title: [Persistent Müllerian ducts syndrome: one case of late hypofertility].
abstract: We report the case of a 35-year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type (group A). The diagnosis was made in adulthood during an infertility workup. Clinical examination revealed an empty scrotum, a normal penis and bilateral inguinal cystic masses. The spermogram found azoospermia. Imaging using MRI and tomotensidometry found the presence of an uterus, two fallopian tubes and two inguinal positions of polycystic testes. A surgical management was performed for surgical testicular biopsy. Histological examination then found a cystic formation of multi-celled mesothelial origin, with atrophic testis Sertoli cell involution and without sperm. PMDS is a rare form of pseudo-internal hermaphroditism characterized by the presence in a man of the uterus, fallopian tubes and upper vagina with external male genitalia and virilized characters. About 200 cases are reported in the literature. The diagnosis is often made in children intraoperatively during a cure of testicular ectopia. The karyotype is 46 XY type. The pathogenesis is related to a deficiency of anti-Müllerian hormone (AMH) or tissue resistance to its action by receptor abnormalities. The regression of the Müllerian duct derivatives can give three types of PMDS : masculine type, feminine type and a transverse type. Surgical treatment is difficult but necessary because of the risk of infertility and ectopic testicular degeneration.
authors.affiliation: Laboratoire d'anatomie et d'organogenèse, UFR de médecine d'Amiens, 3, rue des Louvels, 80036 Amiens cedex 1, France; Service d'urologie-transplantation, CHU Amiens-Picardie, avenue Laënnec, 80480 Salouël, France. Electronic address: viartludovic@gmail.com.
authors.forename: L
authors.initials: L
authors.lastname: Viart
authors.affiliation: Laboratoire d'anatomie et d'organogenèse, UFR de médecine d'Amiens, 3, rue des Louvels, 80036 Amiens cedex 1, France.
authors.forename: J
authors.initials: J
authors.lastname: Peltier
authors.affiliation: Service d'urologie-transplantation, CHU Amiens-Picardie, avenue Laënnec, 80480 Salouël, France.
authors.forename: T
authors.initials: T
authors.lastname: Forzini
authors.affiliation: Laboratoire d'anatomie et d'organogenèse, UFR de médecine d'Amiens, 3, rue des Louvels, 80036 Amiens cedex 1, France.
authors.forename: C
authors.initials: C
authors.lastname: Page
authors.affiliation: Laboratoire d'anatomie et d'organogenèse, UFR de médecine d'Amiens, 3, rue des Louvels, 80036 Amiens cedex 1, France.
authors.forename: P
authors.initials: P
authors.lastname: Foulon
authors.affiliation: Service d'urologie-transplantation, CHU Amiens-Picardie, avenue Laënnec, 80480 Salouël, France.
authors.forename: F
authors.initials: F
authors.lastname: Saint
authors.affiliation: Laboratoire d'anatomie et d'organogenèse, UFR de médecine d'Amiens, 3, rue des Louvels, 80036 Amiens cedex 1, France.
authors.forename: E
authors.initials: E
authors.lastname: Havet
mesh_terms: D000328:Adult; D000367:Age Factors; D058490:Disorder of Sex Development, 46,XY / Q000150:complications*; D006801:Humans; D007248:Infertility, Male / Q000209:etiology*; D008297:Male; D009095:Mullerian Ducts
keywords: Ectopie testiculaire; Infertility; Infertilité; Persistent Müllerian duct syndrome (PMDS); Syndrome de persistance des canaux mésonéphrotiques de Müller; Undescended testicles