Anorectal Malformation, Rectoperineal Fistula with Vaginal Agenesis

From the APSA 2016 Annual Meeting proceedings

OPERATIVE VIDEO: ANORECTAL MALFORMATION. RECTOPERINEAL FISTULA WITH VAGINAL AGENESIS
Victoria A. Lane, MBChB, Richard J. Wood, MD, Carlos Reck, MD, Geri Hewitt, MD,
Marc A. Levitt, MD.
Nationwide Children’s Hospital, Columbus, OH, USA.

Purpose:
We present the operative video of a female infant with a rectoperineal fistula with associated vaginal agenesis, who underwent reconstruction of the anorectal malformation and vaginal replacement.

Methods:
The case of a 6 month old female with a rectoperineal fistula and associated vaginal agenesis is presented. VACTERL screening identified an ASD and a dysplastic thumb. No spinal or renal anomalies were found and her sacrum was normal (Sacral ratio 1.0). At 7 months she underwent operative repair of the rectoperineal fistula and sigmoid colon vaginal replacement. The video demonstrates the initial examination findings of a vestibular fistula, with a normal vaginal introitus, however on closer inspection the vagina was found to be atretic. Standard mobilization of the rectum was performed in the prone position, followed by a lower midline laparotomy in order to examine the internal gynecological structures. A uterus and cervix were identified, but there was agenesis of the distal vagina. The operative technique for rectal pullthrough and simultaneous vaginal replacement, completion of the neo-vaginoplasty, and anoplasty is shown in the operative video.

Results:
One month after surgery the patient underwent an examination under anesthesia and vaginoscopy. The vaginal replacement was found to be healthy and a cervical dimple was seen. The anoplasty had healed well.

Conclusions:
Vaginal atresia is thought to occur in 5-10% of female patients with a rectoperineal/vestibular fistula. These patients require careful inspection of the perineum as the anomaly can be easily missed. The optimal timing of vaginal replacement has not been clearly established, but when rectal mobilization is required, there is a potential technical advantage to simultaneously completing the vaginal pullthrough.