Yan Jun,Chen Yajun,Peng Chunhui,et al.Clinical characteristics and surgical management of total colorectal duplication in children[J].Journal of Clinical Pediatric Surgery,2025,(08):734-740.[doi:10.3760/cma.j.cn101785-202503001-006]
儿童全结直肠重复畸形的临床特点与手术治疗探讨
- Title:
- Clinical characteristics and surgical management of total colorectal duplication in children
- Keywords:
- Duplication Anomaly; Colon and Rectum; Anus; Surgical; Child
- 摘要:
- 目的 探讨儿童全结直肠重复畸形的临床特点、诊断与手术治疗策略。方法 回顾性分析2010年1月至2024年12月于首都医科大学附属北京儿童医院明确诊断后接受手术治疗的全结直肠重复畸形患儿临床资料,并检索2000-2024年PubMed、万方医学网和中国知网数据库收录的"儿童全结直肠重复畸形"相关文献报道。总结分析患儿临床表现、诊断方法与手术治疗方案。结果 5例全结直肠重复畸形患儿中,女性3例、男性2例,手术中位年龄1.1岁(范围:0.6~11.6岁)。均于生后1个月内出现正位肛门排便伴女童前庭漏便、男童尿液含粪渣,3例用力排便时肛门膨出半球形肿物;术前经会阴部查体、超声、直肠与瘘管对比造影明确诊断;均采取重复结肠粪便转流、重复直肠瘘管封闭、正位直肠肛门原位保留的手术策略;术后中位随访时间8.2年(范围:0.3~14.5年),临床症状均消失,排便功能满意。经检索收集符合纳入与排除标准文献23篇,报道27例儿童全结直肠重复畸形病例,女性15例、男性12例。11例女童正位肛门排便伴前庭或会阴漏便、5例男童正位肛门排便伴尿液含粪渣或肛门肿物膨出,其余表现不一;15例主要经对比造影等检查,12例经结肠造瘘或剖腹探查手术确诊;25例行手术治疗,其中14例行粪便转流、6例行重复畸形病变全切或黏膜剥除、5例行肠管全程贯通;20例术后随访2个月至17年,整体预后良好。结论 多数儿童全结直肠重复畸形临床表现有一定的特异性:正位肛门排便,伴女性前庭或男性泌尿生殖道漏便,用力排便时结直肠黏膜呈半球形自肛门膨出。手术前对比造影等检查可明确诊断和指导手术方案制定。"重复结肠粪便转流+重复直肠畸形矫治+正位肛门原位保留"的手术治疗策略,效果确切,预后良好。
- Abstract:
- Objective To explore the clinical characteristics,diagnostic approaches,and surgical treatment strategies for total colorectal duplication in children. Methods A retrospective analysis was conducted on pediatric patients with total colorectal duplication who were surgically treated at Beijing Children‘s Hospital,Capital Medical University,from January 2010 to December 2024.Additionally,relevant literature published between 2000 and 2024 was reviewed using the PubMed,Wanfang Medical Network,and CNKI databases.Clinical presentations,diagnostic methods,and surgical management strategies were summarized and analyzed. Results Among five children diagnosed with total colorectal duplication,there were three females and two males.The median age at surgery was 1.1 years (range:0.6-11.6 years).All patients exhibited defecation through a normally located anus within the first month of life,with additional vestibular fecal leakage in females or fecal debris in urine in males.3 children presented with a hemispherical protrusion of anorectal mucosa during straining.Diagnosis was established preoperatively through perineal physical examination,ultrasound,and contrast studies of the rectum and fistula.The surgical strategy included fecal diversion of the duplicated colon,closure of the duplicated rectal fistula,and preservation of the anayomically correct rectum and anus.After a median follow-up of 8.2 years (range:0.3-14.5 years),all patients had resolution of symptoms and satisfactory bowel function.A total of 23 eligible articles were identified,reporting 27 pediatric cases of total colorectal duplication (15 females and 12 males).Among them,11 girls had normal anal defecation with associated vestibular or perineal fecal leakage,and 5 boys presented with fecal contamination in urine or anorectal bulging; others showed varying symptoms.Diagnosis was achieved in 15 cases primarily through contrast imaging,and in 12 cases via colostomy or exploratory laparotomy.Surgical treatment was performed in 25 cases,including 14 with fecal diversion,6 with total resection or mucosal stripping of the duplicated segment,and 5 with complete unification of the colonic tract.Follow-up (ranging from 2 months to 17 years) showed overall favorable outcomes in 20 cases. Conclusions Total colorectal duplication in children often presents with distinctive clinical features,such as defecation through a normally positioned anus accompanied by vestibular or urogenital fecal leakage in females and males,respectively,and mucosal prolapse during straining.Contrast imaging is typically sufficient for diagnosis and surgical planning.A surgical strategy comprising fecal diversion of the duplicated colon,correction of rectal duplication,and preservation of the anatomically correct anus yields reliable outcomes and favorable prognosis.
参考文献/References:
[1] 赵玉元.64例肠重复畸形的外科治疗[J].临床小儿外科杂志,2007,6(2):12-14.DOI:10.3969/j.issn.1671-6353.2007.02.005. Zhao YY.Operative treatment for 64 cases of intestinal duplication deformity[J].J Clin Ped Sur,2007,6(2):12-14.DOI:10.3969/j.issn.1671-6353.2007.02.005.
[2] Yousefzadeh DK,Bickers GH,Jackson JH Jr,et al.Tubular colonic duplication-review of 1876-1981 literature[J].Pediatr Radiol,1983,13(2):65-71.DOI:10.1007/BF02390103.
[3] Yan J,Yan JY,Ding CL,et al.Clinical features of colorectal duplication in children:a study of 25 cases[J].J Pediatr Surg,2022,57(9):97-101.DOI:10.1016/j.jpedsurg.2021.09.042.
[4] Yan J,Chen YJ,Gu YC,et al.Surgical management strategy of alimentary tract duplication involving the rectum in children[J].Pediatr Surg Int,2022,39(1):31.DOI:10.1007/s00383-022-05316-7.
[5] 李龙,李平,陈幼容,等.切除重复肠管保留主肠管治疗全结肠重复畸形二例[J].中华小儿外科杂志,2001,22(2):117.DOI:10.3760/cma.j.issn.0253-3006.2001.02.022. Li L,Li P,Chen YR,et al.Thoroughly resection of duplication without damaged the colorectal:reports of two cases[J].Chin J Pediatr Surg,2001,22(2):117.DOI:10.3760/cma.j.issn.0253-3006.2001.02.022.
[6] 王皓洁,阿里木江·阿不都热依木,赵静儒,等.全结肠重复畸形1例[J].临床小儿外科杂志,2011,10(6):478.DOI:10.3969/j.issn.1671-6353.2011.06.030. Wang HJ,Abudureyimu ALMJ,Zhao JR,et al.Total colon duplication:report of one case[J].J Clin Ped Sur,2011,10(6):478.DOI:10.3969/j.issn.1671-6353.2011.06.030.
[7] 余东海,施佳,龚一鸣,等.结直肠重复畸形七例诊治经验[J].中华小儿外科杂志,2018,39(6):445-450.DOI:10.3760/cma.j.issn.0253-3006.2018.06.009. Yu DH,Shi J,Gong YM,et al.Management experiences of colorectal duplication in children:a report of 7 cases[J].Chin J Pediatr Surg,2018,39(6):445-450.DOI:10.3760/cma.j.issn.0253-3006.2018.06.009.
[8] Kumara PD,Jayawardane GL,Aluwihare AP.Complete colonic duplication in an infant[J].Ceylon Med J,2001,46(2):69-70.DOI:10.4038/cmj.v46i2.6499.
[9] Kaur N,Nagpal K,Sodhi P,et al.Hindgut duplication-case report and literature review[J].Pediatr Surg Int,2004,20(8):640-642.DOI:10.1007/s00383-004-1248-x.
[10] Sarpel U,Le MN,Morotti RA,et al.Complete colorectal duplication[J].J Am Coll Surg,2005,200(2):304-305.DOI:10.1016/j.jamcollsurg.2004.09.039.
[11] Rathi V,Singh S,Bhargava SK,et al.Diagnosis of tubular colonic duplication by barium follow-through study[J].Australas Radiol,2005,49(2):157-159.DOI:10.1111/j.1440-1673.2005.01363.x.
[12] Banu T,Chowdhury TK,Hoque M,et al.Congenital double anus with total colon duplication:a case report[J].J Pediatr Surg,2007,42(1):E1-E2.DOI:10.1016/j.jpedsurg.2006.11.001.
[13] Fuchs JR,Clark K,Breckler FD,et al.Complete colonic duplication-a case report[J].J Pediatr Surg,2008,43(6):E11-E13.DOI:10.1016/j.jpedsurg.2008.01.057.
[14] Bakir B,G?k?e S,Dursun M,et al.An unusual cause of diffuse ascites in an infant:colonic duplication associated with bladder duplication[J].Diagn Interv Radiol,2009,15(3):210-211.DOI:10.1259/dmfr/28183053.
[15] Alfadhel M,Pugash D,Robinson AJ,et al.Pre-and postnatal findings in a boy with duplication of the bladder and intestine:report and review[J].Am J Med Genet A,2009,149A(12):2795-2802.DOI:10.1002/ajmg.a.33091.
[16] Costa EC,Ferreira CT,Salle JLP,et al.Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome[J].J Pediatr Surg,2011,46(11):2184-2186.DOI:10.1016/j.jpedsurg.2011.08.022.
[17] Khaleghnejad Tabari A,Mirshemirani A,Khaleghnejad Tabari N.Complete colonic duplication in children[J].Caspian J Intern Med,2012,3(2):436-439.
[18] Jellali MA,Mekki M,Saad J,et al.Perinatally discovered complete tubular colonic duplication associated with anal atresia[J].J Pediatr Surg,2012,47(6):e19-e23.DOI:10.1016/j.jpedsurg.2012.01.082.
[19] Castejón-Casado J,Mu?oz Miguelsanz M,Diaz EM,et al.Acute abdomen secondary to complete tubular colonic duplication[J].J Indian Assoc Pediatr Surg,2014,19(3):166-168.DOI:10.4103/0971-9261.136477.
[20] Aworanti O,Twomey E,Awadalla S.Terminal ileum and total colonic duplication associated with a rectovestibular fistula in a child[J].Ir Med J,2014,107(8):241-242.
[21] Bhadoo D,Chand K,Jana M,et al.Colonic duplication:treatment by limited division of common wall[J].J Indian Assoc Pediatr Surg,2015,20(3):146-147.DOI:10.4103/0971-9261.159031.
[22] AbouZeid AA,Mohammad SA,Ibrahim SE,et al.Late diagnosis of complete colonic and rectal duplication in a girl with an anorectal malformation[J].European J Pediatr Surg Rep,2019,7(1):e47-e50.DOI:10.1055/s-0039-1692193.
[23] Duess JW,Zimmermann P,Hirsch FW,et al.Complete colonic duplication and perineal fistula:transanal mucosectomy of the ectopic rectum[J].European J Pediatr Surg Rep,2022,10(1):e102-e106.DOI:10.1055/s-0042-1750028.
[24] Piplani R,Acharya SK,Bagga D.Recto-vestibular fistula with colonic duplication:report of three cases and review of literature[J].Ann Pediatr Surg,2022,18(1):47.DOI:10.1186/s43159-022-00186-z.
[25] Parveen R,Jester I.Complete tubular colonic duplication ending in a common channel as a recto vestibular fistula:a case report and literature review of management options[J].J Indian Assoc Pediatr Surg,2022,27(1):103-105.DOI:10.4103/jiaps.JIAPS_275_20.
[26] Kanbar M,Alomar K,Alkader MA,et al.A unique case of complete tubular colonic duplication with normal anus accompanied by vestibular fistula in a 12 years _ old _ girl-A case report[J].Int J Surg Case Rep,2023,108:108405.DOI:10.1016/j.ijscr.2023.108405.
[27] Wang YH,Wu YL,Chen YH,et al.A 4-month-old female infant with complete colonic duplication presenting with stool leaking out of the vestibule[J].Kaohsiung J Med Sci,2023,39(9):954-955.DOI:10.1002/kjm2.12730.
相似文献/References:
[1]壮文军姜子非谷兴琳.腹会阴肛门成形术17例分析[J].临床小儿外科杂志,2011,10(01):0.
备注/Memo
收稿日期:2025-3-1。
通讯作者:陈亚军,Email:chenyajunmd@aliyun.com