|Year : 2016 | Volume
| Issue : 3 | Page : 125-128
Congenital pouch colon with colovaginal fistula and rectal atresia in a female: An unusual presentation and literature review
Charu Tiwari, Hemanshi Shah, Jayesh Desale, Mukta Waghmare
Department of Paediatric Surgery, TNMC and BYL Nair Hospital, Mumbai Central, Mumbai, Maharashtra, India
|Date of Web Publication||14-Nov-2016|
Department of Paediatric Surgery, TNMC and BYL Nair Hospital, Mumbai Central, Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
Congenital pouch colon (CPC) is a pouch-like dilatation of the colon in cases of high anorectal malformation. It is common in males with male to female ratios ranging from 2.5:1 to 7:1 in various series. In females, CPC is usually associated with cloaca. Only four cases of CPC with rectal atresia (RA) are reported in literature, one being in a female child. We report a 5-day-old female neonate with urogenital sinus and RA who presented with pneumoperitoneum and sepsis. At exploration, she had Type I pouch with two large perforations, absent appendix, a large fistula to the vagina with a bicornuate uterus which was distended with gas and meconium.
Keywords: Congenital pouch colon, female, rectal atresia, urogenital sinus
|How to cite this article:|
Tiwari C, Shah H, Desale J, Waghmare M. Congenital pouch colon with colovaginal fistula and rectal atresia in a female: An unusual presentation and literature review. Saudi Surg J 2016;4:125-8
|How to cite this URL:|
Tiwari C, Shah H, Desale J, Waghmare M. Congenital pouch colon with colovaginal fistula and rectal atresia in a female: An unusual presentation and literature review. Saudi Surg J [serial online] 2016 [cited 2021 May 14];4:125-8. Available from: https://www.saudisurgj.org/text.asp?2016/4/3/125/194003
| Introduction|| |
Congenital pouch colon (CPC) is a complex congenital malformation of the colon in which the entire colon or segments of varying lengths of the colon exhibit enormous dilatations in the form of a pouch and communicate with the urogenital system distally through a large fistula. ,,,, It is commonly associated with high variety of anorectal malformations (ARM) and is classified as a rare variant in Krickenbeck classification of ARM.  It is prevalent in Asia, especially in Northern India, Pakistan, and Nepal.  Although more frequent in males, CPC in females carry special emphasis because of association with wide variety of complex genitourinary anomalies. , Limited literature is available on females with the pouch colon. , The categorization of these patients based on anatomy is lacking.  Consequently, a clear-cut management protocol is not available.
Rectal atresia (RA) is also a rare entity. , Association of CPC with RA is very rare. Only four cases have been reported in the literature.  We report a female neonate with RA and urogenital sinus with Type I CPC.
| Case Report|| |
A 5-day-old female, 1.9 kg, was referred for abdominal distention and passage of stools from an abnormal opening since birth. The child had gross abdominal distention, weak peripheral pulses, and tachypnea [Figure 1]. She had two openings in the perineum: A single large opening in the introitus (suggesting a urogenital sinus) and a normally placed anal opening [Figure 2]. She was passing small amounts of stools from the abnormal single opening in the introitus since birth along with urine. She had no history of passing stools per anum. The anal opening permitted only 3.5 cm of a red rubber catheter suggestive of RA.
|Figure 2: Local examination showing a single large opening in the introitus (suggesting a urogenital sinus) with a normally placed anal opening|
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Erect abdominal radiograph revealed massive pneumoperitoneum. The vertebral column and sacrum were normal. A peritoneal drain was inserted at the bedside to relieve respiratory distress.
Exploration revealed a large 5 cm × 5 cm Type I pouch colon with two large perforations. Appendix was absent. A large fistula to the vagina was present along with a bicornuate uterus which was distended with gas and meconium [Figure 3] and [Figure 4]. In view of the poor general condition of the baby, the pouch was excised and end ileostomy done. Unfortunately, patient succumbed on day 3 secondary to sepsis. Histopathology confirmed the diagnosis of pouch colon with perforation.
|Figure 3: Intraoperative image showing the bicornuate uterus which was distended with gas and meconium and fistula|
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| Discussion|| |
After the first description of CPC by Spriggs in 1912, the anatomical details and various classifications of CPC were given in detail by Narasimharao et al., Wakhlu et al., and Chadha et al. ,, Though extensively described in males, literature regarding the anatomical details in females is lacking.
Singh and Pathak coined the term "short colon" in 1972. , CPC was classified into five types by Wakhlu and Pandey in 1996. , The term "Pouch Colon Syndrome" was suggested by Narasimharao et al.  The anatomical classification suggested by them based on the length of the normal colon present proximal to the dilated pouch has been widely accepted. ,,, This classification of CPC was simplified as "partial short colon" (Type A) with the presence of >8 cm of normal colon proximal to the pouch and "complete short colon" (Type B) with the absence of normal colon or <8 cm of colon proximal to the pouch by Wakhlu and Pandey in 1996. , Gupta and Sharma have classified CPC into two types in 2005 as "Complete CPC" and "incomplete CPC" depending upon the presence or absence of the adequate normal colon for performing the pull-through operation. , CPC cases were classified into five types on the basis of anatomic morphology by Saxena and Mathur in 2008. , CPC has now been accepted and included in the new International Classification (Krickenbeck) of ARM in 2005, as rare anomalies (being only regional). ,
In females, CPC is commonly associated with cloaca. Most cases present in the early neonatal period with an absent anal opening and abdominal distention.  Bilious vomiting with early gross abdominal distention in a case of ARM strongly suggests a CPC.  There may be double or septate vagina. The fistulous communication may open in one of the hemivaginae or between the two into the cloaca. Colocloacal fistula is the most common, followed by colovaginal and colovestibular fistulas.  Occasionally in case of a large fistula as in a case of a large colocloacal or colovestibular fistula, the presentation may be late as the child remains decompressed. 
In cases with perforation that occurs early in cases of grossly distended pouch colon, the baby may present with septicemia, gross abdominal distention with prominent veins, fluid and electrolyte imbalance, and features of peritonitis.  Sometimes, the baby may present with complications such as stenosis or prolapsed of colostomy performed by a surgeon who was unaware of this condition. 
A large loop of bowel with a single air fluid level occupying more than half of the total width of the abdomen on the plain erect abdominal X-ray is diagnostic of CPC. , The pouch is proximal to the pubococcygeal line in the cross table lateral prone X-ray.  The diagnosis can be missed in cases of incomplete pouch and perforation.  False diagnosis can be made in cases with significant sigmoid colon dilatation and large rectouterine fistula where the massive dilatation of the uterus with meconium and gas may mimic CPC. 
The incidence of RA is 1-2%. , However its association with CPC is rare with only four cases described in literature. ,,, Out of the multiple theories proposed to explain the etiology of CPC, the etiology of early intrauterine insult of inferior mesenteric artery holds well for its association with RA as the latter also results from intrauterine vascular insult to the branches of internal iliac arteries. ,,
The theories by Chadha et al. stated that varying extents of vascular insult at the time of the partitioning between the cloaca by the urorectal septum  and by Wakhlu et al. stated that CPC represents a stage in the development of cloacal exstrophy and is the combined effect of defective development of the splanchnic layer of the caudal fold and failure of rotation of gut causing defective longitudinal growth of the colon  - may help to explain the association of UGS with CPC in this patient.
RA with CPC was first reported in 2002 by Mathur et al.; it was a Type II CPC with a fistula to the urinary bladder along with RA in a male child. , Colostomy was performed after which death ensued. The second case was reported by Kazez et al. in 2009; it describes a male baby with CPC Type IV and RA without genitourinary fistula. He was managed by a single-stage abdomino-transanal-endorectal pull-through.  The third case was reported in 2010 by Parelkar et al.; a female baby with Type IV CPC and RA was described who was managed successfully by staged repair. There was no communication of the pouch to the genitourinary system.  The fourth case was described in 2011 by Singh et al. in a male baby. He had an intermediate CPC (between 1 and 2) with a proximal fistula to the genitourinary tract. The distal end of the pouch was free. He was also managed by staged repair. 
Management of pouch colon is controversial with poor long-term result and outcome.  The choice of the various available procedures depends upon the choice of the surgeon, condition of the baby at presentation, technical skill of the surgeon, and the available facilities. ,,,, The aim of the surgery in CPC is to utilize the available length of colon for absorption and storage capacity as well as capability for propelling fecal matter onward with a continent anal opening. ,,,,
The various procedures which are described in a case of CPC include (a) proximal colostomy, window colostomy, or ileostomy with or without fistula division (b) division of fistula, excision of pouch, and end colostomy (c) division of fistula, coloplasty, and end colostomy. ,,,, These procedures are done in neonatal period and are followed by one of the definite procedures such as (1) pouch excision and AP pull-through of colon (2) pouch excision with abdominoperineal posterior sagittal anorectoplasty (PSARP) (3) division of fistula, coloplasty, and AP pull-through of coloplasty colon with proximal ileostomy and (4) pouch excision and AP pull-through of ileum followed by ileostomy closure. ,,,, Another technique known as the hanging bowel technique has recently been described. 
Various surgical procedures, either primary or staged, have been described in literature to manage the association of RA with pouch colon.  The primary procedure is fistula ligation, coloplasty and abdomino-transanal-endorectal pull-through and colo-anal anastomosis.  The proposed staged procedures include the excision of colonic pouch with ileostomy; window colostomy; end colostomy; transverse colostomy; ligation of fistula procedure alone or combined with coloplasty in the first stage. The definitive surgery is abdomino-PSARP or endorectal pull-through and colo-anal anastomosis preserving native anal sphincter complex.  In case of gross abdominal distention and hemodynamic instability of the neonate, staged approach is better and safe. , It has been suggested that colovesical fistula ligation should always be done in the first stage to prevent the lethal complications such as urinary tract infection and hyperchloremic metabolic acidosis. 
The association of CPC with RA is rare and more so in females. Both primary and stages procedures have been suggested. Staged procedure is safer in hemodynamically unstable neonates.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]