Gonads and genital ducts in a case of male pseudohermaphroditism

We describe a patient with a 46,XY karyotype, ambiguous external genitalia and partial 17a-hydroxylase deficiency in whom we performed a light microscopic study of the gonads and genital ducts. The right and left testes and epididymides were hypoplastic whereas the right vas deferens was normal, the left one was atretic and a left infundibular remnant was also present, which could be due to a concomitant deficiency in testicular secretions in the early stages of embryonic development or to the possibility of receptor insensitivity for testicular hormones or to a concomitant gonadal dysgenesis.