Magnetic resonance imaging and clinical features of Mayer-Rokitansky-Küster-Hauser syndrome: A 10-year review from a dedicated specialist centre.

Abstract

Objective: To correlate the clinical history with imaging findings of women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

Design: Retrospective cohort study.

Setting: A UK IOTA and ESGO-certified tertiary referral centre for disorders of reproductive development.

Population: All patients with a diagnosis of MRKH and who had undergone an MRI pelvis between 1 January 2011 and 31 April 2021 were included.

Methods: MRI images were analysed by specialist gynaecological radiologists. Clinical data was extracted from an electronic patient record system. Statistical analysis was computed in R (version 4.1.2), R base stats package and ggstatsplot (v0.5.0).

Main outcome measures: Clinical history and predefined imaging features.

Results: One hundred and thirty-four patients were included. Median age at MRI was 18 years (10-64 years). Half (48.2%) of women presenting had a history of pain, most often abdominal (84.6%) or vaginal (9.2%). Remnants were identified in 91.8% of women (n = 123). 4.5% of women had imaging features of endometriosis (n = 6). Women with a functional remnants were significantly more likely to experience pain (p < 0.001). Pain history was not strongly associated with ectopic ovarian position. Common gynaecological pathology such as endometriosis, ovarian cysts and fibroids were also identified.

Conclusions: We identify that majority of women with MRKH will have uterine remnants with a connecting fibrous band, and an ectopic ovarian position 44.0% of cases. Abdominal pain was significantly associated with functional remnants on MRI. Further work is required to identify how other gynaecological pathology impacts women with MRKH.