7-Year-Old Female Patient Born with Recto-perineal Fistula

Case Summary

• 7-year-old presents for evaluation with a history of recto-perineal fistula that was repaired as a newborn.
• Her medical records noted that she had a urethral, bladder, and vaginal duplication.
• She presents for evaluation because of persistent stool accidents.
• She was having daily bowel movements, but was also smearing in the underwear.
• Workup included abdominal radiograph, contrast enema, and rectal examination under anesthesia.
• Abdominal X-ray showed large stool burden. Contrast enema showed a very dilated colon. Exam under anesthesia showed foley catheters in the left and right urethra.
• She also had two vaginal openings.
• Cystoscope was inserted into the urethras. She had two bladders. Each contained one ureter.
• Vaginal opening was examined, which showed two cervices, uterine didelphis, and one vagina.
• She had a septate hymen. The hymenal septum was resected.

Takeaways

• Hymenal septum is an incomplete perforation of the hymen.
• Vaginal septum can be either longitudinal or transverse.
• The incidence of associated urologic anomalies in patients with anorectal malformation is 50%.
• The incidence of duplication of the Mullerian structures which includes vaginal septum, two hemivaginas and two hemiuterii is 1% in patient with recto-perineal fistula, 5% in patients with recto-vestibular fistula and 60% in patients with cloaca.
• No further surgical treatment is necessary for bladder and urethral duplication.
• Patients with good prognosis for bowel control are disimpacted using enemas then transitioned to laxatives.