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Table of Contents   
CASE REPORT
Year : 2015  |  Volume : 20  |  Issue : 3  |  Page : 148-149
 

Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus


Department of Paediatric Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Web Publication18-Jun-2015

Correspondence Address:
Dr. Vaibhav Pandey
Department of Paediatric Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.154665

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   Abstract 

Congenital pouch colon (CPC) in the female patient presents with highly variable and anomalous anatomy. We herein report the first case of CPC with uterus didelphys having normal anal opening, H-type vestibular fistula, two other fistulous communications between pouch colon and two vagina managed in a single stage with excellent postoperative outcome.


Keywords: Congenital pouch colon, H-type fistula, triplet fistula, uterus didelphys


How to cite this article:
Pandey V, Gangopadhyay AN, Gupta DK, Sharma SP. Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus. J Indian Assoc Pediatr Surg 2015;20:148-9

How to cite this URL:
Pandey V, Gangopadhyay AN, Gupta DK, Sharma SP. Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus. J Indian Assoc Pediatr Surg [serial online] 2015 [cited 2019 Dec 10];20:148-9. Available from: http://www.jiaps.com/text.asp?2015/20/3/148/154665



   Introduction Top


There are few detailed reports in the literature describing the anomalous clinical anatomy of congenital pouch colon (CPC) in female patients. [1],[2] The terminal fistula of the colonic pouch opens at variable levels. It can open into the urethra or in the vestibule at a high or a low level. In most cases, the colonic pouch has been reported to terminate in a fistulous communication with either the vagina or a persistent cloaca. [1],[2] We herein report the first case of CPC with normal anal opening with H-type vestibular fistula. In addition, there were two other fistulous communications between pouch colon and double vagina, making it a case of CPC with triplet fistula with normal anus.


   Case Report Top


A 16-year-old female presented with complaints of passage stool or flatus through the vestibule since birth. She had this complaint since birth, and there was no definite history of vulvar inflammation and fecal leakage from vestibule thereafter. Ultrasonography of abdomen and pelvis showed hugely dilated pelvic bowel loops with inability to visualize rest of pelvic structures. There were no other associated congenital anomalies of the VACTERL type. Bowel preparation was performed with a liquid diet for 1 day, and saline enemas the night before and on the morning of operation and child was taken for examination under anesthesia (EUA). At EUA, four external opening were found in the vestibule, one urethral, two vaginal and one in the lower part of the vestibule communicating with the rectum (1 st fistula) [Figure 1]a and b. Per rectal examination revealed hard fecolith confined to anterior rectal wall, and there was no rectal stenosis. Probe was passed in the fistula and incision was made on the vestibular side of opening. Fistula was excised as that of low H-type fistula (perineal canal). However on bimanual examination it was found that a fecolith was impacted outside the lumen of the rectum, between the rectum and duplex vaginas at a higher level. Mobilization of rectum was performed through anterior perineal incision revealed two more fistulous communications, one each in the duplex vaginas with tracts going higher up. A lower midline laparotomy was also performed which revealed didelphys uterus, a type III pouch colon having fistulous communications from pouch to right vagina (2 cm × 1 cm in size, 2 rd fistula), terminal end of pouch communicating with left vagina (2 cm × 1 cm in size, 3 rd fistula) and distally opening as normal anus [Figure 2]. The fistulas were divided, vaginas were repaired and a complete rectum was mobilized. Excision or pouch colon and pull through of normal colonic segment were performed. Postoperative fasting and intravenous antibiotics were continued for 5 days. The perineum was kept clean and dry by frequent local cleansing. In a follow-up at 1-year child has normal looking perineum, normal vagina with voluntary bowel movements, no soiling and no constipation.
Figure 1: (a) Vestibule showing H-type fistula. (b) Vestibule showing two vaginal openings


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Figure 2: Diagrammatic representation of the anatomy with triplet fistula, normal anus and type III congenital pouch colon


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   Discussion Top


Congenital pouch colon is an anomaly associated with anorectal malformations (ARMs) in which the whole or part of the colon is replaced by a dilated pouch, which may be blind or communicates distally with the urogenital tract. [1],[2] In female patients, the anatomy of the anomaly is more variable, perhaps because of the additional role played by the Müllerian duct system in the development of the internal genital organs, lower urinary tract, and the external genitalia. [2],[3] CPC associated with ARM is rare worldwide, but is common in certain parts of India. [1] Similarly, the incidence of H-type rectovestibular or rectovaginal fistulas for unknown reasons is greater in India and Asia and has been reported to be as high as 7-14% of all ARMs in females. [4],[5] The etiology of H-type fistulas is unclear, and it is generally thought to be congenital in cases associated with ARMs and in cases where a preceding perineal inflammation is absent. [6] In most cases, the colonic pouch has been reported to terminate in a fistulous communication with either the vagina or a persistent cloaca. [7] In other reports, the colonic pouch has been described as ending in a vestibular fistula, a colovesical fistula, or, as in one case, a perineal fistula. [7],[8] Our case was a girl with normal anus and presented at post puberty. We managed our case in a single stage with continence in follow up. Only a few cases have been reported where presentation was in adolescent, and all previously reported series have reported a staged management in neonatal or early childhood. [7]


   Conclusion Top


All such cases need reporting owing to their complicated anatomical variations for a better understanding of anatomy and management. Single stage management can be done with good postoperative outcome.

 
   References Top

1.
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. 1
[PUBMED]    
2.
Puri A, Chadha R, Choudhury SR, Garg A. Congenital pouch colon: Follow-up and functional results after definitive surgery. J Pediatr Surg 2006;41:1413-9.  Back to cited text no. 2
    
3.
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. 3
    
4.
Rintala RJ, Mildh L, Lindahl H. H-type anorectal malformations: Incidence and clinical characteristics. J Pediatr Surg 1996;31:559-62.  Back to cited text no. 4
    
5.
Chatterjee SK, Talukder BC. Double termination of the alimentary tract in female infants. J Pediatr Surg 1969;4:237-43.  Back to cited text no. 5
[PUBMED]    
6.
Tsuchida Y, Saito S, Honna T, Makino S, Kaneko M, Hazama H. Double termination of the alimentary tract in females: A report of 12 cases and a literature review. J Pediatr Surg 1984;19:292-6.  Back to cited text no. 6
[PUBMED]    
7.
Chadha R, Choudhury SR, Pant N, Jain V, Puri A, Acharya H, et al. The anomalous clinical anatomy of congenital pouch colon in girls. J Pediatr Surg 2011;46:1593-602.  Back to cited text no. 7
    
8.
El Shafie M. Congenital short intestine and cystic dilatation of the colon associated with ectopic anus. J Pediatr Surg 1971;6:76.  Back to cited text no. 8
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    Figures

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
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