MRKH综合征的超声影像学特征及其诊断价值

目的分析MRKH综合征的超声影像学特征,探讨超声在诊断及鉴别诊断MRKH综合征中的临床价值。 方法回顾性分析2021年1月至12月深圳市罗湖区人民医院经手术证实的75例MRKH综合征患者的超声表现,并与手术结果进行对比分析,总结MRKH综合征典型及非典型的超声影像学特征。 结果本组75例MRKH综合征患者均经腹腔镜手术证实,其术前超声检查结果均与手术结果一致,诊断符合率为100%。62例（82.7%）MRKH综合征表现为典型的“四联征”超声特征：在盆腔左右两侧分别可见梭形或长条形肌性回声的始基子宫,未见宫颈和子宫内膜回声;可见连接两侧始基子宫下缘条带状的低回声索状带;双侧卵巢紧邻同侧始基子宫;膀胱尿道后壁与直肠前壁之间未见正常阴道结构,仅见低回声结缔组织。13例（17.3%）MRKH综合征表现为非典型的超声特征：4例始基子宫位置异常,位于腹股沟区;5例始基子宫内有功能性子宫内膜回声;2例索状带静脉扩张;4例卵巢位置异常。其他伴发异常：2例单侧始基子宫肌瘤,3例双侧始基子宫腺肌病,18例合并卵巢病变,4例合并肾脏发育异常。 结论大部分MRKH综合征具有典型的“四联征”超声特征,准确识别MRKH综合征典型及非典型的超声表现,可为临床诊治MRKH综合征提供可靠的影像学诊断依据。

Ultrasonographic imaging features and diagnostic value of MRKH syndrome

ObjectiveTo analyze the ultrasonographic imaging features of MRKH syndrome and explore the clinical value of ultrasonography in the diagnosis and differential diagnosis of MRKH syndrome. MethodsA total of 75 cases of MRKH syndrome were retrospectively analyzed. All the cases were diagnosed by ultrasound and confirmed by surgery at Shenzhen Luohu People's Hospital from January to December 2021. The typical and atypical ultrasonographic imaging features of MRKH syndrome were reviewed and compared with the surgical findings. ResultsAll the 75 patients with MRKH syndrome in this group were confirmed by laparoscopic surgery. The results of preoperative ultrasonography were consistent with the surgical results and the diagnostic coincidence rate was 100%. Sixty-two (82.7%) cases presented with typical "tetrad" ultrasonic signs of MRKH syndrome: fusiform or elongated muscular echoes of the rudimentary uterus (with no cervix or endometrium) on both sides of the pelvic cavity; hypoechoic fibrous band connecting the lower margin of the bilateral rudimentary uterus; ovaries adjacent to the rudimentary uterus; absence of ultrasonic features of the normal vagina between the urethra and bladder posteriorly and the rectum anteriorly. Thirteen (17.3%) cases presented with some atypical features of MRKH syndrome: ectopic rudimentary uterus in the groin region in 4 cases, rudimentary uterus with functional endometrium in 5, varicosis in the fibrous band in 2, and ectopic ovary in 4. Other complications included: leiomyoma in the unilateral uterus in 2 cases, bilateral uterine adenosis in 3, ovarian mass in 18, and renal dysplasia in 4. ConclusionMost patients with MRKH syndrome present with typical "tetrad" ultrasonic signs in ultrasound examination. Identifying the typical and atypical ultrasonic manifestations of MRKH syndrome exactly could provide a reliable imaging basis for clinical treatment.