PERSISTENT MULLERIAN DUCT SYNDROME

Persistent mullerian duct syndrome (PMDS) is a rare form of male pseud ohermaphroditism. We present 5 cases with PMDS (2 cases associated wit h testicular malignancy) and discuss the diagnosis and management. Man agement strategies of PMDS have changed. Whereas in the past, removal of the mullerian remnants was targeted together with orchidopexy or -e ctomy, this is no longer recommended. However, testicles that cannot b e descended at an early stage are at a high risk of malignancy and sho uld, therefore, be removed. if this is necessary on both sides, there is the additional problem of lifelong testosterone substitution which requires efficient patient monitoring and good patient compliance. In cases where this cannot be achieved, compromises, such as temporarily delayed orchidectomy, may be considered.