Pompili G, Munari A, Franceschelli G, Flor N, Meroni R, Frontino G, Fedele L, Cornalba G
Servizio di Radiologia Diagnostica e Interventistica, Università di Milano, Ospedale San Paolo, Milano, Italy.
Radiol Med. 2009 Aug;114(5):811-26. doi: 10.1007/s11547-009-0407-5. Epub 2009 May 29.
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 Müllerian 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.
我们评估了磁共振成像（MRI）对疑似迈耶-罗基坦斯基-库斯特-豪泽综合征（MRKH综合征，即先天性阴道和子宫缺失但卵巢正常）的年轻原发性闭经女性的诊断准确性。
对58名年龄在14至30岁（平均20.9岁）的原发性闭经女性进行了研究，使用1.0-T超导磁体（飞利浦NT Intera）进行MRI检查。所有患者均仰卧位，使用相控阵线圈（四通道）进行检查。在矢状面、轴面和冠状面获取快速自旋回波T2加权图像，参数如下：TR 4750 - 6686，TE 100 - 120，视野350 - 375，层厚4至5毫米，层间距0.4至0.5毫米，激励次数6。在轴面和冠状面获取T1加权图像（TR 470，TE 15，视野350，层厚4毫米，层间距0.6毫米，激励次数3）。两名经验丰富的放射科医生共同评估所有检查，以评估阴道、子宫、卵巢和肾脏的存在、位置和形态以及任何盆腔异常情况。根据41例患者的腹腔镜检查结果判断MRI结果。
56例患者确诊为MRKH综合征，敏感性和特异性均为100%。MRI在34/56（61%）的患者中发现双侧苗勒管芽，在10/56（18%）的患者中发现单侧苗勒管芽。MRI的敏感性为81.42%，与腹腔镜检查有良好的一致性（k = 0.55），在MRI与术中超声检查对空洞的识别方面完全一致。54例患者双侧卵巢均可见，其中46例（82.1%）形态正常，10例（17.8%）为多囊性，47例（83.6%）位于盆腔内，8例（14.5%）位于盆腔外。我们发现6例患者存在上尿路相关异常（4例为孤立肾，2例为肾下垂）。
MRI是MRKH综合征术前评估的有用诊断工具，比腹腔镜检查成本更低且侵入性更小。强烈建议放射科医生和外科医生密切合作。
