Skip to main content


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

Persistent mullerian duct syndrome: report of four cases

Article metrics

  • 235 Accesses


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.


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.


  1. 1.

    BERKMEN F.: Persistent müllerian duct syndrome with or without transverse testicular ectopia and testis tumors. J. Urol., 1997,79: 122–126.

  2. 2.

    CHANDRASEKERA S.K., BARBER N.J., SHERIFFDEEN H.H.: Persistent mullerian duct syndrome with transverse testicular ectopia. Urology, 2003, 62: 1120.

  3. 3.

    COHEN-HAGUENAUER O., PICARD J.Y., MATTEI M.G. et al.: Mapping of the gene for anti-mullerian hormone to the short arm of human chromosome 19. Cytogenet. Cell. Genet., 1987, 44:2–6.

  4. 4.

    CUSSENOT O., FOURNIER G.: Infertilité, ambiguités sexuelles masculines et cryptorchidies. Progr. Urol., 2000, 10: 957–988.

  5. 5.

    DODAT H., DAVID L., CHAPPUIS J.P. et al.: Pseudohermaphrodisme mâle interne (homme à utérus). A propos de 6 observations. Chir. Pediatr., 1980, 21: 397–400.

  6. 6.

    GIRAUD J.P., YAO J.G.: Trois nouvelles observations de pseudo hermaphrodisme mâle interne. Chir. Pédiatr., 1990, 31: 100–102.

  7. 7.

    HERSHLAG A., LERNAU D.Z., SPITZ I.M. et al.: Persistent mullerian structures in infertile male. Urology, 1986, 28: 138–141.

  8. 8.

    HOCHREITER W., STENZL A., ALTERMATT H.J., KRAFT R., SPIEGEL T.: Urogenital malformation complex including the mullerian system. Urologe A, 1994, 33: 154–158.

  9. 9.

    JOSSO N., PICARD J.Y.: Anti mullerian hormone. Physiol. Rev., 1986, 66: 1038–1090.

  10. 10.

    JOUINI R., LEFI M., SAMI C., MANEF G., MOHSEN B., NOURI A.: Ectopie testiculaire transverse. Progr. Urol., 2002, 12: 666–667.

  11. 11.

    LANG-MURITANO M., BIASON-LAUBER A., GITZELMAN C., BELVILLE C., PICARD Y., SCHOENLE E.J.: A novel mutation in the anti-mullerian hormone gene as cause of persistent mullerian duct syndrome. Eur. J. Pediatr., 2001, 160: 652–654.

  12. 12.

    LOEFF D.S., IMBEAU S., REYES H.M. et al.: Surgical and genetic aspects of persistent mullerian duct syndrome. J. Pediat. Surg, 1994, 29: 61–65.

  13. 13.

    LUKUSA T., FRYNS J.P., VAN DEN BERGE H.: Internal male pseudohermaphroditism in a 6 week old child. Eur. J. Pediat., 1986, 145: 570–571.

  14. 14.

    MITRE A.I., CASTILHO L.N., AVARESE de figueiro A., ARAP S.: Persistent mullerian duct syndrome and prostate cancer. Urology, 2002, 60: 698.

  15. 15.

    NILSON O.: Hernia uteri inguinalis beim Mann. Acta. Chir. Scand., 1939, 83:231.

  16. 16.

    NJEH M., KOUBAA A., MASMOUDI M.L., HADJ kacem F., JLIDI R., MHIRI M.N.: Le pseudo-hermaphrodisme mâle interne ou homme à utérus. Ann. Urol., 1994, 28: 277–279.

  17. 17.

    OTSUKA T., NAKAZONO T., MATSUO Y., KANOU T., MASAKI Z., KUDO S.: Persistent mullerian duct syndrome suggested by magnetic resonance imaging. Radiat. Med., 2003, 21: 277–279.

  18. 18.

    POUMIER C., MOULIES D., ALAINJ L.: Pseudohermaphrodisme mâle interne ou homme à utérus. A propos de deux observations. Rev. Pediatr., 1982, 18: 401–405.

  19. 19.

    ROMERO F.R., FUCS M., CASTRO M.G., GARCIA C.R., FERNANDES rede C., PEREZ M.D.: Adenocarcinoma of persistant mullerian ducts remnants: case report and diffrentiel diagnosis. Urology, 2005, 66: 194–195.

  20. 20.

    SHINMURA Y., YOKOI T., TSUTSUI Y.: A case of clear cell adenocarcinoma of the mullerian duct in persistent mullerian duct syndrome: the first report case. Am. J. Surg. Pathol., 2002, 26: 1231–1234.

  21. 21.

    TAHA S.A.: Male pseudohermaphroditism: factors determining the gender of rearing in Saudi Arabia. Urology, 1994, 43: 370–373.

Download references

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.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

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

Download citation

Mots clés

  • dérivés mullèriens
  • hormone antimullérienne
  • cryptorchidie
  • hernie inguinale


  • mullerian remnants
  • antimullerian hormone
  • cryptorchidism
  • inguinal hernia