Congenital pouch colon with ileovesical and colovesical fistulae: A new variant.

We report a case of persistent cloaca and type I congenital pouch colon associated with ileovesical and colovesical fistulae. Such a malformation has not been reported so far.

INTRODUCTION

Congenital pouch colon (CPC) is a complex anorectal malformation (ARM). A number of associated anomalies and variants of CPC have been reported. A big fistula of the pouch colon with the genitourinary system is one of key features of CPC .[1-3] The presence of two fistulae with the genitourinary tract in a patient with CPC, one from the ileum proximal to the colonic pouch and the other from the pouch itself, has not been reported earlier in the literature. We are reporting a case of persistent cloaca and type I CPC associated with two fistulae.

CASE REPORT

A five-day-old female was admitted in a state of shock and not passing meconium since birth. The prenatal period was uneventful and the baby was delivered at home. The patient was initially resuscitated and a nasogastric tube was inserted to decompress the abdomen. She had a single perineal opening and imperforate anus. The abdomen was grossly distended.The abdominal radiograph showed dilated bowel loops. An ultrasound of abdomen revealed dilated bowel loops and a distended urinary bladder.

A small incision was made in the left lower abdomen for colostomy. The colon was not approachable through that incision; therefore, the incision was extended and it revealed a type I CPC lying in the left abdomen. There was no appendix or cecum and the ileum was opening into the CPC and posterior wall of the urinary bladder [Figures 1 and 2]. The CPC was also having a fistula with the posterior wall of the urinary bladder. Both the fistulae were opening into the posterior wall of the urinary bladder and were 1 cm apart. The ileum was separated from the pouch colon and the urinary bladder. The pouch was also disconnected from the urinary bladder. The fistulae were repaired and an end ileostomy formed. The patient remained critical throughout the admission and expired on the third postoperative day.

Figure 1

Operative photograph showing, (1) ileum opening into CPC, (2) ileum opening into urinary bladder, (3) CPC openinginto urinary bladder, and (4) CPC that had been emptied

Figure 2

Line diagram of the anomaly

DISCUSSION

‘Pouch colon syndrome’ or CPC is a severe type of anorectal malformation. Our case was of type I classification (as there was no appendix, and the cecum and ileum were followed by the pouch) according to Rao et al., which was based on the length of the normal colon present .[1] The important characteristics of CPC are its association with ARM, presence of a pouch colon, malrotation, abnormal vascular supply of CPC, absence of appendices epiploicae, and haustrations on the CPC, and a big muscular fistula with the urogenital system, along with gross vesicoureteral reflux. The fistula is usually big and muscular. In males it has been reported to be exclusively between the CPC and the urinary bladder, although a few cases were reported with urethral fistula. In females the fistula has been reported to be between the CPC and cloaca, vestibule, vagina, and urinary bladder. In all cases the fistula is single and occurs between the CPC and the urogenital tract .[134] In our case there were two fistulae; the ileum was opening into the CPC as well as into the posterior wall of the urinary bladder.

Ileovesical fistulae have rarely been reported in literature. Yurtçu et al., have reported a case of total colonic agenesis with ileovesical fistula .[5] In their case the colon was absent. However, in our case the entire normal colon was replaced by a pouch. Our case may reflect an intermediate event in a sequence of development of total colonic agenesis, which is associated with ARM, as reported by Yurtçu et al. A search of Google scholar and Pubmed was performed and no similar case report was found. This may be considered a new variant of CPC.