O1052: The timing of gynecologic management during cloaca repair with co-existing Müllerian agenesis: a paradigm shift

Background: Among patients with cloaca, partial Müllerian agenesis/vaginal atresia without connection to the common channel (CC) is rare. Historically, these cases were managed with a vaginal pull-through with transposition graft or a blind-ending bowel neovagina during the primary repair. However, grafts and bowel neovaginas have been associated with complications such as stenosis, dehiscence, prolapse, diversion colitis, cancer, and bothersome mucous production. As there is no continuity between the urologic and Müllerian systems, these patients are spared of the risks associated with uro-gynecologic fistulae, including hydrocolpos, hydronephrosis, and recurrent UTIs. We believe these patients do not require gynecologic surgery during the primary repair, and thus our reconstructive team has shifted away from early graft to delaying consideration of gynecologic reconstruction after puberty. We report our experiences managing these patients prior to and subsequent to adopting this new surgical approach.

Methods: A review of a single-institution, prospective database of patients undergoing cloacal repair was conducted (2019–2025). We identified cases of partial Müllerian agenesis/vaginal atresia with no common channel (CC) connection and assessed the intraoperative management of these patients.

Results: Of 101 patients with cloaca, 6 (6%) had partial Müllerian agenesis/vaginal atresia with no CC connection. All underwent primary repair at 3-36 months of age. CC length ranged from 1-7 cm, and urethral length from 0.5-2.5 cm. Four patients had rectums that extended below the pubococcygeal line. In all cases, the rectal fistula was dissected off the CC, converting the CC to a neourethra, and a perineal body sparing posterior sagittal anorectoplasty (PSARP) was performed. Prior to adopting our new surgical approach, one patient underwent an exploratory laparotomy, resection of bilateral Müllerian structures, and colonic neovagina creation. The subsequent five patients all underwent a diagnostic laparoscopy for assessment of Müllerian anatomy. Four of these patients had bilateral ovaries and atretic Müllerian structures, one of whom was felt to have vaginal tissue. One patient had unilateral agenesis of all gynecologic structures, including one ovary. In these 5 patients, atretic Müllerian structures were not resected and neither vaginal replacement nor vaginal pull-through was performed. Consideration of the gynecologic repair was deferred until after puberty.

Conclusion: Among a large cohort of patients with cloaca, 6% had partial Müllerian agenesis/vaginal atresia with no connection to the CC. This phenotype appears to be independent of CC and urethral length. Because prepubertal evaluation of the functional potential of Müllerian remnants is often incorrect, we recommend delaying consideration of gynecologic surgery until menarche, thereby potentially avoiding the need for neovaginal creation and its associated complications. This approach also spares the pelvis and perineal body from dissection in anticipation of future introitoplasty. These patients should undergo imaging during puberty to reassess their gynecologic structures and formulate a surgical plan.