Endometriosis in a patient with Mayer-Rokitansky-Küster-Hauser syndrome and complete uterine agenesis: evidence to support the theory of coelomic metaplasia.

Abstract Background There are many hypotheses regarding the pathogenesis of endometriosis. Most theories, including retrograde menstruation, require the existence of a uterine structure and endometrial tissue. We report endometriosis with the absence of a uterus. This finding supports the theory of coelomic metaplasia. Case A 20-year-old with Mayer-Rokitansky-Kuster-Hauser syndrome presented with increasing pelvic pain and underwent laparoscopy. Uterine, cervical, vaginal, and tubal agenesis was confirmed. Stage I endometriosis was visualized in the posterior cul-de-sac and destroyed. She received medical therapy for 5 years until she represented with pain and underwent another laparoscopy, at which endometriosis was again identified and destroyed. Summary and Conclusion This case of endometriosis in a patient with complete uterine agenesis supports the theory of coelomic metaplasia.