[Absence of the inhibitor factor of the Mullerian structures. Report of a case]

A 30 year old patient with normal masculine phenotype, karyotype 46 XY with a congenital left inguinal hernia, was studied. This hernia contained (at surgery): uterus, tubes and a hypoplasic vagina, as well as testis. The Müllerian structures and the right testicle, were resected, and left orchidopexy was performed. FSH, LH, testosterone, prolactine, B-HCG in blood by radioimmunoanalysis, total estrogens, 17-ketosteroids and 17-hydroxicorticosteroids in urine, were all normal. Roentgenologic and endoscopic urologic studies were normal. A post-operative study of semen showed moderate oligospermia. The factors involved in masculine sexual differentiation, were reviewed, specially the inhibitory factor of Müllerian structures.