To analyze the ultrasonographic imaging features of MRKH syndrome and explore the clinical value of ultrasonography in the diagnosis and differential diagnosis of MRKH syndrome.

A 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.

All 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.

Most 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.