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Diseases

Genetic and Rare Diseases Information Center (GARD)

Persistent Mullerian duct syndrome


Other Names for this Disease
  • Female genital ducts in otherwise normal male
  • Persistent mullerian duct syndrome, types 1 and 2
  • Hernia uteri inguinale
  • Persistent oviduct syndrome
  • PMDS
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Treatment

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How might persistent Müllerian duct syndrome be treated?

Treatment for persistent Müllerian duct syndrome (PMDS) focuses on reducing the risk of malignancy (cancer), while maintaining maximal fertility. In affected males with undescended testes, the rate of testicular cancer is about 12%.[1] Therefore, surgery to place the testes within the scrotum (orchidopexy) is recommended.

Beyond orchidopexy, whether to remove Müllerian structures has been controversial. The decision to remove the Müllerian structures depends largely on maximizing testicular function (and fertility) and minimizing morbidity. Several authors have stated that the Müllerian structures should not be removed due to a presumed low risk of malignancy; difficulty removing the structures; and a risk of damaging blood supply to the testes. However, there have since been increasing reports of Müllerian malignancies associated with leaving the Müllerian structures. Therefore, some authors of more recent articles have recommended orchidopexy with simultaneous removal of Müllerian remnants when possible. The decision to leave or remove Müllerian remnants may be made on a case-by-case basis in order to best preserve the testes and fertility potential. If Müllerian remnants are left intact, long-term follow-up may be needed.[2][3][1]
Last updated: 10/20/2015

References
  1. Ahdoot M, Qadan M, Santa-Maria M, Kennedy WA 2nd, Ilano A. Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review. Can Urol Assoc J. January-February, 2013; 7(1-2):E121-124.
  2. Farikullah J, Ehtisham S, Nappo S, Patel L, Hennayake S. Persistent Müllerian duct syndrome: lessons learned from managing a series of eight patients over a 10-year period and review of literature regarding malignant risk from the Müllerian remnants. BJU Int. December, 2012; 110(11 Pt C):
  3. Mahmoud M. Shalaby, Adel Kurkar, Mohamed A. Zarzour, Amr A. Faddan, Mahmoud Khalil, and Mohamed F. Abdelhafez. The management of the persistent Müllerian duct syndrome. Arab J Urol. September, 2014; 12(3):239-244.


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Other Names for this Disease
  • Female genital ducts in otherwise normal male
  • Persistent mullerian duct syndrome, types 1 and 2
  • Hernia uteri inguinale
  • Persistent oviduct syndrome
  • PMDS
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.