The Investigation Of Endometriosis And Adenomyosis In Patients With Mayer-Rokitansky-Kuster-Hauser Syndrome

Objective:To investigate the endometriosis and adenomyosis happened in MRKH syndrome patients and to explore the mechanism of endometriosis and adenomyosis.Methods:Review all the patients who came to Women’s Hospital, Zhejiang University School of Medicine during January1,1999to December31,2013and were diagnosed with MRKH syndrome. Compare the incidence rates of endometriosis and adenomyosis in MRKH syndrome between normal endometrium group and no normal endometrium.Results:In219cases of MRKH syndrome,48cases had normal endometrium, including7cases with adenomyosis and6cases with endometriosis, while171cases had no normal endometrium, including2cases with adenomyosis and1case with endometriosis. There was significant diference in the incidence of endometriosis between the normal endometrium group and the no normal endometrium group (p<0.05). Significant diference was also found in the incidence of adenomyosis between the normal endometrium group and the no normal endometrium group (p<0.05).Conclusion:The morbidity rate of endometriosis and adenomyosis in MRKH syndrome with normal endometrium was significantly higher than in MRKH syndrome without normal endometrium, which illustrated the important role of normal endometrium in the development of endometriosis and adenomyosis. However, endometriosis and adenomyosis may still occur in MRKH syndrome without normal endometrium. It was speculated that the onset of endometriosis and adenomyosis can be explained by the coelomic metaplasia theory. PART2Adenomyosis in Mayer-Rokitansky-Kuster-Hauser Syndrome without Normal Endometrium:Two Case Reports AbstractAdenomyosis is identified as a result of abnormal in-growth and invagination of the basal endometrium into the myometrium. It seems impossible to occur in patient without normal endometrium. Here we present two unique cases of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with no normal endometrium to suffer from adenomyosis. Both patients complained of amenorrhea and lower abdominal pain, pelvic examination revealed the normal genitalia, blind vagina and pelvic mass, and ultrasound examination demonstrated the heterogeneous hypoechoic mass without "endometrial line" in the pelvic cavity. Laparoscopy showed enlarged rudimentary uteri and normal ovaries. Their high levels of serum CA125returned to normal following the excision of the rudimentary uteri. Histological examination showed adenomyosis in the rudimentary uteri with no endometrium. We conclude that the adenomyosis possibly occurred in patients with MRKH syndrome, may develop from the mullerian remnants.