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

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.