Type I Congenital Colonic Pouch Resection With Terminal Ileostomy in a 43-year-old Woman. Case Report

INTRODUCTION: Congenital colonic pouch syndrome is an extremely rare condition, particularly in Western countries. In this condition, the colon is replaced with a partially or completely abnormal pouch, connected to the genitourinary tract by a �stula or may end in a blind pouch. As it is a congenital condition, the cases reported are found in the neonatal population, so its detection in adulthood is extremely rare. CASE PRESENTATION: We present the case of a 43-year-old female, who was admitted to the general surgery service with suspected �stulizing CROHN's disease. She attended with a two-month clinical picture, presenting a painful ulcer in the left perianal and gluteal region, accompanied by discharge of purulent material and feces through the vaginal introitus. Laboratory tests (CAT, colonoscopy and MRI) did not present conclusive information. An exploratory laparotomy was performed, �nding a congenital type I colonic pouch, so it was resected, and a terminal ileostomy was performed. CONCLUSIONS: The present case aimed to report our experience with a pathology that has not been reported in this particular age group in our country. Imaging studies before surgery were unable to diagnose the pathology, so the exploratory laparotomy was decisive for the treatment.


Introduction
Congenital colonic pouch (CCP) is a rare variant of ano-rectal malformations (ARM), endemic from northern India, where about 92.2% of reported cases originate (1).In this condition, the colon is replaced with a partially or completely abnormal pouch connected to the genitourinary tract by a stula (colovesical).CCP has been included, as a rare variant, in the Krickenbeck classi cation of ARM (2).
There are several anatomical classi cations of the congenital colonic pouch (3), but basically four types are considered based on the extension of the involved colon described by Narasimharao et al. (4).In type I the normal colon is completely absent and the opening of the ileum enters directly into the colonic pouch; in type II the cecum and a segment of normal colon are present proximal to the colonic pouch; in type III the normal proximal colon extends at least to hepatic exure, but does not reach the descending colon; in type IV the descending colon is almost normal and only the terminal portion, the rectum and a variable length of sigmoid, are included within the colonic pouch.The CP, especially in newborns, is thinwalled and distended at the expense of meconium and atulence.However, with a wide stula or in older children, it may be smaller and thick-walled.In Type I/II CCP, the proximal ileum or colon usually enters the pouch from right to left in a low position, close to the stula (5).Subtypes I and II are more common in women.In most cases, the colonic pouch is reported to open into the vagina or into a persistent cloaca.
The aim of this study is to present the clinical case of a congenital colonic pouch type I, diagnosed in a 45-year-old woman who was surgically treated with a terminal ileostomy.
The trans-surgical ndings are detailed as follows: Multiple omentum-abdominal wall adhesions were found, omentum-colon (Congenital colonic pouch) Zulkhe III, IV, V. Congenital colonic pouch type 1, ileocecal valve with junction to the cecum which ends in a blind pouch, where it joins with the sigmoid colon, presenting a dilatation of approximately 20-25 cm, in addition, the absence of the cecal appendix was identi ed (Image No.4).

Discussion And Conclusions
CCP is an unusual anomaly in which a pouch-like dilatation with a variable degree of shortening of the colon is associated with an ARM.The pouch usually ends in a stulous communication with the genitourinary tract.The M: F ratio ranges from 2.25:1 to 7:1 (5).In this study, we present a case of CCP in a female patient.The clinical presentation is usually in the neonatal period with abdominal distension and the presence of an anorectal malformation; there may be fecaluria in 50% of cases in male patients due to communication of the urinary tract with the colonic pouch (6).Our patient has a clinical history of gastrointestinal pathology of several years of evolution, especially linked to CROHN syndrome, so the presence of a colonic pouch had never been suspected.It was not until she was 43 years old that we found it as part of an important nding, after an exploratory laparotomy.This is especially important to consider the possibility that a pathology that is normally expressed and resolved in the neonatal stage, may be present even in adulthood.In addition, it may be masked by other conditions that make its diagnosis and resolution di cult.In a retrospective study by Singh et al., where all cases of colonic pouch, managed over a period of 5 years, were recorded, the age of presentation ranged from 1 to 15 days after birth (7).Of 64 cases reviewed, coloplasty was performed on 34 occasions, with excellent cosmetic and functional results after stoma closure.
In our country the presence of this type of malformation is highly unusual, which makes its diagnostic suspicion very di cult; therefore, an adequate pre-surgical anamnesis can help to plan its approach.However, there is always the possibility that the diagnosis cannot be made until surgical exploration.It should be recalled that our patient was carefully examined with the appropriate laboratory and laboratory studies; nonetheless, the reports obtained were confusing and some even suggested the existence of sequelae of a previous, non-existent surgery.Gallegos et al., present the clinical case of a 2-day-old female whose diagnosis (Congenital colonic pouch) was made until the time of laparotomy (8).Gallegos mentions that, in most cases, radiography may be su cient to establish the diagnosis; however, in the case they present, none of the studies performed helped to establish the diagnosis with certainty.Timely diagnosis, in addition to offering adequate treatment, allows avoiding the multiplicity of approaches aimed at other pathologies, as was the case of our patient who was 43 years old, with treatments focused on treating a stula secondary to CROHN's disease.

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