Male form of persistent mullerian duct– A case report

Abstract

Persistent Müllerian duct syndrome(PMDS) is a developmental disorder. The males suffering from this disorder also bear uterus and fallopian tube which belong to the female reproductive system. During the development of fetus a structure known as the Mullerian or the Paramesonephric  duct gives rise to the uterus and the fallopian tube. Generally, Mullerian duct degenerates in males during the early development , however if this persist it leads to PMDS. Affected males although have the normal genotype (46,XY) and normal external male genitalia. Males with persistent Müllerian duct syndrome usually present with undescended testes (cryptorchidism) or inguinal hernias.The uterus and fallopian tubes are usually discovered when surgery is performed to treat these conditions. PMDS (PMDS) is usually an accidental finding either during orchipexy or during routine inguinal hernia repair in male patients. It is caused by a defect in the mullerian inhibiting substance system. PMDS is a rare disorder; however, the prevalence of the condition is unknown. The authors report a case of a 11year old male child with PMDS and bilateral  undescended testis.