Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium

The aim of this study was to evaluate the accuracy of preoperative magnetic resonance imaging (MRI) in the diagnosis of malformations associated with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome and identification of uterine endometrium to optimise the clinical management. We retrospectively reviewed 214 consecutive MRKH patients, mean age 19 years, who underwent laparoscopy-assisted neovagina creation. A total of 115 patients (53.7 %) met the inclusion criterion of sufficient preoperative MRI. In 110 of them (95.7 %), MRI findings were correlated with laparoscopy and associated malformations. In 39 cases (35.5 %) uterine rudiments were removed and analysed histopathologically. Ten per cent (11/110) of the patients showed complete uterine agenesis. The others presented with either unilateral (n = 16; 14.5 %) or bilateral (n = 83; 75.5 %) uterine rudiments. MRI detection of uterine rudiments agreed in 78.2 % (86/110) with laparoscopy. In 85.4 % of the removed rudiments, MRI could correctly diagnose the existence of the endometrium. Compared to laparoscopy, MRI could exactly detect ovaries in 97.3 % (107/110). Renal or ureteral malformations were seen in 32 cases (27.8 %). In 83 % of unilateral renal agenesis and unilateral rudiment, the latter was located at the side of the kidney. MRI is useful for preoperative detection of MRKH-associated malformations and assessment of the endometrium to further optimise MRKH patient treatment. aEuro cent Pelvic MRI is useful for preoperative detection of MRKH-associated malformations. aEuro cent MRI can diagnose uterine endometrium in MRKH patients with high precision. aEuro cent Preoperative MRI can optimise clinical management of patients with MRKH syndrome.