Imperforated Anus with Recto-Urethral Fistula

• Term fetus in a Latin American country has a prenatal ultrasound that identifies an abdominal cystic mass.
• Cystic abdominal mass was first identified on the 16-wk ultrasound and has been consistently growing.
• Medical team was concerned about fetal peritonitis or lymphatic malformation based on the large size of this mass.
• C-section was performed at term.
• Newborn male had imperforate anus and recto-urethral fistula was suspected.
• No associated malformations were detected.
• Right lower quadrant laparotomy was performed during which it was noted that a huge sigmoid bowel loop occupied the entire lower abdomen.
• Suspicion of colonic atresia. Diverting colostomy was placed in the left colon proximal to the sigmoid loop.
• Recto-bulbar fistula was confirmed by urethrography. At 3.5 mo of age, the baby was brought to the our (Presenter's) country for evaluation.
• On physical exam, an imperforate anus with “good looking” perineum was identified.
• Radiographs of the sacrum were normal.
• Distal colostogram showed proximal sigmoid of normal size, huge dilated section of sigmoid, then huge distal rectum.
• 500 ml of urine was present in the sigmoid.
• Patient was taken to the operating room for surgery. During the procedure, the transurethral Foley catheter was inserted but it repeatedly went into the rectum and the urinary bladder could not be reached.
• There was a right angle between the tiny posterior urethral and bulbar urethra.
• The surgery had to be performed without urethral Foley.
• Suprapubic catheter was inserted postoperatively. He was discharged 48 h after surgery. The suprapubic catheter was removed on postoperative day 8.
• On postoperative day 12 anal dilations were initiated. Stomas were taken down using a transverse laparotomy incision. Plication remodeling of the large sigmoid was performed.
• Bowel management was started immediately.
• Six months after surgery the patient has poor rectal function and daily rectal irrigations are necessary.

Takeaways

• Megasigmoid/ mega rectum could be associated with a fetus with imperforate anus. In this case, the bowel was full of urine coming from the recto-urethral fistula since early in fetal development.
• Primary rectal “ectasia” or “primary” megarectum has been related to constipation, fecal impaction, hypomotility and poor rectal function.
• When facing the surgical treatment of recto-urethral fistula, the insertion of a Foley catheter is mandatory but in some cases, it is not possible.
• Rectal extraluminal remodeling was performed due to social situation for this patient. Otherwise, a rectosigmoid resection would have been performed.
• This patient may require sigmoid resection and antegrade enema procedure in the future.
• Cystoscope with a guide wire could have been used to catheterize the patient.
• Patients with a colon pouch have atypical blood supply.