[The persistent müllerian duct syndrome with transverse testicular ectopia. A hypothesis on the role of müllerian inhibiting factor in the process of testicular migration].
The authors report a rare case of persistent mullerian duct syndrome (PMDS) with transverse testicular ectopia and inguinal hernia in a 2-year-old child with family history for this syndrome. At operation the observation of a very long and thin gubernaculum and extreme mobility of both testes and uterus, which are located in the same hernial sac, allowed the Authors to propose a hypothesis to explain the role of MIF (Mullerian Inhibiting Factor) in testicular descent. Patients with PMDS present a normal outgrowth and migration phases of the gubernaculum but lack of the gubernacular regression phase. These data suggest an important function of the MIF in this phase of testicular descent.
Asero
L
L
Divisione di Chirurgia Pediatrica, Ospedale Vittorio Emanuele II, Catania, Italia.
Meli
R
R
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D054304:Anti-Mullerian Hormone; D002675:Child, Preschool; D003456:Cryptorchidism / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D006023:Glycoproteins; D006131:Growth Inhibitors / Q000502:physiology*; D006552:Hernia, Inguinal / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008297:Male; D009095:Mullerian Ducts / Q000002:abnormalities* / Q000601:surgery; D013577:Syndrome; D013735:Testicular Hormones / Q000502:physiology*; D013737:Testis / Q000601:surgery
D006023:Glycoproteins; D006131:Growth Inhibitors; D013735:Testicular Hormones; D054304:Anti-Mullerian Hormone