Agenesis of female internal reproductive organs, the Mayer- Rokitansky-Küster-Hauser syndrome

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the most common cause of uterine and uterine tubes absence/underdevelopment and the second most common cause of primary amenorrhea. It is characterized by a congenital agenesis of the uterine tubes, uterus, cervix, and upper part of the vagina. This study presents our 10 years of experience with the diagnostics and therapeutic management of patients with MRKH syndrome. We also focused on the description of anatomical deviations of the female reproductive organs.

We studied a cohort of 16 patients hospitalized with MRKH syndrome between 2011-2020. We analysed the age at diagnosis, clinical signs and symptoms, diagnostic techniques, methods of neovagina creation, and anatomical anomalies of the female reproductive organs.

The mean age of our patients at the time of diagnosis was 16.75 years. The most frequent clinical manifestations were primary amenorrhea (56.25 %), pelvic pain (31.25 %) and coitus-associated problems. We used two methods of neovagina creation. In six patients we used the non-surgical Frank's dilators method. The vaginal length at the beginning of the dilation was 1.5-2 cm. In 4 patients the vaginal length was under 1 cm, so we performed surgical neovagina creation using the laparoscopic Vecchietti method. An-other two patients could create the vagina via regular sexual intercourse. Based on magnetic resonance imaging and ultrasound examination, the following anomalies were observed: complete agenesis of the uterus (50 % of cases), fibrous band (25 %) and rudimentary uterus (25 %).

Satisfactory results in the attempt to create a neovagina can be obtained by both non-surgical and surgical methods. Modern medicine provides many therapeutic measures, which make it possible for the affected women to have a normal sexual life (Tab. 1, Fig. 3, Ref. 37).