Article
Persistent Müllerian duct syndrome – benefits and risks of treatment
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Published: | February 25, 2016 |
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Persistent Müllerian duct syndrome (PMDS) is a rare disorder of sexual differentiation characterized by non-regression of Müllerian duct structures (uterus, cervix, fallopian tubes, proximal vagina) in a 46 XY male with normal appearing external genitalia. In childhood, patients commonly present cryptorchidism or inguinal hernia. Laparoscopy is regarded as gold standard for diagnosis, but surgical management is still controversial. A complete removal of the Müllerian structures with simultaneous orchidopexy should be intended as preserving fertility and preventing malignant transformation of remnants and testes is the central goal of treatment. Our case report demonstrates different ways of treatment as well as their benefits and potential risks. Still guidelines are needed to provide a reliable postoperative management and an adequate long-term follow-up.