Abstract

Video Objective We present a laparoscopic surgical approach for hysterectomy in Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with cervicovaginal agenesis. Laparoscopic approaches for removal of uterine remnants have been published with only reports of laparotomy described for hysterectomy with cervicovaginal agenesis. Setting We discuss the diagnosis, management considerations and preoperative planning for definitive surgical treatment of a patient with MRKH with cervicovaginal agenesis presenting with hematometra and pain. Patient initially presenting with primary amenorrhea and pain, with previous medical treatment to achieve amenorrhea. Interventions Laparoscopic hysterectomy with bilateral ureterolysis and uterine artery ligation in a patient with cervicovaginal agenesis. Conclusion We show that laparoscopic hysterectomy is a viable and safe option in patients with cervicovaginal agenesis. The importance of preoperative imaging to rule out associated anomalies and assist in surgical planning is stressed. We highlight necessary alterations in surgical technique to overcome the lack of traction, identify potential anomalous vasculature and ultimately maintain hemostasis, clear delineation of surgical anatomy, and avoid injury. Tissue extraction through laparoscopic in bag morcellation is performed thus avoiding mini laparotomy altogether.

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