gms | German Medical Science

62. Kongress der Nordrhein-Westfälischen Gesellschaft für Urologie

14. - 15.04.2016, Münster

Persistent Müllerian duct syndrome – benefits and risks of treatment

Meeting Abstract

  • presenting/speaker P. Paffenholz - Krankenhaus Düren, Klinik für Urologie und Kinderurologie, Urologische Onkologie, Düren, Germany
  • F. vom Dorp - HELIOS Marien Klinik, Urologische Klinik, Duisburg, Germany
  • H. Rübben - Universitätsklinikum Essen, Klinik und Poliklinik für Urologie, Essen, Germany
  • C. Börgermann - Krankenhaus Düren, Klinik für Urologie und Kinderurologie, Urologische Onkologie, Düren, Germany

Nordrhein-Westfälische Gesellschaft für Urologie. 62. Kongress der Nordrhein-Westfälischen Gesellschaft für Urologie. Münster, 14.-15.04.2016. Düsseldorf: German Medical Science GMS Publishing House; 2016. DocP2.2

doi: 10.3205/16nrwgu69, urn:nbn:de:0183-16nrwgu692

Published: February 25, 2016

© 2016 Paffenholz et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.


Outline

Text

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.