PERSISTENT MULLERIAN DUCT SYNDROME (HERNIA UTERI INGUINALE)

Ambigous genitalia comprises of male pseudohermaphrodite, female pseudohermaphroditism,mixed gonadal dysgenesis, and true hermaphrodite. We are dealing with male pseudohermaphrodite in this article, Transeverse testicular ectopia is a rare disorder which arises from a lack of anti Mullerian hormone(AMH or Mullerian Inhibiting substance-MIS.)MIS acts on the Mullerian ducts, resulting in the presence of Mullerian structures in a normally virilized XY male. PMDS is due to either a mutation in the AMH gene or from a defect in the AMH type II receptor. These mutations display an autosomal recessive mode of inheritance. Risk of testicular malignancy is the same as that for an intra abdominal testis. Testicular tumours such as seminoma , non seminomatous germ cell tumours, testicular carcinoma and clear cell adenocarcinoma have all been reported and can cause obstructive uropathy.