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

Mayer-Rokitansky-Küster-Hauser综合征的磁共振成像和临床特征:一家专科中心10年回顾性研究

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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.

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