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The Habsburg Jaw
The Habsburg Jaw
The family members occupying the Spanish Throne for some 200 years were not profiles in beauty.
William J. Maloney, D.D.S.
The Habsburg dynasty was one of the primary dynasties of Europe for hundreds of years, starting with their rule of Austria in 1282. The Habsburgs ruled Spain from the early 1500s until the death of Charles II in 1700. Charles’s death marked the end of this period, during which the Habsburg family controlled kingdoms in Italy, Spain, the Netherlands, Germany and Hungary. The collapse of most dynasties and regimes usually stems from factors such as war, political or religious turmoil, corruption or financial upheaval. The demise of the Habsburgs was due simply to genetics, the ultimate result of their practice of intermarriage, which they ironically thought would ensure their continued place on the thrones of Europe and was best illustrated by a craniofacial anomaly collectively known as “The Habsburg jaw.”
The name Habsburg is derived from the castle that bore that name, built in 1020 by Werner, bishop of Strasbourg and his brother-in-law Count Radbot, overlooking the Aar River in present-day Switzerland.[2] Hapsburg jaw, mandibular prognathism, is a rare, genetic developmental defect characterized by the abnormal forward projection of the mandible beyond the standard relation of the cranial base.[3] In the general population, prognathism affects between 2.0% and 4.0% of adults.[4] In most cases, it is simply an inherited condition and is not indicative of any other underlying medical condition. However, in some instances, prognathism may be a symptom of certain medical conditions, such as acromegaly, Crouzon syndrome, Down syndrome, acrodysostosis and basal cell nevus syndrome.[4]
The familial members of the House of Habsburg exhibited certain physical manifestations and characteristics of the Habsburg jaw throughout the centuries. These include a prominent nose,[5] thickened lower lip,[5] flat molar areas,[5] an oxycephalic head shape due to synostosis of all cranial sutures,[5] and a maxillary deficiency that is a condition whereby the bones that form the upper jaw don’t fully develop.[6]
“Your Majesty, shut your mouth, the flies of this country are very insolent.”
Shouted to the Holy Roman Emperor Charles II of Spain by a Spanish peasant[1]
Unfortunate Unions
Many royal families have viewed marriage as a means of making alliances and ensuring continuity of their reign. As a result, the Habsburgs were not the only royal family to be affected by genetic disorders. Queen Victoria’s recessive trait for hemophilia spread throughout the ruling families of Europe, with particularly tragic consequences for the Romanov family of Russia. In the case of the Hapsburgs, the many deleterious genetic effects of intermarriage guaranteed their fall from power. The Spanish Habsburg rulers had 34 children, half of whom died before the age of 10, some even before their first birthday. By contrast, the death rate for the average family of the time was only one in five, even though these nonroyals had significantly fewer resources than the royal Habsburgs.[7]
Modern research has determined the average inbreeding coefficient of the Habsburgs to be .093. This means that over 9% of the genes of each member of the Habsburg family were identical. As a point of reference, the inbreeding coefficient of two first cousins is .0625.[8]
Charles II was the last in the line of Spanish Habsburg kings. The genetic effect of intermarriage manifested itself to a terrible degree in Charles. His physical characteristics earned him the nickname El Hechizado, or “The Bewitched.” Charles’s inbreeding coefficient was .25, as his mother and father, already being highly inbred themselves, were niece and uncle.[8] Francesco Niccolini, the Apostolic Nuncio to Portugal, described Charles, at the age of 25, as being “short rather than tall, not badly formed, ugly of face, he has a long neck, a long face that curves inwards; the lower lip typical of the House of Austria … He can’t straighten his body, rather when he walks he curls up against a wall, a table or something else. His body is as weak as his mind.” [9]
Charles II died at the age of 54 with no children. The War of the Spanish Succession (1701-1714) followed his death. His heirs, Philip of Anjou and Charles of Austria, struggled for control of the Spanish Empire amid conflicting claims.
Felled by His Family Tree
The Habsburg jaw illustrates the genetic conditions that led to the downfall of a dynastic family that ruled throughout Europe for centuries. The medical dangers of consanguinity and the severity of the genetic maladies faced by the Habsburgs are evident in the autopsy report of Charles II. The doctor wrote that his body “did not contain a single drop of blood, his heart was the size of a peppercorn, his lungs corroded, his intestines rotten and gangrenous; he had a single testicle, black as coal and his head was full of water.”[10]
Modern researchers feel that Charles suffered from pituitary hormone deficiency, posterior hypospadias, monarchism, an atrophic testicle, a congenital monokidney with stones and infections and an intersexual state.[10] It is no wonder that the genetic condition referred to as The Habsburg jaw led to the collapse of one of the greatest dynastic families in world history.
Dr. Maloney is clinical associate professor, New York University. He holds a postgraduate certificate in healthcare writing from Harvard Medical School, is a fellow of the New York Academy of Medicine and a member of The Explorers Club. Queries about his article can be sent to Dr. Maloney at wjm10@nyu.edu.
REFERENCES
1. Grabb WC, Hodge GP, Dingman RO, O’Neal RM. The Habsburg jaw. Plast Reconstr Surf 1968;420:442-445.
2. Web Gallery of Art. Hapsburg, House of. Accessed on 6/16/23. Available at: https:/www.wga.hu/tours/gothic/history/ habsburg.html.
3. Genetic and Rare Diseases Information Center. Prognathism mandibular. Accessed on: 6/15/23. Available at: https:// rarediseases.info.nih/gov/diseases/10319/prognathism-mandibular.
4. Cleveland Clinic. Prognathism. Accessed on 6/15/23. Available at: https://my.clevelandclinic.org/health/diseases/22872-prognathism.
5. Wolff G, Wienker TF, Sander H. On the genetics of mandibular prognathism: analysis of large European noble families. J Med Genet 1993 Feb; 30 (2):112-116.
6. Saplakoglu Y. Inbreeding caused the distinctive ‘Habsburg Jaw’ of 17th century royals that ruled Europe. 12/2/19. Live Science. Accessed on 6/6/23. Available at: https://livescience.com/habsburg-jaw-inbreeding.html.
7. Yong E. How inbreeding killed off a line of Kings. National Geographic. April 14, 2009.
8. Thulin L. The distinctive ‘Habsburg Jaw’ wasn’t likely the result of the royal family’s inbreeding. Smithsonian Magazine. December 4, 2019.
9. Ansende M. Research confirms that intermarrying caused the ‘Habsburg jaw’ in Spanish royals. El Pais. December 4, 2019.
10. Medium. The autopsy of Charles II of Spain. Accessed on 6/13/23. Available at: https://worldroyals.medium.com/theautopsy-of-charles-ii-of-spain-d659015cb30.
