ABSTRACT
Plato, Aristotle, Galen, and Hippocrates were among many early physicians and philosophers whose hypotheses on the origin of the intersex child were based on natural phenomena. This is in marked contrast to the Romans, who saw the birth of such children as an evil omen, and promptly destroyed these children by drowning or abandoning them in an open field to die of exposure.7,8 This fear and loathing of the intersex child persisted throughout the Dark Ages. Antide Colles of Dole (1599) was accused of having intercourse with the devil, as ‘evidenced’ by her ambiguous genitalia. She was tortured until she confessed to the act, and then burned at the stake for her ‘sin’. Ambrose Pare published a paper entitled ‘Monsters and Marvels’ in 1573, which was the beginning of the naturalization and medicalization of intersex and other congenital disorders.9-13
In the USA and in most western European countries, female pseudohermaphroditism secondary to congenital adrenal hyperplasia is the most common intersex disorder. In South Africa, however, true hermaphroditism is the most common disorder;14 this may be explained by the fact that children with adrenogenital syndrome may die from adrenal insufficiency before they can be evaluated. The karyotypes vary tremendously in true hermaphrodites in various parts of the world. In South Africa, most true hermaphrodites are 46XX. In Europe, over 50% of true hermaphrodites are mosaics. In Japan, virtually all true hermaphrodites are XY. In the USA, roughly 80% of true hermaphrodites have an XX karyotype, with the remainder being mosaics and XYs.14-19 There have been 449 cases of true hermaphroditism recorded in the world literature since 1899; of these, 25% were diagnosed after the subject reached the age of 20.20,21 True hermaphroditism has been reported in siblings.22
