Preoperative Considerations for Uterine Fibroid Removal in Patients With Mayer-Rokitansky-Küster-Hauser Syndrome and Klippel-Feil Syndrome: A Case Report

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a congenital disorder characterized by dysplasia of the uterus and upper vagina due to Müllerian duct dysplasia. Fibroids arise from the rudimentary uterus. The implementation of surgical intervention for uterine fibroids in patients with MRKHS presents several challenges and considerations. The rudimentary uterus is tiny, making it difficult to determine the location of the pelvic mass and diagnose uterine fibroids. Preoperative planning is often inadequate, necessitating intraoperative decision-making. In addition, patients with MRKHS may present with Klippel-Feil syndrome (KFS), characterized by cervical spine fusion, further complicating intraoperative management. In contrast to the typical practice, careful surgical planning along with anesthesia administration and orthopedic surgery is required. In this case, magnetic resonance angiography was utilized to determine the location of the mass and the rudimentary uterus. Screening was conducted to detect any concomitant malformation, such as KFS. Subsequently, a safe laparoscopic surgery was carried out. In this case report, we present the importance and unique considerations of preoperative planning for the surgical intervention of uterine fibroids in patients with MRKHS.