Crossed ectopia of ovotestis in a case of true hermaphroditism

Abstract True hermaphroditism is a rare form of intersex disorder. The authors report a case of true hermaphroditism with crossed ectopia of an ovotestis from the left to the right scrotum. The karyotype of the patient was 46,XX, without mosaicism. The majority of the ovotestis was comprised of testicular tissue. In other cases of human true hermaphroditism, descent of a pure ovary has not been noted to occur, despite the presence of the contralateral testis. Additionally, in congenital adrenal hyperplasia occurring in genotypic female subjects, pure ovaries do not descend into the scrotum despite the high level of androgens. Taken together, we believe that the demonstrated descent of mostly testicular ovotestes supports the idea that the testis produces a substance other than androgen that is important in guiding its descent.