Fróðskaparrit - 01.01.1965, Page 92

100
True Hermaphroditism
external genitalia may present many varieties. The gonads may
be formed as a testis on one side and an ovary on the other.
According to Hinman’s classification16 this is designated lateral
hermaphroditism. Often a bilateral hermaphroditism with
testicular and ovarian tissue on the same side will be found,
sometimes in the form of an ovotestis. The most frequent
finding is unilateral hermaphroditism characterized by an ovo-
testis on one side and an ovary or a testis on the other17. The
external genitalia are most often of masculine appearance. A
characteristic finding is gynaecomastia after the age of puberty
and the frequent development of inguinal hernia (43%), during
the repair of which the condition is most often revealed18.
There is often hypospadias, and sometimes urethroscopy will
reveal a small vaginal opening in the posterior urethra, through
which menstrual bleeding may mix with the urine at monthly
intervals in some cases. Sexual asymmetry of the body has been
described. Unilateral gynaecomastia associated with an ovary
on the same side and ovotestis on the other has been observed19,
and this condition is analogous with the gynandromorphism
which is seen in some insects.
The aetiology of true hermaphroditism is unknown and un-
doubtedly multifarious. It may be theorized that the under-
lying cause is gene mutations, unknown intrauterine causes,
disturbances of the cortico-medullary balance, or chromo-
somal abnormalities arising during meiosis or mitosis and
resulting in abnormal numbers of chromosomes, abnormal
chromosomal combinations or arrangements. Only once
familial occurrence of true hermaphroditism has been re-
ported27.
Diagnostic aids include the chromatin and chromosome stu-
dies previously discussed and a general physical examination
including hormone analysis and the important gonadal bi-
opsy24. As is the case in any disorder where the genitalia
deviate from normal, the investigations must aim at deter-
mining 1) the genetic sex, 2) the gonadal sex, 3) the somatic
sex, 4) the hormonal status, and 5) the psychical structure20.