Clinical Features and Management of Endometriosis among Patients with MRKH and Functional Uterine Remnants

Objective: This study aimed to determine the clinical features of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome patients with functional uterine remnants and endometriosis in a large cohort of Chinese patients. Design: This was a retrospective study. Participants: This study had a cohort of 511 MRKH patients. A total of 34 MRKH patients with functional remnant were finally included. Setting: This study included a tertiary referring hospital in China . Methods: Patients with MRKH diagnosed and treated at Peking Union Medical College Hospital from January 2009 to January 2020 were recruited. A cohort of 511 MRKH patients were retrospectively screened, and a total of 34 MRKH patients with functional remnant were finally included. Relevant clinical data were reviewed retrospectively from medical charts. Results: Of 34 patients with MRKH and functional uterine remnants, 23 (68%) had endometriosis. These patients had a greater mean age at MRKH diagnosis than patients without endometriosis (15.9 ± 3.3 years vs. 13.2 ± 3.5 years; p = 0.03). Similarly, these patients experienced a longer time between age at onset of symptoms and age at operation than patients without endometriosis (45.5 ± 39.6 years vs. 19.8 ± 13.2 years; p = 0.04). In addition, the CA125 level was significantly higher in patients with endometriosis than in those without it (64.9 ± 85.9 U/mL vs. 25.5 ± 19.1 U/mL; p = 0.03). Limitations: The number of patients with MRKH analyzed in this study was low as we restricted inclusion to patients with at least 1 functional uterine remnant or endometriosis. Conclusions: It is reasonable to monitor the uterine remnant of patients with MRKH closely, regardless of age, to achieve early intervention. The level of CA125 might be helpful to differentiate active uterine remnants with endometriosis and schedule individualized treatments.