Uterine Remnants and Pelvic Pain in Females with Mayer-Rokitansky-Küster-Hauser Syndrome

Objective: To assess the association between pelvic pain and uterine remnants and review the management of pelvic pain in females with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Design: Retrospective cohort. Setting: Department of Obstetrics and Gynecology at a tertiary referring medical center. Patients: Forty-eight females with MRKH presenting from 1997 to 2011 with anatomy confirmed by magnetic resonance imaging (MRI). Interventions: None. Main Outcome Measure: Prevalence Of uterine remnants and the association of uterine remnants with pelvic pain in females with MRKH. Results: Of the 48 females with MRKH, 23 (48%) had uterine remnants and 22 (46%) had pelvic pain. Presence of endometrium was associated with pelvic pain (RR = 2.3; 95% CI = 1.2-4.7) in females with MRKH. Of the females with MKRH and pain, 9/22 had laparoscopy, with endometriosis seen in 5/9 of the uterine remnants at stages higher than are usually seen in teenagers (56%). Nine patients with pain and uterine remnants (8 with endometrium, 1 without) had laparoscopic removal of uterine remnants with resolution of pain. Conclusions: Given the high prevalence of uterine remnants in females with MRKH, anatomic evaluation with MRI should be considered when assessing the etiology of pelvic pain. Presence of endometrium within uterine remnants, and subsequent endometriosis, in females with MRKH may be associated with pelvic pain necessitating surgical or medical management.