Mayer-Rokitansky-Kuster-Hauser综合征患者的超声特征

Ultrasonic features of patients with Mayer-Rokitansky-Kuster-Hauser syndrome

目的探讨Mayer-Rokitansky-Kuster-Hauser(MRKH)综合征患者腹部超声检查的影像学特点。方法选择2000年至2018年,因“进入青春期后,无月经初潮”于四川大学华西第二医院就诊,而被诊断为MRKH综合征的17例患者为研究对象。回顾性分析其腹部超声的影像学特征。本研究遵循的程序,符合2013年修订的《世界医学协会赫尔辛基宣言》。结果这17例患者的年龄为(18±5)岁,染色体核型分析及血清性激素水平检查结果均正常。其中,15例被诊断为MRKH综合征Ⅰ型(88.2%),其余2例被诊断为MRKH综合征Ⅱ型(11.8%)。其超声检查结果均未查见阴道气体线回声。其中,1例(5.9%)无子宫,6例(35.3%)正常发育子宫,3例(17.6%)正常宫体、无宫颈,2例(11.8%)单侧始基子宫、无宫颈,4例(23.5%)双侧始基子宫,1例(5.9%)双子宫无宫颈。10例(58.8%)子宫内膜正常,3例(17.6%)附件区见囊性占位,2例(11.8%)患者合并重复肾畸形。结论无阴道气体线声像图,是MRKH综合征患者的主要超声表现,超声检查时,还应同时注意患者有无合并其他畸形。腹部超声检查,可为该病患者的准确诊断及分型提供影像学依据。

Objective To explore features of abdominal ultrasonic images of patients with Mayer-Rokitansky-Kuster-Hauser(MRKH)syndrome.Methods From 2000 to 2018,a total of 17 patients with MRKH syndrome diagnosed in West China Second University Hospital,Sichuan University due to"no menarche after entering puberty",were selected as research subjects.Their abdominal ultrasound diagnosis results were analyzed retrospectively.The procedure followed in this study was in line with the World Medical Association Declaration of Helsinki revised in 2013.Results The average age of 17 patients was(18±5)years old,and results of karyotype analysis and serum sex hormone levels examination were normal.Among them,15 cases were diagnosed as MRKH syndrome typeⅠ(88.2%),and the remaining 2 cases were diagnosed as MRKH syndrome typeⅡ(11.8%).Ultrasound examination results showed that no vaginal gas line echo were found in all patients.Among them,1 case(5.9%)had no uterus,6 cases(35.3%)had a normal developing uterus,3 cases(17.6%)had a normal uterus without cervix,2 cases(11.8%)had unilateral primordial uterus without cervix,4 cases(23.5%)had bilateral primordial uterus,1 case(5.9%)had double uterus without cervix.There were 10 cases(58.8%)had normal endometrium,3 cases(17.6%)had cystic space in adnexa area,2 cases(11.8%)had malformation of renal duplication.Conclusions No vaginal gas line sonogram is the main ultrasound manifestation of patients with MRKH syndrome,and when having ultrasound examination,attention should be paid to whether they have other malformations or not.Abdominal ultrasonography could provide a imaging basis for accurate diagnosis evidences and classification of patients with MRKH syndrome.