Features of persistent mullerian duct syndrome:
(1) XY male with pseudohermaphroditism or less often an XX female
(2) presence of both female and male genital structures (uterus with fallopian tube and ovary, testes)
With hernia uteri inguinale, female reproductive structures are present in an inguinal hernia, with or without undescended testes.
Possible pathologic outcomes:
(1) infertility
(2) germ cell neoplasm in the undescended testicular tissue
Issues:
(1) presence of a testis
(2) viability of the testis
(3) vascular stalk to the testis
(4) concern about malignancy
Orchiopexy is done:
(1) if there is a viable testis with an independent blood supply
(2) if the testis is viable, the blood supply can be dissected from the other female structures, and there is minimal concern about malignancy
Orchiectomy is performed:
(1) if there is a noviable testis
(2) if the testis does not have an independent blood supply and there is a concern about malignancy
The Mullerian duct structures in the inguinal hernia are removed in the male.
If orchiopexy is performed then the patient should be followed for malignancy.
