠造瘘，二期关瘘，随访严重污粪2年后逐渐好转。其余患儿围手术期顺利，远期功能良好，无复发病例。结论结直肠重复畸形是一种罕见疾病，初诊时可因先天性直肠肛管畸形等非特异性表现就诊而导致误诊。在手术前应进行充分评估，手术方法需根据重复畸形类型的不同灵活选择。

ObjectiveTo explore the diagnosis and treatment of colorectal duplications, and to improve its treatment outcomes.MethodsFour boys and 3 girls with colorectal duplications between January 2001 and June 2017 were reviewed. Their clinical features, diagnosis, concomitant anomalies and treatment were retrospectively analyzed. The pathological examination verified the diagnosis. The onset time was from birth to 11 months old. And the diagnosis was confirmed from 2 days to 11 years old. There were 3 tubular shapes, 3 cystic types and 1 tubular/cystic type. The primary diagnoses were congenital anorectal disorders （n=3）, currarino syndrome （n=1） and rectovesical fistula （n=1） and rectal navicular fistula （n=1） and rectourethral fistula with rectal prolapse. Only two cases were diagnosed correctly prior to initial operation. The concomitant disease included congenital anorectal disorders （n=4）, currarino syndrome （n=1） and recto-urethral/rectovesical fistula ＆ posterior urethral valves （n=3） respectively.ResultsFor two cases of complete colorectal duplication with recto-urethral fistula, one underwent total colorectal resection ＆ ileorectal anastomosis while another had duplication resection yet retained colorectal segment. For one case of long-segment colorectal duplication with recto-urethral fistula, septum of distal colon was removed with left colectomy. One case of colonic duplication with imperforate anus underwent septal resection and anoplasty. Three rectal duplications had thorough resection. An average of 3.57 （1-7） operations were performed. Anastomotic leakage occurred in one case of complete colorectal duplication after total colorectal resection. Severe postoperative soiling continued for 2 years and there was no other perioperative complication.ConclusionsBased on our cases and literature review, we have adopted the following guidelines： （a） colorectal duplication is easily misdiagnosed for multiple clinical features; （b） preoperative evaluations should be conducted; （c） individualized solutions should be adopted after careful examinations.