Where Is the Vagina? A Rectal Stricture After a Presumed Cloacal Repair Turns out to Be a Mobilized Vagina and a Missed High Rectovaginal Fistula

Introduction: Anorectal malformations (ARMs) can have a wide spectrum of anatomic complexity. Defining the anatomy prior to surgery is vital to ensure a repair that maximizes the patient’s potential for urinary and fecal continence as well as gynecologic function.

Case Description: A 10-month-old female was referred to our center for an anal stricture. She had a previous diagnosis of short channel cloaca and underwent a posterior sagittal anorectoplasty (PSARP) at another institution, with a report of rectal mobilization only without any urogenital surgery. On exam, we found the urethra to be hypospadic, at a 0.5 cm depth from the clitoris. Cystoscopy showed a normal urethra and bladder but we could not identify a vagina. The “anoplasty” was in good position but was severely strictured for 1.5 cm. A contrast study through the mucous fistula was obtained (Figure 1) which showed that the vagina had been mistaken for the rectum and subsequently pulled through as the “anoplasty”. The rectum ended in a high rectovaginal fistula, which had not been touched. 3D imaging shows these findings, as well as an accessory urethra anterior to the bladder (Figure 2). In our reconstruction, we laparoscopically mobilized the high rectum and dissected the rectovaginal fistula but left it attached to the posterior vagina. The patient was then positioned prone, and we mobilized the native vagina off the “anoplasty” via a posterior sagittal incision, and placed it at the introitus, just behind the urethral orifice. The rectum was detached from the back of the vagina to create the anoplasty, and the perineal body was reconstructed. The urethral meatus was visible, facilitated easy catheterization, and did not require any mobilization. The accessory urethra was left untouched as it did not drain any urine.

Discussion: Preoperative planning with a multidisciplinary approach, including endoscopy and multimodal imaging are vital to achieve the successful, definitive anatomic repair of ARMs. At our institution, endoscopy with 3D imaging is performed for complex malformations to understand the bladder, ureteric, urethral, vaginal, and common channel anatomies as well as to visualize the location of the rectal fistula and the height of the rectum. Anomalies of the genital tract such as a vaginal septum, bicornuate uterus, or uterine didelphys may be diagnosed. 3D reconstructed fluoroscopy (as well as 3D cloacagram in cloacal malformations) is performed to obtain lengths of the common channel, urethra, calculate a sacral ratio, and assess spatial relations of the rectal fistula and other structures relative to the pelvis.

Conclusion: This case demonstrates a rare complication which occurred during what was thought to be a cloacal reconstruction in which the vagina was pulled through as the anoplasty. Multimodal imaging allowed us to delineate the anatomy and plan a successful reconstructive strategy.