Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome

We evaluated the accuracy of magnetic resonance imaging (MRI) in young women with primary amenorrhoea with suspected Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome (congenital absence of both vagina and uterus and presence of normal ovaries). Fifty-eight women (age range 14-30 years, mean 20.9) with primary amenorrhea were studied with MRI performed with a 1.0-T superconducting magnet (Philips NT Intera). All patients were examined in the supine position using a phased-array coil (four channels). Turbo spin-echo T2-weighted images were acquired in the sagittal, axial and coronal planes with the following parameters: TR 4,750-6,686, TE 100-120, FOV 350-375, 4- to 5-mm sections with a 0.4- to 0.5-mm intersection gap and NSA 6. T1-weighted images were acquired in the axial and coronal planes (TR 470, TE 15, FOV 350, 4-mm sections with a 0.6-mm intersection gap, NSA 3). Two experienced radiologists evaluated all the examinations in consensus to assess the presence, position and morphology of vagina, uterus, ovaries and kidneys and any pelvic abnormalities. MRI results were judged on the basis of laparoscopic findings in 41 patients. MRKH syndrome was confirmed in 56 patients with 100% sensitivity and specificity. MRI identified bilateral Mullerian buds in 34/56 (61%) and unilateral in 10/56 (18%) patients. MRI sensitivity was 81.42%, and there was good agreement with laparoscopy (k=0.55) and full agreement in the identification of cavitation between MRI and intraoperative sonography. Both ovaries were visualised in 54 patients, with regular morphology in 46 (82.1%), polycystic in 10 (17.8%), pelvic in 47 (83.6%) and extrapelvic in eight (14.5%). We found associated abnormalities of the upper urinary tract in six patients (solitary kidney in four and ptosis in two). MRI is a useful diagnostic tool in the preoperative evaluation of MRKH syndrome and is less expensive and invasive than laparoscopy. Strong cooperation between radiologists and surgeons is highly recommended.