Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys
A 3-month-old girl was brought with the absence of the anal opening and passage of urine and stools from a single perineal opening. On examination, there was a single, fairly wide, perineal opening, and a clinical diagnosis of a persistent cloaca was made. Laparotomy revealed a type-III CPC malformation with 8 cm of normal colon proximal to the distended colonic pouch that appeared to end in a colocloacal fistula. The child had uterus didelphys with one fallopian tube and uterus flanking the distal portion of the colonic pouch and the colocloacal fistula on each side. The fistula was ligated, subtotal excision of the colonic pouch with tubularization of its outer portion performed (tubular colorraphy) and an end-colostomy fashioned.\nPostoperative abdominal ultrasonogram (US) and intravenous urogram (IVU) were normal. Radiographs showed a normal sacrum. At the age of 15 months, one-stage repair of the cloacal malformation was performed. Initial dissection by the posterior sagittal approach revealed a short but wide common channel (around 2 cm long) with a double vagina. The anterolateral walls of the common channel were tubularized to reconstruct the urethra over an 8 Fr. catheter. With the child in the supine-lithotomy position, a short segment of ileum was isolated, its upper end sutured to the margins of the double vagina and its lower end brought to the perineum. The colostomy was mobilized and brought down to the site of the neoanus. A protective loop-ileostomy was constructed and closed 3 months later.\nThe girl was lost to follow-up for 5 years and brought again with complaints of urinary and fecal incontinence. The child had partial urinary incontinence (UI) with passage of urine in a stream a few times daily along with continuous dribbling of urine. On examination, the urethral and vaginal openings were separate and the vaginal opening was adequate in caliber. The child was advised daily administration of saline enema at home. A micturating cystourethrogram (MCU) showed a bladder of adequate capacity with a patulous bladder neck. The parents were not willing for clean intermittent catheterization or any further surgical management for UI.\nAt 11 years of age, the girl presented with primary amenorrhea and severe monthly cyclical pain in both flanks and the pelvis. Investigations were advised but the child was lost to follow-up for 3 years and brought again only when the symptoms had worsened considerably. A US of the abdomen and pelvis showed well-defined lobulated masses with cystic lesions in the pelvis and both adnexal regions. The ovaries and uteri could not be seen separately from the pelvic masses. A diagnosis of hematometra and hematocolpos was made. Magnetic resonance imaging (MRI) scan of the abdomen and pelvis [] showed 2 uteri and fallopian tubes that were distended and filled with blood and blood products. Poorly developed cervical tissue was seen and could be traced only to the right uterus. The vagina was around 4 cm long. Bilaterally, the adnexal structures appeared to be filled with blood and hemorrhagic cysts.\nEUA with cystourethroscopy showed a separate urethral orifice leading to a short urethra with a widely open bladder neck. Per-vaginal examination showed tense, hard masses on both sides in the pelvis. No cervix could be felt. Laparotomy revealed dense pelvic adhesions, uterus didelphys with both uteruses distended with blood and blood products, bilateral endometrial cysts, and a large left hematosalpinx []. The endometrial cysts were aspirated, the walls of the cysts were enucleated, and bilateral hysterectomy with left salpingectomy performed. There was very poorly developed cervical tissue without a lumen on the right side and complete cervical atresia on the left side. The postoperative period was uneventful. The histopathological examination of the endometrial cysts showed evidence of endometriosis. Sections from one of the uterine horns showed endometriosis while those from the other horn were unremarkable.\nPresently, at the age of 16 years, the girl is relieved from her complaints of cyclical abdominal and pelvic pain. She is able to hold urine during the day for periods ranging from ½ to 2 h but has severe nocturnal UI. The child is dry after an enema for 4-5 h. The child is fairly well-adjusted psychologically. The parents are not willing for any procedure for treatment of UI.