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LETTER TO THE EDITOR—BRIEF COMMUNICATION| Volume 159, ISSUE 1, P231-232, November 2011

MRKH syndrome with endometriosis: case report and literature review

  • Lei Yan
    Affiliations
    Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, 324 Jingwu Road, Jinan, Shandong 250021, People's Republic of China
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  • Xingbo Zhao
    Correspondence
    Corresponding author. Tel.: +86 531 85186384.
    Affiliations
    Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, 324 Jingwu Road, Jinan, Shandong 250021, People's Republic of China
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  • Xiaoyan Qin
    Affiliations
    Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, 324 Jingwu Road, Jinan, Shandong 250021, People's Republic of China
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      We would like to report a case of endometriosis in a patient with complete uterine agenesis. A 23-year-old girl was admitted to hospital with primary amenorrhea post puberty. On pelvic examination, the uterus could not be touched clearly. Trans-abdominal ultrasound showed a 2.3 cm × 1.9 cm × 1.0 cm hypoechoic nodules (single uterine bud) with no functional endometrium line resonance, no vaginal gas line resonance was found and the ovaries were in normal size. She was diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome by clinical symptoms, followed up with endorectal exploration and abdominal ultrasound. Pelvic MRI was not routinely used in China for the diagnosis of MRKH at that time. During trans-abdominal sigmoid transplantation for vaginal reconstruction, a purple and brown nodule measured 0.5 cm on the right ovary surface was found which was proved to be an ectopic endometriotic lesion (old bleeding site possibly from the previous postoperative pathohistologic analysis, See Fig. 1). As there was no functioning endometrium in the uterine buds, the endometriotic site might come from metaplasia of the existing epithelium in the ovary which supports the theory of coelomic metaplasia.
      Figure thumbnail gr1
      Fig. 1Pathohistologic analysis of the endometriotic lesion on the right ovary (old bleeding site, H&E, ×200).
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      References

        • Meyer R.
        The current question of adenomyositis and adenomyomas in general and particularly seroepithelial adenomyositis and sarcomatoid adenomyometritis.
        Zentralbl Gynakol. 1919; 43: 745-750
        • Zheng W.
        • Li N.
        • Liang S.X.
        • et al.
        Initial endometriosis showing direct morphologic evidence of metaplasia in the pathogenesis of ovarian endometriosis.
        Int J Gynecol Pathol. 2005; 24: 164-172
        • Gaetje R.
        • Holtrich U.
        • Kaufmann M.
        • et al.
        Endometriosis may be generated by mimicking the ontogenetic development of the female genital tract.
        Fertil Steril. 2007; 87: 651-656
        • Cho M.K.
        • Kim C.H.
        • Oh S.T.
        Endometriosis in a patient with Rokitansky-Kuster-Hauser syndrome.
        J Obstet Gynaecol Res. 2009; 35: 994-996
        • Enatsu A.
        • Harada T.
        • Yoshida S.
        • Iwabe T.
        • Terakawa N.
        Adenomyosis in a patient with the Rokitansky-Kuster-Hauser syndrome.
        Fertil Steril. 2000; 73: 862-863