INTRODUCTION: Caudal duplication syndrome describes a constellation of rare and complex congenital gastrointestinal, genitourinary, and distal neural tube malformations. While hysterectomy for women with müllerian anomalies has been described, minimally invasive hysterectomy in a woman with additional gastrointestinal and urinary anomalies has not yet been demonstrated. OBJECTIVE: To demonstrate a challenging minimally invasive total hysterectomy with bilateral salpingectomy in an adult female with caudal duplication syndrome, including complete urogenital and gastrointestinal duplication. METHODS: A 38-year-old G2P0202 woman with caudal duplication syndrome, including gastrointestinal, urogenital sinus, müllerian duplications (uterine didelphys, bicollis, longitudinal vaginal septum), and spina bifida, presented with a history of dysmenorrhea and heavy menstrual bleeding with irregular cycle exacerbating recurrent urinary tract infections. She was complete with child-bearing and strongly desired definitive management with hysterectomy, but was previously counseled that she was a poor surgical candidate due to her complex urinary anatomy. Surgical history is significant for bilateral anoplasty as a newborn, an exploratory laparotomy with resection of an ileal duplication to the left colon at 5 years old, cesarean section x 2, and a left simple cystectomy with right augmentation ileocystoplasty and a left ureteral reimplantation into the right bladder. Medical management with continuous combined contraceptive pills, levonorgestrel intrauterine device in each uterine horn, Depo-Lupron, and Depo-Provera was attempted over a 10-year period with brief episodes of amenorrhea and ultimately return of abnormal uterine bleeding. A multidisciplinary surgical team, consisting of specialists in complex benign gynecology, urology, and colorectal surgery, reviewed magnetic resonance imaging and past operative reports for preoperative planning. Cystoscopy was performed for prophylactic ureteral stent placement with indocyanine green and Botox (AbbVie, Chicago, IL) injection for bladder spasm. Robot-assisted laparoscopy was performed under general anesthesia using the Da Vinci robot platform (Intuitive, Sunnyvale, CA). The patient's abdomen was entered at Palmer's point laparoscopically, and pneumoperitoneum was obtained. Significant bowel adhesions to the anterior abdominal wall, which prevented access to the pelvis, required extensive lysis of the adhesions. The Da Vinci robot was docked, and pelvic lysis of adhesions was completed. Firefly fluorescence was activated to aid in the identification of the ureter. Total removal of the didelphic uterus with bilateral salpingectomy was then performed with a uterine manipulator with a colpotomy cup in the right horn and an EEA sizer in the left vagina. The specimen was removed through the right vagina, and the colpotomies were closed in two parts with barbed suture. RESULTS: Total hysterectomy with bilateral salpingectomy was successfully completed with robot-assisted laparoscopy. There were no complications during or after the surgery. CONCLUSIONS: Caudal duplication syndrome is a rare constellation of congenital anomalies that may require multiple surgical interventions starting in early life. This poses potential further unique anatomical challenges. This unique case demonstrates that a minimally invasive approach is feasible and safe to attempt to accomplish definitive surgical management of abnormal uterine bleeding with hysterectomy. However, a multidisciplinary team and thoughtful preoperative planning are critical for success.
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