Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:487 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size

Table of Contents   
Year : 2020  |  Volume : 25  |  Issue : 1  |  Page : 10-14

Type IV congenital pouch colon in male children: Anatomical variations and a proposed new subclassification

Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission02-Sep-2018
Date of Decision05-Oct-2018
Date of Acceptance29-Dec-2018
Date of Web Publication27-Nov-2019

Correspondence Address:
Dr. Prema Menon
Level 3A, Room No. 3103, Advanced Pediatric Center, Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_189_18

Rights and Permissions



Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformation. In male patients, CPC communicates distally with the urogenital tract by a large fistula. The CPC cases which do not fulfill the criteria as mentioned in the classical description are reported along with the pertinent literature review.
Materials and Methods: This was a retrospective study from January 2004 to December 2017 of male children with Type IV CPC. We evaluated clinical presentation, primary management, anatomical relationship, previous surgical intervention, definitive management, result, and outcome in terms of continence status on Templeton score.
Results: Fifty-one children were included in the study among whom 36 children (Group 1) had a colovesical fistula and 15 children (Group 2) had no communication of the pouch with the genitourinary tract. In Group 2 children, the clinical presentations and management were varied: 4 underwent primary pull-through procedure, whereas 11 underwent staged procedure. Group 2 included three children in whom a narrow and thin-walled anal canal or anal canal with lower rectum was present, which was incorporated during the pull-through procedure. On continence assessment, only one child in Group 1 had “good” continence score compared to four children (three having anal canal) in Group 2.
Conclusion: CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence.

Keywords: Anal stenosis, anorectal malformation, congenital pouch colon, fecal continence

How to cite this article:
Solanki S, Menon P, Nayak S, Samujh R, N. Rao K L. Type IV congenital pouch colon in male children: Anatomical variations and a proposed new subclassification. J Indian Assoc Pediatr Surg 2020;25:10-4

How to cite this URL:
Solanki S, Menon P, Nayak S, Samujh R, N. Rao K L. Type IV congenital pouch colon in male children: Anatomical variations and a proposed new subclassification. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2022 Jan 25];25:10-4. Available from: https://www.jiaps.com/text.asp?2020/25/1/10/271729

   Introduction Top

Congenital pouch colon (CPC) is an anomaly in which a pouch-like dilatation of a shortened colon is associated with an anorectal malformation (ARM). The most widely used classification of CPC described by Narasimha Rao et al. categorizes CPC into four subtypes (Types I–IV) based on the length of the normal colon proximal to the colonic pouch.[1] Type V also has been described in the literature.[2],[3] Type I is the most severe form and Type IV is the least. In males, the pouch usually terminates in a colovesical fistula just proximal to the bladder neck.[1],[4] CPC has been included, as a rare variant, in the Krickenbeck classification of ARM. A recent review showed that about 90% of reported cases in the world were from India.[5] According to Gupta and Sharma, CPC should have some anatomical criteria: (1) there is anorectal agenesis, (2) total length of the colon is short, (3) colon has a pouch formation for a varying length – saccular or diverticular with the collection of meconium or fecal matter, (4) the blood supply to the pouch is abnormal, (5) the colon wall is thick and muscular with hypertrophied mucosa, (6) the fistula with the genitourinary tract is large, muscular, and long and it is closely adherent with the bladder wall, and (7) there is no transitional zone between the pouch colon and the normal bowel. The pattern changes suddenly and sharply.[5] In this study, we are discussing Type IV CPC cases which do not fulfill all the above-mentioned criteria.

   Materials and Methods Top

This was a retrospective study from January 2004 to December 2017, conducted at a tertiary care center. Data were collected from previous records maintained by us. Ethical clearance was obtained from the institutional review board (IEC/2018/000831). We included all male patients with CPC Type IV, irrespective of their age at presentation, any operative intervention before presenting to our center, and also children who were still awaiting a definitive procedure. All other types of male ARM including CPC Types I, II, and III as well as female patients with ARM were excluded as well as children with incomplete data and children/parents who refused to participate in the study.

All children routinely underwent two-dimensional echo and ultrasonography of the kidney, ureter, and bladder. Other investigations were guided by clinical examination findings. Wherever feasible, either a contrast enema or distal cologram was performed before the definitive procedure [Figure 1]a. Our routine protocol for children with CPC presenting in the newborn period with an absent anal opening and abdominal distension is pouch excision, fistula ligation, and end colostomy at the first stage and later on abdominoperineal pull through (APPT) at 9 months–1 year of age. In all cases (except where pouch excision was already done at another hospital), the excised pouch was sent for histopathological examination. We evaluated clinical presentation, primary management, anatomical configuration, previous surgical intervention, definitive management, result, and outcome in terms of continence status. Continence assessment was done according to the Templeton score questionnaire.[6] Only those children/parents were interviewed where the child was at least 6 months after completion of all stages of surgery and was above 3 years of age.
Figure 1: (a) Barium enema study of a child presenting with constipation postanoplasty for low anorectal malformation. Black arrow is showing sharp cutoff of narrow and dilated area. (b) Intraoperative images of pouch colon. Black arrow is showing abrupt change of caliber without transition zone. White arrow is showing abnormal vasculature

Click here to view

Data were analyzed using the software, Statistical Package for the Social Sciences (SPSS) for Windows (IBM SPSS Statistics for windows, version 22.0. Armonk, NY, USA). The difference between the occurrence of associated malformations in both the groups and also the difference between the outcomes (continence scores) in different groups were compared by Pearson's Chi-squared test. P < 0.05 was considered statistically significant.

   Results Top

A total of 51 male children of Type IV CPC were included for the analysis with 36 (Group 1) having colovesical fistula and 15 (Group 2) without any genitourinary communication. A total of 27 associated anomalies were present [Table 1]. In Group 1, all patients initially underwent end colostomy and fistula ligation. One child died after initial colostomy due to septicemia. Of the remaining, 31 have undergone APPT and 4 are awaiting APPT. In Group 2 patients, the clinical presentations and management were varied [Table 2] and [Table 3]. Among the staged procedures in Group 2, in three children, the anal canal was present. This was subsequently incorporated in bowel continuity: in two, the upper sigmoid was anastomosed to the anal canal, and in one, the descending colon was anastomosed to the lower rectum. All children were operated by either of two surgeons (KLNR/PM). In all cases, histopathology of excised pouch confirmed the diagnosis with certain distinguishing pathological findings, i.e., acute and chronic inflammation of the mucosa and submucosa, focal or generalized thinning of muscle layers, especially of the outer muscle coat, disorganized and disrupted muscle layers, decreased number of mature ganglion cells, neuronal hyperplasia, and hypertrophy in nerve plexuses.
Table 1: Associated malformations in the study group

Click here to view
Table 2: Clinical presentation of Type IV congenital pouch colon without genitourinary communication

Click here to view
Table 3: Surgical interventions done in Group 2 children

Click here to view

The mean follow-up duration was 68.2 months (range: 3 months–14 years) and 35.8 months (range: 6 months–8 years) for Group 1 and Group 2, respectively. In the entire study group, 41 patients had increased frequency of stool, whereas 5 children had constipation.

To assess continence status, 34 children were eligible for interview. The remaining were either awaiting definitive surgery (n = 4) or could not be contacted (n = 6), or age was <3 years (n = 6). Continence was significantly better in Group 2, especially in those with anal canal preservation [Table 4].
Table 4: Distribution of children according to the Templeton score of continence

Click here to view

   Discussion Top

During the preliminary descriptions of CPC, there was a difference of opinion regarding the existence of this entity. At present, it is now a well-established diagnosis with certain anatomical features with various classifications. Rao's Type I CPC was the most common form reported in the literature previously, but gradually, Type IV has become more common. It may be because of the decreased severity of pouch colon or because of proper identification of Type IV cases which were missed out previously.[5],[7],[8] In this type, rectum or rectosigmoid is converted into a pouch and rest of proximal intestine is normal.[1] In most descriptions, CPC in male children presented with absent anal opening and had communication with the genitourinary tract.[4],[5] In our study also, while the most common presentation was similar to that described in the literature, i.e., absent anal opening and colovesical communication, 15 children presented in a different manner, and consequently, the management was also different.

For better understanding and proper comparison, we propose that Type IV CPC should be subclassified as mentioned in [Figure 2]. We stress on the presence of anal canal and its location within a normally placed sphincter muscle complex, with resulting beneficial effect on continence. The anal canal in these patients seems narrow and thin walled. On careful examination, the dentate line can be seen. The anal opening in these patients is located at the normal place but is very small. The external appearance is variable, and the anus may be pulled up or protruding outside with a whorled appearance of the surrounding skin [Figure 3]a.
Figure 2: Proposed subclassification of Type IV pouch colon

Click here to view
Figure 3: Operative images of sigmoid to anal canal anastomosis. (a) Preoperative image is showing anal stenosis. Note the whorled appearance of the narrow anal opening. (b) The anal opening is widened and the sigmoid everted for anastomosis to anal canal. (c) Completed anastomosis and reposition of bowel inside the anal opening

Click here to view

Urogenital malformations (undescended testis, vesicoureteral reflux, renal agenesis, etc.) have been previously reported as the most common associated anomalies with CPC.[9],[10],[11] However, in spite of the high supralevator nature of CPC, major associated malformations are not common. A review of 566 cases showed hydronephrosis and hydroureteronephrosis in 7% and 28.2%, respectively, vesicoureteral reflux in 6.36%, and renal agenesis in 1.4%.[5] In our study also, urogenital malformations were most commonly associated. Associated anomalies were common in Group 1 (58.3%) compared to Group 2 (40%) although not statistically significant.

In our study, three children had initially presented with constipation after an anoplasty despite an adequate anal opening. On subsequent contrast enema, CPC Type IV was diagnosed. Pavai et al. also reported a similar experience wherein two male children presenting with constipation after anoplasty for low ARM were found to have a Type IV CPC on further evaluation.[9] We want to emphasize the point that when children with low ARM do not improve after anoplasty (in spite of an adequate anal opening), we should reconsider the diagnosis and perform a contrast enema to delineate the anatomy.

A review of the literature shows the use of single stage as well as staged procedures in the management of Type IV CPC.[12],[13] While both have advantages and disadvantages, we prefer an individualized approach. In a stable child with well-defined anatomy, we preferred to do a one-stage procedure. In acute presentation and poor general condition, staged procedure should always be considered. The ability to prepare the bowel properly before a single-stage pull through should also be considered as it is difficult to clear a loaded colon in the presence of anal stenosis. Intraoperatively, it is important to differentiate between pouch colon and bowel dilatation due to distal obstruction, CPC being identified by the sharp cutoff between normal bowel and pouch (no transition zone), abnormal vascularity, and thickened wall [Figure 1]b.[5] This can be confirmed later by biopsy of resected segment as histopathological criteria are also well defined.[14],[15]

In our study, a total of eight children had a dysplastic anal canal, i.e., narrow and thin walled. However, only in three children, the anal canal could be incorporated in bowel continuity during APPT [Figure 3]. In these three children, postoperative continence status was superior [Table 4]. They presented with constipation (like low ARM patients) during initial follow–up while most of the other children had stool frequency (like HARM patients) and soiling during follow-up. These three children had normal bowel continence and were completely asymptomatic at the last follow-up. In our view, this adaptation of surgical technique according to the peculiar anatomy led to better functional results. The presence of anal canal signifies well-formed sphincter complex with intact sensory-motor pathways. Therefore, we recommend to preserve the native distal most bowel and incorporate it into bowel continuity even though it may appear apparently very narrow and thin.

   Conclusion Top

CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence.

Informed consent

Patients' consent was obtained for the surgery. It was a retrospective study. Data were collected from the old records. Permission was obtained from the Institute Ethical Board for “Waiver for consent of the patients to participate in the study” (IEC/2018/000831).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Narasimha Rao KL, Yadav K, Mitra SK, Pathak IG. Congenital short colon with imperforate anus (pouch colon syndrome). Ann Pediatr Surg 1984;1:59.  Back to cited text no. 1
Saxena AK, Mathur P. Classification of congenital pouch colon based on anatomic morphology. Int J Colorectal Dis 2008;23:635-9.  Back to cited text no. 2
Rawat JD, Singh S, Pant N, Chaubey D. Type V congenital pouch colon: An extremely rare variant of anorectal malformations. J Indian Assoc Pediatr Surg 2017;22:122-3.  Back to cited text no. 3
[PUBMED]  [Full text]  
Chadha R, Khan NA. Congenital pouch colon. J Indian Assoc Pediatr Surg 2017;22:69-78.  Back to cited text no. 4
[PUBMED]  [Full text]  
Gupta DK, Sharma S. Congenital pouch colon-then and now. J Indian Assoc Pediatr Surg 2007;12:5-12.  Back to cited text no. 5
  [Full text]  
Templeton JM, Ditesheim JA. High imperforate anus, quantitative result of long term fecal continence. J Pediatr Surg1985;20:645-52.  Back to cited text no. 6
Rao KL, Menon P. Congenital pouch colon associated with anorectal agenesis (pouch colon syndrome). Pediatr Surg Int 2005;21:125-6.  Back to cited text no. 7
Pathak IC. Pouch colon syndrome: A changing scenario. Ann Natl Acad Med Sci 1999;35:69-81.  Back to cited text no. 8
Pavai A, Pillai SD, Shanthakumari S, Sam CJ, Shylaja M, Sabarivinoth R, et al. Congenital pouch colon: Increasing association with low anorectal anomalies. J Indian Assoc Pediatr Surg 2009;14:218-20.  Back to cited text no. 9
[PUBMED]  [Full text]  
Mathur P, Rana YP, Simlot A, Soni V. Congenital pouch colon with duplicate bladder exstrophy. J Pediatr Surg 2008;43:E9-11.  Back to cited text no. 10
Bangroo AK, Tiwari S, Khetri R, Sahni M. Congenital pouch colon with prune belly syndrome and megalourethra. Pediatr Surg Int 2005;21:474-7.  Back to cited text no. 11
Gangopadhyay AN, Shilpa S, Mohan TV, Gopal SC. Single-stage management of all pouch colon (anorectal malformation) in newborns. J Pediatr Surg 2005;40:1151-5.  Back to cited text no. 12
Ghritlaharey RK, Budhwani KS. Two-staged management for all types of congenital pouch colon. Afr J Paediatr Surg 2013;10:17-23.  Back to cited text no. 13
[PUBMED]  [Full text]  
Agarwal K, Chadha R, Ahluwalia C, Debnath PR, Sharma A, Roy Choudhury S, et al. The histopathology of congenital pouch colon associated with anorectal agenesis. Eur J Pediatr Surg 2005;15:102-6.  Back to cited text no. 14
Udawat H, Nunia V, Mathur P, Udawat HP, Gaur KL, Saxena AK, et al. Histopathological and immunohistochemical findings in congenital pouch colon: A Prospective study. Pathobiology 2017;84:202-9.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1], [Table 2], [Table 3], [Table 4]


Print this article  Email this article


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (906 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Materials and Me...
    Article Figures
    Article Tables

 Article Access Statistics
    PDF Downloaded91    
    Comments [Add]    

Recommend this journal

Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer 

  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05