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ORIGINAL ARTICLE
Year : 2007  |  Volume : 12  |  Issue : 1  |  Page : 13-16
 

Experience with 40 cases of congenital pouch colon


Department of Pediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals Bhopal, MP - 462 001, India

Correspondence Address:
R K Ghritlaharey
Department of Pediatric Surgery, Gandhi Medical College and Associated Kamla Nehru and Hamidia Hospitals, Bhopal, MP - 462 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.31082

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   Abstract 

Aim : The aim of this study was to analyze the clinical and management aspect of congenital pouch colon. Materials and Methods : This retrospective study was carried out on 40 cases of congenital pouch colon managed in the department of paediatric surgery from 01, January 2000 to 31, December 2005. Results : The incidence of congenital pouch colon (CPC) in the present study was 11.290 % (40 of 354) of all anorectal malformations (ARM) and 19.60% (40 of 204) of high ARM. Of these 40 cases of CPC included in the study, 82.5% were male and 17.5% were female. The average age of presentation to hospital was 3.36 days with a range of 1-11 days; of these 25% patients were admitted with poor general condition. Preoperative diagnosis of CPC was possible in 75% of cases in our study. 60% ( n =24) of cases had incomplete pouch colon (Type III and IV) while 40% ( n =16) had complete pouch colon (Type I and II). Right transverse colostomy was done in 16 patients and colostomy at descending colon just proximal to pouch in two patients for incomplete (Type III and IV) CPC as a primary initial procedure. Ileostomy was done in 14 patients and window colostomy in three patients for complete (Type I and II) CPC. As definitive procedures, pouch excision and abdomino-perineal pull-through of colon was done in 17 patients, coloplasty and abdomino-perineal pull-through of coloplasty colon was done in four patients and pouch excision and abdomino-perineal pull-through of terminal ileum in four patients in our series. None of our patients underwent primary single stage procedure. Conclusion: (1) CPC comprises 11.290 % of all ARM and 19.60% of high ARM. (2) Right transverse colostomy was opted for incomplete pouch colon, while Ileostomy and window colostomy were opted for complete pouch colon as initial surgical procedures. (3) Staged surgical procedures for management of CPC were well tolerated in our series.


Keywords: Anorectal malformation, congenital pouch colon, congenital short colon, staged surgical procedures


How to cite this article:
Ghritlaharey R K, Budhwani K S, Shrivastava D K, Gupta G, Kushwaha A S, Chanchlani R, Nanda M. Experience with 40 cases of congenital pouch colon. J Indian Assoc Pediatr Surg 2007;12:13-6

How to cite this URL:
Ghritlaharey R K, Budhwani K S, Shrivastava D K, Gupta G, Kushwaha A S, Chanchlani R, Nanda M. Experience with 40 cases of congenital pouch colon. J Indian Assoc Pediatr Surg [serial online] 2007 [cited 2019 May 24];12:13-6. Available from: http://www.jiaps.com/text.asp?2007/12/1/13/31082



   Introduction Top


Congenital pouch colon (CPC) is defined as a condition in which whole or part of the colon is replaced by a pouch like dilatation and communicates with the urogenital tract by means of fistula. CPC is a variety of anorectal malformations (ARM), which is also known as congenital short colon.[1],[2] A CPC-like condition was first described in 1912 by Spriggs in London hospital museum specimen, but it was not named at that time. Trusler reported a pouch colon in 1959 from Canada. In 1972 Singh and Pathak from India reported six cases and named this anomaly as 'Short Colon'.[2] Narsimha Rao et al . in 1984 suggested the name as 'Pouch Colon syndrome' and also proposed an anatomical classification of the condition, which has been widely accepted.[2] The incidence of CPC among all cases of ARM has been reported to occur from 2 to 15.1%.[3],[4],[5] Almost all the series reported male preponderance. We are reporting our experience with management of 40 cases of CPC with a brief review of literature.


   Materiala and Methods Top


This retrospective study includes the cases of congenital pouch colon admitted in the department of paediatric surgery between 01, January 2000 and 31, December 2005, clinically diagnosed or by laparotomy. Details of clinical presentation, diagnostic work up and operative procedure done at various stages were studied in details. Plain skiagram of abdomen/invertogram was the initial and sole investigation that was needed. Ultrasonography of abdomen and pelvis were also done in all the patients either during initial admission or during follow-up periods. Distal cologram was done in all the cases before definitive procedure.


   Results Top


In our study of 40 cases, CPC accounts for 11.290 % of all ARM and 19.60% of high ARM [Table - 1]. We observed male predominance in our series as 4.7-1 in favour of male. All male children presented with an absent anal opening with abdominal distension and female children with a passage of meconium from abnormal opening. Twenty-five male patients had a history of passage of meconium in urine. The average age on presentation was 3.36 days with ranges from 01 to 11 days; females presented later. We were able to diagnose CPC preoperatively in 3/4th of cases [Table - 1]. All males ( n =33) had associated colovesical fistula. Among female patients ( n =7) two had colo-vaginal fistula, two had colo-vestibular fistula and three had colo-cloacal fistula.

Five male patients of our series presented with colostomy done by a general surgeon at another hospital with a diagnosis of high ARM with colostomy. Various operative procedures performed in 40 cases of CPC are given in [Table - 2].

In a two-staged procedure, we for opted ligation of fistula, excision of pouch and abdomino-perineal pull-through of colon without proximal protective colostomy for definitive procedure. Eight of our patients are waiting for definitive procedure. None of our patients underwent single-staged procedure for CPC.

The complications we encountered were skin excoriation in seven cases, wound infection in five, anal stricture in two, anal mucosal prolapse in two, colostomy prolapse in three, faecal incontinence in one, stoma necrosis in one and burst abdomen in one. We observed 17.5% deaths (7 of 40 patients) in our series on initial procedure, which were due to delayed presentation with poor general condition. Three patients who presented with peritonitis with free gas under diaphragm had poor general condition on admission and they finally died. Two patients who needed re-explorations, one for stoma necrosis and another for burst abdomen, also died due to septicaemia. One patient died of respiratory failure and another died of anaesthetic complication.


   Discussion Top


Trusler reported the first case of pouch colon in 1959 from Canada. Singh and Pathak from India in 1972 reported the first series of six cases of CPC. This anomaly is seen much more frequently in Northern, North Western and Central part of India. Few cases have also been reported from other parts of the world and Western part of India.[2],[4],[5],[6],[7],[8],[9],[10],[11],[12] The cause of this unique geographical distribution is yet to be ascertained. The exact cause and embryogenesis of CPC is not known. In 1967, Dickinson described that interference in the longitudinal growth of the hindgut distal to allantois and failure of its migration into pelvis in early embryonic life might result in short colon.[12]

A large air fluid level occupying more than half of the width of the abdomen on plain skiagram either erects or inverted position is a preoperative diagnostic of CPC.[12],[13],[14]

Various options are available for the management of CPC with their own merits and demerits.

Classical/standard management of CPC consists of staged surgical procedures as follows:[15],[16],[17],[18]

  1. Initial operative procedure: It includes (a) proximal colostomy, window colostomy or ileostomy with or without fistula division, (b) division of fistula, excision of pouch and end colostomy, and (c) division of fistula, coloplasty and end colostomy.
  2. Definitive operative procedure: It includes (1) pouch excision and abdomino-perineal pull-through of colon, (2) pouch excision and posterio sagital anorectoplasty (PSARP), (3) division of fistula, coloplasty and abdomino-perineal pull-through with proximal ileostomy, (4) division of fistula, coloplasty and sacro-abdomino-perineal pull-through, (5) Division of fistula, coloplasty // colorrhaphy and PSARP, and (6) pouch excision and abdomino-perineal pull-through of ileum.
  3. Colostomy/ileostomy closure:

    In 2005, primary single-staged management of all types of congenital pouch colon in a newborn was recommended with better continence and cosmesis with a low morbidity and mortality at a low cost.[5] Recently a patch of pouch colon was successfully used over a pull-through ileum in a patient with long segment pouch colon. This technique was based on the theory and experiences of use of patch of colon in the management of total colonic aganglioneurosis.[19] In a patient with CPC associated with vaginal atresia, a rare occurrence, vagina and ano-rectum can be reconstructed by longitudinal splitting of pouch colon.[20]


In an analysis of histopathological examination of excised pouch for the management of CPC, the most frequent findings were acute and chronic inflammations of the mucosa and submucosa, focal or generalized thinning of muscle layers, disorganized muscle layers, decreased number of mature ganglion cells, neuronal hyperplasia and hypertrophy in nerve plexuses. The above neuromuscular abnormalities explain a weak or poor peristalsis as well as propensity to undergo marked dilatation even after tubularization.[21],[22] We also observed similar histological findings of excised pouch colon submitted for histological examinations in our series.

Associated anomalies are reported to occur with CPC. In the present study of 40 cases of CPC, 40% (16 of 40) had associated congenital anomalies other than genitourinary fistula and were mostly related to gastrointestinal and genitourinary systems. The congenital anomalies observed by us were Meckel's diverticulum, double appendix, hypospadias, bicornuate uterus, etc.

Complications are known to occur with CPC whether managed by multiple stage or single stage procedures and include skin excoriation, prolapse, stenosis, diarrhoea, constipation, anaemia, malnutrition, etc.[2],[5],[23]

In the management of CPC, earlier series reported mortality as high as 20-45% among cases, which was due to delayed presentation with poor general condition.[4],[24] Recent series has reported only 10-15% of mortality.[5],[25]

 
   References Top

1.Wakhlu AK, Wakhlu A, Pandey A, Agarwal R, Tandon RK, Kureel SN. Congenital short colon. World J Surg 1996;20:107-14.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Wakhlu AK, Pandey A. Congenital pouch colon. Text Book of Neonatal Surgery by D K Gupta. Modern Publishers: New Delhi; I Ed 2000. p. 240-8.  Back to cited text no. 2    
3.Chadha R, Bagga D, Malhotra CJ, Mohta A, Dhar A, Kumar A. The embryology and management of congenital pouch colon associated with anorectal agenesis. J Pediatr Surg 1994;29:439-46.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Gangopadhyay AN, Gopal SC, Gupta DK, Sinha CK. Management of pouch colon with anorectal malformation. J Indian Assoc Pediatr Surg 1996;1:55-9.  Back to cited text no. 4    
5.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. 5  [PUBMED]  [FULLTEXT]
6.Chadha R, Bagga D, Mahajan JK, Gupta S. Congenital pouch colon revisited. J Pediatr Surg 1998;33:1510-5.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.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  [PUBMED]  [FULLTEXT]
8.Kumar A, Agarwala S, Srinivas M, Bajpai M, Bhatnagar V, Gupta DK, et al . Anorectal malformations and their impact on survival. Indian J Pediatr 2005;72:1039-42.  Back to cited text no. 8    
9.Herman TE, Coplen D, Skinner M. Congenital short colon with imperforate anus (pouch colon): Report of a case. Pediatr Radiol 2000;30:243-6.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Denes J, Ziszi K, Bognar M, Schlaffer E, Toth J. Congenital short colon associated with imperforate anus (Zachary-Morgan syndrome). Acta Paediatr Hung 1984;25:377-83.  Back to cited text no. 10  [PUBMED]  
11.Arestis NJ, Clarke C, Munro FD, Micallef C, O'Sullivan MJ. Congenital pouch colon (CPC) associated with anorectal agenesis: A case report and review of literature. Pediatr Dev Pathol 2005;8:701-5.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Shenoy MU, Kulkarni MS, Bhagwat AD, Kapur VK. Short colon variant. J Postgrad Med 1995;41:107.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Singh A, Singh R, Singh A. Short colon malformation with imperforate anus. Acta Paediatr Scand 1997;66:589-94.  Back to cited text no. 13    
14.Humberto L Lugo-Vincente. Congenital pouch colon. Pediatr Surg Update 2001;16:3.   Back to cited text no. 14    
15.Wakhlu AK, Pandey A, Wakhlu A, Tandon RK, Kureel SN. Coloplasty for congenital short colon. J Pediatr Surg 1996;31:344-8.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Budhiraja S, Pandit SK, Rattan KN. A report of 27 cases of congenital short colon with an imperforate anus: So-called pouch colon syndrome. Trop Doct 1997;27:217-20.  Back to cited text no. 16  [PUBMED]  
17.Sharma AK, Harjai MM. Simplified colorrhaphy and posterior sagital anorectoplasty for the management of congenital short colon with imperforate anus. Br J Surg 1997;84:389.  Back to cited text no. 17  [PUBMED]  
18.Chadha R, Gupta S, Mahajan JK, Bagga D, Kumar A. Congenital pouch colon in females. Pediatr Surg Int 1999;15:336-42.  Back to cited text no. 18  [PUBMED]  [FULLTEXT]
19.Ratan SK, Rattan KN. Pouch colon patch graft: An alternative treatment for congenital short colon. Pediatr Surg Int 2004;20:801-3.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]
20.Wester T, Lackgren G, Christofferson R, Rintala RJ. The congenital pouch colon can be used for vaginal reconstruction by longitudinal splitting. J Pediatr Surg 2006;41:e25-8.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]
21.Agarwal K, Chadha R, Ahluwalia C, Debnath PR, Sharma A, Roy Choudhury S. The Histopathology of congenital pouch colon associated with anorectal agenesis. Eur J Pediatr Surg 2005;15:102-6.  Back to cited text no. 21  [PUBMED]  [FULLTEXT]
22.Chadha R, Bagga D, Gupta S, Prasad A. Congenital pouch colon: Massive redilatation of the tubularized colonic pouch after pull - through surgery. J Pediatr Surg 2002;37:1376-9.  Back to cited text no. 22  [PUBMED]  [FULLTEXT]
23.Singal AK, Bhatnagar V. Colostomy prolapse and hernia following window colostomy in congenital pouch colon. Pediatr Surg Int 2006;22:459-61.  Back to cited text no. 23  [PUBMED]  [FULLTEXT]
24.Wardhan H, Gangopadhyay AN, Singhal GD, Gopal SC. Imperforate anus with congenital short colon (pouch colon syndrome): Review of 18 cases. Pediatr Surg Int 1990;5:124-6  Back to cited text no. 24    
25.Sharma S, Gupta DK, Bhatnagar V, Bajpai M, Agarwala S. Management of congenital pouch colon in association with ARM. J Indian Assoc Pediatr Surg 2005;10:S-22.  Back to cited text no. 25    


    Tables

[Table - 1], [Table - 2]


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    Abstract
    Introduction
    Materiala and Me...
    Results
    Discussion
    References
    Article Tables

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