Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review

Authors

  • Michael Ahdoot Stanford University Medical Center and Stanford University School of Medicine, Stanford, California
  • Motaz Qadan Stanford University Medical Center and Stanford University School of Medicine, Stanford, California
  • Monica Santa-Maria Kaiser Santa Clara, Santa Clara, California
  • William A. Kennedy II Stanford University Medical Center and Stanford University School of Medicine, Stanford, California
  • Aaron Ilano Kaiser Santa Clara, Santa Clara, California

DOI:

https://doi.org/10.5489/cuaj.253

Abstract

A 32-year-old male presenting with a direct inguinal hernia was found to have uterine tissue extending through the inguinal canal, warranting a diagnosis of persistent Mullerian duct syndrome (PMDS). PMDS is an extremely rare form of internal male pseudo-hermaphroditism in which female internal sex organs, including the uterus, cervix and proximal vagina, persist in a 46XY male with normal external genitalia. The condition results from a congenital insensitivity to anti-Mullerian hormone, or lack of anti-Mullerian hormone, leading to persistence of the female internal sex organsin a male. Clinically, this condition is associated with cryptochoridism. Controversy persists regarding the appropriate treatment of PMDS, since resection of the remnant structures is associated with potential morbidity, but retention risks development of occasional malignancies. We review the literature and discuss various aspects of pathophysiology, diagnosis, and management of PMDS.

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Published

2013-02-20

How to Cite

Ahdoot, M., Qadan, M., Santa-Maria, M., Kennedy II, W. A., & Ilano, A. (2013). Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review. Canadian Urological Association Journal, 7(1-2), E121–4. https://doi.org/10.5489/cuaj.253

Issue

Vol. 7 No. 1-2 (2013): CUAJ February

Section

Case Report

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