Persistent mullerian duct syndrome with an irreducible inguinal hernia.

Received January 2008 Accepted October 2008 INTRODUCTION Persistent mullerian duct syndrome (PMDS), or hernia uteri inguinale, describes a group of patients with a 46,XY karyotype and normal male external genitalia, but internal mullerian duct structures. Typically, these phenotypic males have unilateral or bilateral undescended testes, bilateral fallopian tubes, a uterus, and an upper vaginal draining into a prostatic utricle. The condition is commonly diagnosed after mullerian tissue is encountered during inguinal herniorrhaphy or orchidopexy.(1) PMDS is a form of male pseudohermaphroditism. When it presents through an inguinal hernia, it is referred as hernia uteri inguinale.(2) Persistent mullerian duct syndrome results from either a deficiency of antimullerian hormone (AMH) activity or by an abnormality in its receptor. Antimullerian hormone, produced by fetal testicular Sertoli cells, is responsible for the involution of embryonic mullerian structures in normal males.(2) Approximately, 150 cases of PMDS have been reported, whereas hernia uteri inguinale is even scarcer.(3) Familial association has been found in some cases.(4) The condition may occur sporadically or be inherited as an X-linked, autosomal dominant, or autosomal recessive pattern.(5) In transverse testicular ectopia, both testes descend through the same inguinal canal into the same scrotal sac. Persistent mullerian duct syndrome associated with transverse testicular ectopia is much rarer.(6) We report a case of PMDS with liomyoma incidentally found during surgery for irreducible inguinal hernia.