Persistent Mullerian duct syndrome: a case-based algorithm.

Persistent Mullerian duct syndrome (PMDS) is a rare, autosomal recessive disorder. It is a form of male disorder of sexual differentiation in which Mullerian duct structures are present in male phenotypes and 46XY karyotypes. Affected individuals have a uterus, tubes, cervix, and vagina [1] [2] [3] [4]. Uterus and tubes are usually discovered during inguinal hernia surgery or diagnostic laparoscopy for impalpable testis. A testis and the Mullerian derivatives may be found in an inguinal hernia, a condition known as hernia uteri inguinalis. The contralateral testis may also have moved into the hernia, a condition known as transverse testicular ectopia [5] [6].