Approach to Hysterectomy in the Setting of Müllerian Anomalies

INTRODUCTION: The müllerian or paramesonephric ducts form the internal genitalia of the female reproductive system, including the uterus, cervix, fallopian tubes, and upper one-third of the vagina. Development of the ducts is regulated by the absence of antimüllerian hormone (AMH) as well as signaling molecules and gene expression. Failure of müllerian ducts to develop normally during embryogenesis results in various müllerian anomalies, including uterine didelphys (which accounts for 5% of all müllerian anomalies) and bicornuate uterus (which accounts for 10% of all müllerian anomalies). Patients can present with symptoms such as abnormal uterine bleeding, pelvic pain, and adverse obstetrical outcomes. OBJECTIVE: The objectives of this video are to showcase the multidisciplinary treatment of a complex müllerian anomaly as well as demonstrate a minimally invasive approach using robotic surgery for hysterectomy in the setting of müllerian anomalies and pelvic organ prolapse. METHODS: Our clinical scenario involves a 42-year-old who presented as a transfer from an outside hospital due to left lower quadrant pain and concern for pyelonephritis in the setting of congenital bladder and müllerian anomalies. Her past medical and surgical history was significant for insulin-dependent diabetes mellitus and a prior cystectomy with ileal conduit. Physical exam demonstrated a urostomy in the right lower quadrant and pelvic exam showed incomplete uterovaginal prolapse with 2 cervices prolapsing through the vagina to +2 as well as a longitudinal and transverse vaginal septum. A CT abdomen/pelvis and MRI pelvis were obtained for preoperative planning, which showed a large leiomyoma with concern for degenerative changes. The patient was admitted to the gynecologic oncology for evaluation and urogynecology and urology services were consulted. Patient desired definitive surgical management due to pain and she underwent a combined procedure with the gynecologic oncology and urogynecology services. RESULTS: The patient’s incomplete uterovaginal prolapse with the descent of 2 cervices in the setting of a longitudinal and transverse vaginal septum complicated the standard approach to hysterectomy using a uterine manipulator. A longitudinal vaginal septoplasty was performed prior to uterine manipulator placement in order to accommodate both cervices within the manipulator cup. Upon abdominal entry, significant adhesive disease was noted from the bowels to the abdominal and pelvic sidewalls requiring left-sided rather than pelvic docking of the DaVinci Xi robot. Once lysis of adhesions had been safely completed, hysterectomy was performed. A defect in the ileal conduit was repaired. For the patient's uterovaginal prolapse, a unilateral uterosacral ligament suspension was performed as well as a posterior colporrhaphy and a horizontal advancement flap in order to preserve vaginal caliber. CONCLUSIONS: In conclusion, knowledge of pelvic anatomy and adherence to foundational surgical principles are crucial in the management of complex surgical patients with müllerian anomalies.