Persistent müllerian duct syndrome--a case report.

An eighteen month old phenotypically and genotypically normal male child was admitted with a left inguinal hernia and a right undescended testis. At operation, he was found to have a uterus, bilateral fallopian tubes, and a vagina in the left hernial sac. Bilateral orchidopexies and excision of the persistent Mullerian duct structures were carried out. This rare case of persistent Mullerian duct syndrome is due to a defect in Mullerian regression, which is in turn controlled by the Mullerian inhibiting substance (MIS). Orchidopexy with excision of the persistent Mullerian duct structures is usually not possible without damage to the vas deferens which is closely adherent to the wall of the uterus. The alternative of leaving the persistent Mullerian duct structures alone and performing a staged or primary orchidopexy has been suggested.