Persistent müllerian duct syndrome: report of two boys with associated transverse testicular ectopia.

Persistent mullerian duct syndrome (PMDS) is an unusual form of male pseudohermaphroditism characterized by the presence of a uterus and fallopian tubes in an otherwise differentiated male with a 46XY karyotype. Since the first report in 1939, approximately 150 new cases have been reported. Most refer to isolated cases, while a few involve siblings. Transverse testicular ectopia (TTE) is a rare condition in which one-testis crosses the midline and both the two testes are found on one inguinal sid. The ectopic testis may lie at the internal ring, in the inguinal canal or in the scrotum. Lenhossek in 1886 first described this entity and since then about 100 cases have been reported. The association of PMDS and TTE is recorded in adult literature, but to date only 9 pediatric cases have been reported in the English literature (Table ).