A Mayer-Rokitansky-Kuster-Hauser patient with leiomyoma and dysplasia of neovagina: a case report

Abstract

Background Most patients with congenital uterus and vaginal aplasia (i.e., Mayer–Rokitansky–Kuster–Hauser [MRKH] syndrome) have rudimentary pelvic uterine structures that contain smooth muscle. Although leiomyomas and dysplasia of vaginal mucosa are relatively common in the general population, they are rare in MRKH patients. Data on the vulnerability of neovaginas to HPV-associated dysplasia are limited. Case presentation A rare case of an MRKH patient with two gynaecological conditions detected during long-term gynaecological follow-up is presented. At the age of 21, the patient was treated for HPV-associated neovaginal dysplasia. At the age of 47, a pelvic leiomyoma was detected with transvaginal ultrasound and confirmed with magnetic resonance imaging. Conclusion A Pap smear or human papillomavirus testing is indicated in sexually active MRKH women. Uterine rudiments contain smooth muscle, which facilitates the development of oestrogen-dependent diseases, such as leiomyomas and adenomyosis. Although magnetic resonance imaging is recommended in cases of a pelvic mass, easily attainable and cost-efficient transvaginal ultrasound offers high diagnostic accuracy in patients with a surgically created neovagina and is suitable for the patients’ follow-up. Guidelines for the gynaecological follow-up of MRKH patients are warranted.

Giant hysteromyoma after vaginoplasty in a woman with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: case report and review of the literature

“…Published reports regarding the course of patients with MRKH syndrome, whose rudimentary uteri were preserved during vaginoplasty, are summarised in Table 1. [17][18][19][20][21][22] In the present literature review, the Abbe-McIndoe operation was one of the most commonly used methods of vaginal reconstruction (performed in two cases). 17,18 Williams' vaginoplasty was performed in three cases, 19,20 Vecchietti's vaginoplasty in one case, 21 and Davidov's vaginoplasty in one case.…”

“…17,18 Williams' vaginoplasty was performed in three cases, 19,20 Vecchietti’s vaginoplasty in one case, 21 and Davidov’s vaginoplasty in one case. 22 Oestrogen levels were normal in almost all cases, 17,19–21 except in one peri-menopausal patient, and cyclic endometrial changes were found by ultrasound in some patients’ rudimentary uteri before they received vaginoplasty. 17,21 These patients preserved their uterus either because the vaginoplasty was performed via an external approach, or no abnormality was found during surgical exploration until hysteromyoma was detected by postoperative imaging examination or new symptoms appeared.…”

“…Published reports regarding the course of patients with MRKH syndrome, whose rudimentary uteri were preserved during vaginoplasty, are summarised in Table 1. 17–22…”

“…Around 2-7% of patients with MRKH syndrome have a functional endometrium in the rudimentary uteri, 5 and the presence of endometrium in rudimentary uteri may lead to pelvic pain. Of 48 females with MRKH syndrome, 48% (23) were found to have uterine remnants, and 46% (22) reported pelvic pain. 24 The presence of endometrium was also found to be associated with pelvic pain (risk ratio 2.3; 95% confidence interval 1.2, 4.7) in females with MRKH syndrome.…”

The Rare, Unexpected Condition of a Twisted Leiomyoma in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Etiopathogenesis, Diagnosis and Management. Our Experience and Narrative Review of the Literature

“…Apparently, there are only 27 cases described of uterine leiomyoma in MRKH not complicated by acute abdomen ( Table 1 ) [ 2 , 3 , 4 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 ]. The average age at the time of diagnosis was 41.8 years.…”