[True hermaphroditism. Late diagnosis. Surgical treatment and a 15-year follow-up].

Abstract

True hermaphroditism is an usual cause of ambiguous genitalia. In some social areas, the diagnosis is often late and raised as pubertair abnormally. We report a case of a 12 years old Sicilian child, seen in 1975, raised as a boy and whose the main complain was a gynaecomastia. Clinical and paraclinical investigations revealed a small testicle on the one side and on ovary with an uterus and an obturated tube on the opposite side. A small recurvated penis, partially adherent to the scrotum is noticed. A structure embryologycally close to a vagina is also found behind the bladder. Cytogenetic structures showed a mixte karyotype: mosaicim 46 XX/46 XY with a ratio of a 80/20. Hormonal assessment showed a normal level of estrogen while testosterone is below the inferior threshold. A surgical treatment is carried out in three steps: removal of the internal female organs and testicular prosthesis replacement in the one side after castration, reconstruction of the recurvated penis and replacement of the other testicular prosthesis, and finally construction of the anterior urethra. Since the very first step of the surgical management and adjuvant hormonotherapy (testosterone) is administrated in order to decrease the gynaecomastia but also to allow the normal growth of the male organs. We discuss the benefice of a such therapeutic option in the true hermaphroditism lately diagnosed recording to organic and psychological data. We also point out the difficulty in therapeutic choice, mainly when the patient has raised as a boy. The follow-up in this case in 15 years.