OMIM 400045 eMedicine med/1702 | DiseasesDB 29664 MeSH D050090 | |
True hermaphroditism, now clinically known as ovotesticular disorder of sex development, is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue. More commonly one or both gonads is an ovotestis containing both types of tissue.
Contents
Although similar in some ways to mixed gonadal dysgenesis, the conditions can be distinguished histologically.
Etymology
The term derives from the Latin: hermaphroditus, from Ancient Greek: ἑρμαφρόδιτος hermaphroditos, which derives from Hermaphroditos ( Ἑρμαϕρόδιτος), the son of Hermes and Aphrodite in Greek mythology. According to Ovid, he fused with the nymph Salmacis resulting in one individual possessing physical traits of both sexes; according to the earlier Diodorus Siculus, he was born with a physical body combining both sexes. The word hermaphrodite entered the English lexicon in the late fourteenth century.
Presentation
External genitalia are often ambiguous, the degree depending mainly on the amount of testosterone produced by the testicular tissue between 8 and 16 weeks of gestation.
Causes
This condition is very rare. There are several ways in which this may occur.
Human prevalence
There are no documented cases in which both types of gonadal tissue function. Encountered karyotypes are 47XXY, 46XX/46XY, or 46XX/47XXY, and various degrees of mosaicism.
Although fertility is possible in true hermaphrodites, there has yet to be a documented case where both gonadal tissues function, contrary to the misconception that hermaphrodites can impregnate themselves. As of 2010, there have been at least 11 reported cases of fertility in true hermaphrodite humans in the scientific literature, with one case of a person with XY-predominant (96%) mosaic giving birth.
M.C. v. Aaronson
The U.S. legal case of M.C. v. Aaronson, advanced by intersex civil society organization interACT with the Southern Poverty Law Centre was brought before the courts in 2013. The child in the case was born in December 2004 with ovotestes, initially determined as male, but subsequently assigned female and placed in the care of South Carolina Department of Social Services in February 2005. Physicians responsible for M.C. initially concluded that surgery was not urgent or necessary and M.C. had potential to identify as male or female, but, in April 2006, M.C. was subjected to feminizing medical interventions. He was adopted in December 2006. Aged 8 at the time the case was taken, he now identifies as male. The Southern Poverty Law Centre state: "In M.C.’s condition, there is no way to tell whether the child will ultimately identify as a boy or a girl. Instead, the doctors decided to assign M.C. female and change his body to fit their stereotype of how a girl should look." The defendant in the case, Dr Ian Aaronson, had written in 2001 that "feminizing genitoplasty on an infant who might eventually identify herself as a boy would be catastrophic".
The defendants sought to dismiss the case and seek a defense of qualified immunity, but these were denied by the District Court for the District of South Carolina. In January 2015, the Court of Appeals for the Fourth Circuit reversed this decision and dismissed the complaint, stating that, "it did not “mean to diminish the severe harm that M.C. claims to have suffered” but that a reasonable official in 2006 did not have fair warning from then-existing precedent that performing sex assignment surgery on sixteen-month-old M.C. violated a clearly established constitutional right." The Court did not rule on whether or not the surgery violated M.C.'s constitutional rights. State suits were subsequently filed, and these remain outstanding.