G. Krob et al., TRUE HERMAPHRODITISM - CLINICAL FINDINGS, DIAGNOSTIC METHODS AND TREATMENT, Monatsschrift fur Kinderheilkunde, 144(4), 1996, pp. 362-368
True hermaphroditism is a disorder of sex determination in which ovari
an as well as testicular tis sues are present in the same individual.
Most patients present at birth with ambiguous genitalia; primary ameno
rrhoea, gynaecomastia or hematuria are common symptoms in many patient
s during puberty. Physical examination, ultrasonography, hormonal stim
ulation tests as well as genetic investigations are needed prior to ex
ploratory laparotomy to determine true hermaphroditism. Sex-assignment
should be guided by the anatomical aspect. Female true hermaphrodites
are often fertil (10 pregnancies have been published), in contrast to
infertility of male patients. The prevalence of gonadal tumours is 4.
6% in 283 patients with a higher risk for patients with a 46,XY karyot
ype. Thus the removal of the testicular tissue and replacement therapy
at puberty is recommended. Surgical treatment in true hermaphrodites
includes correction of the external genitalia and resection of the ova
rian tissue in male patients.