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

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 pseudohermaphroditism 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 organs in 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.

Uncommon content in congenial inguinal hernia

“…The presence of the uterus may be found in association with other several disorders of sexual development. [ 2 ] Diagnosis is usually clinical and rarely requires any evaluation. The imaging modality of investigation is not a routine in our set up for congenital inguinal hernia, but many authors recommend it in cases of palpable movable mass in the groin of infants.…”