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Year : 2021  |  Volume : 26  |  Issue : 3  |  Page : 153-161

Congenital pouch colon: Case series and review of evidences for resection

1 Department of Salute della Donna e del Bambino, Division of Pediatric Surgery, University of Padova, Padova, Italy
2 Department of Pediatric Surgery, Vittore Buzzi Children Hospital, Milano, Lombardia, Italy
3 Mother and Child Department, Pediatric Surgery Unit, “Cà Foncello Hospital” Treviso, Treviso, Italy, Italy

Date of Submission28-Feb-2020
Date of Decision24-May-2020
Date of Acceptance20-Sep-2020
Date of Web Publication17-May-2021

Correspondence Address:
Dr. Francesco Fascetti-Leon
Department of Women's and Children's Health, Division of Paediatric Surgery, University of Padova, Via Giustiniani 2, Padua 35128
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_53_20

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Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformations (ARM) with its highest reported incidence in India. We aimed to describe five patients affected by CPC, in which the tissue from the terminal dilated colon has been successfully used and to discuss our results on the light of an extended revision of the literature.
Materials and Methods: The clinical details of five cases treated for CPC in two Italian Centers were retrospectively reviewed assessing the fate of the terminal dilated colon.
Results: In all cases, the tissue from dilated colon has been used. The double vascular system of the dilated pouch allowed increasing bladder capacity (case 4), reconstruction of the vagina (case 3, 5), and lengthening of the colon (case 1, 2, 5). In our series, 3/5 have a good bowel control with daily bowel management after ARM correction. In literature, there are not differences in terms of dependence from bowel management in patients with pouch resected and in patients with pouch saved (P = 0.16).
Conclusions: We acknowledge that the analysis of the available literature is limited by the absence of studies with high level of evidence and the removal or the preservation of the abnormal colon tissue seems to follow the surgeon preferences.

Keywords: Anorectal malformation, augmentation bladder capacity, congenital pouch colon, vaginal reconstruction

How to cite this article:
Duci M, Fascetti-Leon F, Pergola EL, Midrio P, Gamba P. Congenital pouch colon: Case series and review of evidences for resection. J Indian Assoc Pediatr Surg 2021;26:153-61

How to cite this URL:
Duci M, Fascetti-Leon F, Pergola EL, Midrio P, Gamba P. Congenital pouch colon: Case series and review of evidences for resection. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2023 Mar 30];26:153-61. Available from: https://www.jiaps.com/text.asp?2021/26/3/153/316012

   Introduction Top

Congenital pouch colon (CPC) is a rare variant of anorectal malformations (ARM) with its highest reported incidence in India.[1] It may present therapeutic challenge for surgeons who are not familiar with the malformations' peculiar anatomy. In CPC, the distal part of the colon, shorter than normal, forms a dilated pouch that is frequently connected to the urinary tract. Male are preponderant, with the male-to-female ratio between 3 to 1 and 7 to 1.[2] Different classifications have been proposed: Narasimharao et al. classified CPC into four subtypes based on the length of normal colon proximal to the pouch.[3] More recently, Saxena added the fifth type as a double pouch colon, whose distal part terminates in a fistula with the urinary tract, with short normal interposed colon segment.[4] Other classifications divided patients with CPC in two groups: Complete CPC which include type 1, 2 and 5 and Incomplete CPC which include type 3 and 4.[5]

Almost hundred papers have been published in the past 3 decades on the CPC; however, there in not consensus about the surgical management of the dilated colon. The treatment varies accordingly to the characteristics of each single case and most authors advise not to preserve the pouch in order to reduce the risk of complications (i.e. pouch redilation, soiling, incontinence, or constipation) supported by the pathology findings of abnormal muscle layer.[6],[7],[8],[9] By contrast, some reports advise to maintain part of pouch to improve water absorption and reservoir function.[10],[11],[12] These discrepant ideas along with the possibility of using the pouch tissue as a source for genitourinary reconstruction, prompted us to search systematically the literature and revise our experience.

   Materials and Methods Top

The clinical details of consecutive cases treated for CPC in the two Centers (Paediatric Surgery Unit of Padova and Paediatric Surgery Unit of Treviso, Italy) were retrospectively reviewed assessing the fate of the terminal dilated colon [Table 1].
Table 1: Case series from two Italian centres

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   Results Top

Case series

A total number of 5 cases were included in this case series [Table 1]. Three were males (60%) and 2 were female (40%) with a median birth weight of 2654 g (range 1900–3200 g). The age of presentation was ranged from 1 day to 7 months; all the male cases had an absent anal opening and were presented with abdominal distension, whereas the female cases (2 cases) were presented with the passage of meconium from a single perineal opening (cloaca). In all cases there was a fistula connected to the urinary tract. The primary surgical procedure consisted of performing a divided ileostomy in 4/5 cases and a colostomy in one case. In 4/5 cases a complete pouch colon was identified, whereas in 1 case incomplete pouch was noticed. The reconstructive surgery was performed after a median of 12.2 months (range 4–26 months). During the second surgery, the ligation of fistula with the bladder was performed. In [Table 1], the use of pouch tissue is summarized. In particular, in case 2, the two hemi-pouches were tubularised and anastomosed [Figure 1]a and [Figure 1]b obtaining a 28 cm long colonic segment. In 3/5 cases the pouch colon was pulled through via the posterior sagittal approach while 2 patients are still awaiting for definitive correction. The postoperative recovery was uneventful for all cases. Following our center policy, all patients underwent spinal magnetic resonance imaging at 1 year of life to detect spinal cord anomalies. In 3/5 tethered cord was revealed without related symptoms. However, the patients with spinal cord anomalies were followed up with periodical clinical evaluations of lower urinary tract symptoms and renal bladder ultrasound to detect urinary tract dysfunction. The median of long-term follow-up was 86 months (range 12–180 months) during which 3 out of 5 patients had a good bowel control with daily bowel management after ARM correction.
Figure 1: Complete congenital pouch colon with recto-bladder fistula. The congenital pouch colon which was fed by two vascular pedicles (a) was split medially. The two Emi-pouches were then tabularised over a Hegar dilator size 11 and anastomosed (b)

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   Discussion Top

CPC was first described by Trusler et al. in 1959.[14] In India, it affects 7-9% of children with ARM, while in Western Countries, a pediatric surgeon can hardly encounter more than a single case during his/her career.[15],[16] In 2005, CPC had been accepted and introduced in the International Classification of ARM (Krickenbeck) as a regional variant of ARM.[17]

The association of CPC and ARM may be initially suspected from the plain abdominal X-ray even if the definitive type of malformation and the corrections' plan can only be determined at surgical exploration.[18],[19]

The left-side “hockey stick” incision has been considered as the optimal route to define the anatomy and correctly classify the malformation.[19] We approached one patient with this incision at the time of definitive correction. This allowed optimal view and space for good mobilization of the pelvic structures.

The main debate in the treatment of CPC is the fate of the pouch itself.

There is not yet a clear agreement about the surgical management of the dilated colon also on the basis of the contractile and histopathologic patterns. The pouch colon is thought to be an abnormal developed tissue suggesting its resection, to reduce the risk of complications (i.e. pouch redilation, soiling, incontinence, or constipation).[6],[7],[20],[21] Gangopadhyay et al. recently described, in a large series, various histologic abnormalities, in the muscle layer of the pouch, strongly suggesting to remove the abnormal colon.[8] Similarly, Tyagi et al.[9] advised to remove the pouch based on lacking of natural spontaneous contractions and histologic anomalies such as muscular abnormalities and decreased/absence of ganglion cells.

However, the existing literature could not identify outcomes differences related to pathology findings. Furthermore, the histological analysis of our cases did not report microscopic abnormalities and showed normal ganglion cells representation. Still, some of the abnormalities described by Gangopadhyay et al.[8] can be secondary to the obstruction and no data exist on the long-term histology changes if the pouch is left in place or refashioned. Remodeling the pouch colon, with a staged or direct pull-through, have been described as technically feasible and safe from many authors.[22],[23]

The current literature did not offer comparison between cases treated maintaining the pouch tissue and those, which have the pouch resected, in terms of both short- or long-term outcome.

We searched scientific databases (PubMed, Cochrane, Embase and Web of Science MeSH) using the keywords “CPC” until December 2017. Search criteria were english language, cases description sufficient for malformation's type definition (according to Saxena Classification), description of the surgical treatment and outcome measure.[4] We founded 56 studies describing 619 cases [Table 2].
Table 2: All studies included in the review

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In 326/619 cases pouch was spared whereas in 293/619 pouch was resected [Table 3] and [Table 4] respectively]. Only 19 paper for a total of 93 patients (63 saved pouch, 30 resected) reported follow-up details [Table 2]. Median length follow-up, when reported, was 18 months for resected pouch and 57 months for saved pouch group. 53/63 patients of spared pouch group (84%) and 21/30 patients of resected pouch group (70%) were dependent from bowel management (irrigation) and/or affected by soiling. This difference does not reach statistical significance. Furthermore, due to the wide discrepancy in terms of reporting complications, meaningful conclusion cannot be drafted by comparing the published series.
Table 3: Follow-up in patients where pouch colon was spared

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Table 4: Follow-up in patients where pouch colon was resected

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Our patients after the stoma reversal have a good bowel control using daily bowel management.

Several authors[10],[11],[12] suggested a tubular reduction of the dilated pouch avoiding its complete resection in order to preserve the absorptive function. The anatomic basis of this procedure is the presence of the double vascular arcades, which allows pouches splitting and lengthening. Trusler et al. first described the procedure on the dilated colon, obtaining a longer, tubular structure, with normal caliber, to preserve absorption properties.[14] Although our series is limited, the double vascular system of the dilated pouch allowed increasing of the bladder capacity (case 4), reconstruction of the vagina (cases 3 and 5), and lengthening of the colon (case 2 and 4).

Some authors showed the advantages to split the pouch to reconstruct a neo-vagina and/or to augment the bladder capacity. Peña et al. first described vaginal reconstruction using the rectum.[24] Following this principle, Wester et al. reported two cases of vaginal reconstruction by longitudinal splitting of the CPC using the redundant part of the pouch wall to create the vagina and recently Chadha et al. suggested to use a segment of the CPC for bladder augmentation.[25],[26] In two cases from our case series (case 3 and case 5) a part of the pouch colon was used to reconstruct a neo vagina. No solid data are available to support these approaches but, from our experience the reconstruction of the vagina using a part of the dilated colon was effective. The case 3 of 7 years has a good bowel control with daily bowel management. Secretion of the neo-vagina is complained even tough improving. The case 5 is in a good compensation even if she is awaiting for further corrective surgery.

In the fourth case part of the pouch was used to augment the bladder capacity and part to lengthen the colon. We believe that in such a complex case, even with a short follow-up, the decision to take advantage of extra tissue to augment the bladder capacity and convert an ileostomy in permanent colostomy, turned out to be important for patient management.

Re-dilatation requiring surgical re-intervention (retubularization or resection) occurred in 24/63 (38%) patients who had pouch used.[11],[15] However, despite re-dilatation needing re-intervention accounts as a complication, there were not significant differences between the two groups in terms of incidence of complications.

This is confirmed by our research that did not show significant differences in terms of short- and long-terms complications between the two groups of treatment. Most of the CPC cases are associated with complex ARM malformation, which quite often requires some bowel management.

Furthermore, there were no difference in the mortality between the two groups and rate of mortality in CPC is not different from mortality of severe ARM.

   Conclusions Top

The removal or the preservation of the abnormal colon tissue seems to follow the surgeon preferences and apparently does not influence negatively the outcome. However, we encourage to tailor the corrections technique on the basis of the anatomy and to consider the use of pouch tissue in particular to contribute to Genito-urinary reconstruction.

Informed consent

Patients' consent was obtained for the surgery. It was a retrospective study. Data were collected from the old records.

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

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Singh S, Pathak IC. Short colon associated with imperforate anus. Surgery 1972;71:781-6.  Back to cited text no. 2
Narasimharao KL, Yadav K, Mitra SK, Pathak IG. Congenital short colon with imperforate anus (pouch colon syndrome). Ann Pediatr Surg 1984;1:59-67.  Back to cited text no. 3
Saxena AK, Praveen M. Classification of congenital pouch colon based on anatomic morphology. Int J Colorectal Dis 2008;23:635-9.  Back to cited text no. 4
Gupta D, Sharma S. Congenital pouch colon-then and now. J Indian Assoc Pediatr Surg 2007;12:5-12.  Back to cited text no. 5
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  [Figure 1]

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


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