Transverse testicular ectopia and persistent Mullerian duct syndrome: video-assisted treatment

Persistent Mullerian duct syndrome (PMDS) is a rare autosomal recessive disorder in which, due to a deficiency of the Mullerian inhibiting substance (MIS), the complete development of the Mullerian duct structures occurs in the presence of a male phenotype and a 46 XY karyotype. In these patients, a uterus, tubes and vagina are often present which open into a normal male prostatic urethra. These female structures are usually found during surgical correction of an inguinal hernia or during a laparoscopic exploration for non palpable testis. Transverse testicular ectopia (TTE) is frequently present. Here we present the case of a 6-month-old boy with this rare association and its treatment by video-assisted excision of the Mullerian remnant, video-assisted orchidopexy and laparoscopic closure of the inguinal defects.