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Le syndrome de la persistance des dérivés mullèriens: A propos de quatre observations

Persistent mullerian duct syndrome: report of four cases

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RéSUMé

Le syndrome de la persistance des dérivés mullèriens est une anomalie héréditaire caractérisée par la non régression des canaux de Müller. II se traduit par la présence d’un utérus et/ou de trompes et de la partie supérieure du vagin chez un sujet masculin ayant un caryotype 46 XY.

Nous en rapportons quatre observations; l’âge de nos patients varie 20 mois à 43 ans. La découverte de ces structures a été faite en per opératoire lors d’une cure chirurgicale d’une ectopie testiculaire bilatérale chez deux patients et lors d’une cure chirurgicale d’une volumineuse hernie inguinale chez les deux autres; ces structures ont été enlevées dans deux cas et laissées en place dans les deux autres. L’abaissement des deux testicules a été réalisé dans les 4 cas.

Abstract

Persistent mullerian duct syndrome is a relatively rare inherited defect of sexual differentiation characterised by failure of regression of the mullerian ducts in males. In affected individuals, the uterus and tubes are present due to a defect of synthesis or action of antimullerian hormone normally produced by testicular Sertoli cells.

The authors report four cases with a mean age of 20 years. All patients were phenotypically normal males, with bilateral cryptorchidism in two cases and unilateral irreducible inguinal mass in the other two cases. The mullerian ducts were removed in two cases and left in place in two cases as they were intimately adherent to the vas deferens. Long-term follow-up was decided due to the risk of malignant transformation of these remnants recently described in literature.

According to the authors, the best management of these structures is excision and orchidopexy. In the case of adult infertility with testicular atrophy, gonadectomy and androgen replacement therapy are recommended.

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Author information

Correspondence to Hammadi Fakhfakh or Lobna Ayadi or Ines Samet or Kamel Chabchoub or Mohamed Njeh or Salah Boujelben or Ali Bahloul or Tahya Boudawara or Mohamed Nabil Mhirm.

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Fakhfakh, H., Ayadi, L., Samet, I. et al. Le syndrome de la persistance des dérivés mullèriens: A propos de quatre observations. Androl. 17, 49–54 (2007) doi:10.1007/BF03041155

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Mots clés

  • dérivés mullèriens
  • hormone antimullérienne
  • cryptorchidie
  • hernie inguinale

Key-words

  • mullerian remnants
  • antimullerian hormone
  • cryptorchidism
  • inguinal hernia