EAU15: The Spanish angle: Urological problems of Charles II (1661-1700)

24 March 2015

In addition to the poster sessions, in which research into the history of our field were presented, a special, in-depth “Hispanic Urological Tales” session took place on Saturday, 21 March. Three presentations were given in the space of an hour, each highlighting a different national urological topic.

Prof. Remigio Vela Navarrete (ES) looked at the discovery and spread of syphilis, in the New World and in Europe in the early 16th Century. Prof. Schultheiss (DE), EAU History Office Chairman, discussed the hypersexuality that is evident in the works of Pablo Picasso. What emerged was a changing perspective of the artist; from participant in erotic scenes to being a spectator in his later years.

It was perhaps the presentation of Prof. Philip Van Kerrebroeck (NL) that raised the most eyebrows among the audience. Instructed to speak on the “urological problems in Spanish Royalty”, Van Kerrebroeck began with some trepidation in a room full of historically-minded Spaniards.

Charles II

Beginning with a general background on some of the Spanish dynasties of the Middle Ages, the focus soon lay on the extraordinary case of Charles II, the last and heirless Habsburg King of Spain. The result of several generations of royal inbreeding, Charles II was already born with several difficulties. Throughout his childhood he was extremely fragile and developmentally challenged. Intellectually stunted, his thirty-year rule was ineffective and dominated by the absence of heirs.

Despite being married twice, Charles II produced no offspring. After his death, just short of his fortieth birthday, a rudimentary autopsy was performed, with the following results: “a very small heart, lungs corroded, intestines putrefactive and gangrenous, three large stones in the [!] kidney, a single testicle black as coal, and his head full of water.”

Prof. Van Kerrebroeck used this description to paint a clinical picture: “We can conclude that Carlos suffered from posterior hypospadias, monorchism and an atrophic testicle. He probably had an intersexual state with ambiguous genitalia, and a congenital monokidney with stones and infections.”

Physical phenotype

Van Kerrebroeck postulated that Charles’s condition tended towards true hermaphroditism, though he was most probably XX male. He gave several possible diagnoses:

  • Klinefelter syndrome: which includes small testicles and a short penis, 47-XY/47-XXY.
  • Fragile X syndrome: a cause of 30% mental retardation, a long face, prominent ears and macrophelia, joint slackness, hyperextensibility and muscular hypotonia.
  • True hermaphroditism: expression of ovarian and testicular tissue, most commonly 46-XX/46-XY/47-XXY mosaicism, an intra-abdominal ovary (unconfirmed in the case of Charles II), cryptorchid testicle, hypospadias.
  • Sexual Inversion: male phenotype, XX karyotype with anomalous translocation of Y to X.
    Small size, ambiguous genitalia, hypospadias and testicular atrophy.

Prof. Van Kerrebroeck concluded that a combination of sexual inversion and fragile X was the most likely correct diagnosis, yielding the extraordinary symptoms that Charles II suffered with his entire life.