Mayer-Rokitanksy-Kuster-Hauser Syndrome: Surgical Removal of Uterine Remnant

Objectives: Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH) is a rare congenital disorder affecting the female reproductive system. It occurs in about 1 in 4,000-5,000 46 XX females. It is characterized by agenesis of the vagina and varying degrees of cervical and/or uterine agenesis. Occasionally, these patients can present with cyclic abdominal pain as a result of hematometra from trapped menses. The objective of this video is to introduce a unique case demonstrating a robotic-assisted laparoscopic approach for removal of a uterine remnant with hematometra in a patient with MRKH and abdominal pain.

Methods: We review the case of a 23-year-old female with MRKH syndrome who presented with a chief complaint of cyclic pelvic pain and primary amenorrhea. MRI imaging demonstrated a left hematosalpinx and left uterine horn with diffuse hematometra distending the left uterine cavity, cervical atresia, and a non-communicating right rudimentary horn. Patient failed initial conservative management with ovulary suppression medication and wished to proceed with definitive surgical management to remove the uterine remnant. In this video, we demonstrate a systematic approach and safe techniques to perform robot assisted laparoscopic removal of a uterine remnant in a patient with this rare anatomy.

Clinical Relevance: MRKH can present in a variety of forms due to varying degrees of development of the Müllerian duct at the embryologic stage. As such, it is associated with variable pelvic anatomy and uterine abnormalities depending on the degree of original Müllerian duct development. Given the differences in presentation, it is essential to learn more about the approach to workup and management in order for physicians to best serve these patients. Our video presents a case of failed medical management requiring surgical intervention for symptom relief. We demonstrate appropriate surgical planning with MRI as well as a systematic operative approach to carefully dissect target organs, identify and control vascular supply, recognize important surrounding structures, and tips to identify uterine-vaginal junction in patients who lack a cervix. With more guidance on how to manage these cases, providers will ultimately be able to provide the optimal care for patients suffering from MRKH.