PERSISTENT MULLERIAN DUCT SYNDROME WITH BILATERAL TOTAL ABSENCE OF WOLFFIAN DUCT STRUCTURES

Persistent Mullerian duct syndrome was found in a phenotypically norma l male with a normal 46XY karyotype. Bilateral total absence of Wolffi an duct structures was encountered during surgery. Although many cases with one or a combination of abnormalities of Wolffian duct structure s have been described, to our knowledge, this finding has not been pre viously reported. Clinical, hormonal and radiological findings are pre sented, possible explanations are discussed.