Related Experiment Video
Updated: Jun 22, 2026

Murine Prostate Micro-dissection and Surgical Castration
Published on: May 11, 2016
[Charles II: From spell to genitourinary pathology]
Angel García-Escudero López1, A Arruza Echevarría, J Padilla Nieva
1Servicio de Urología, Hospital Txagorritxu, Vitoria, Alava, España. agareslo@telefonica.net
Insights
The Hapsburg dynasty ended with Charles II due to his complex genetic and genitourinary disorders, likely including hypospadias and a XX male syndrome, leading to infertility and death. Further genetic studies of his remains could clarify his condition.
Area of Science:
- Medical history
- Genetics
- Endocrinology
Background:
- Charles II of Spain, the last Hapsburg monarch, suffered from a debilitating genitourinary disorder that prevented him from producing an heir.
- His condition was exacerbated by a history of consanguineous marriages within the Hapsburg dynasty, leading to widespread speculation and the belief he was 'bewitched'.
Discussion:
- Analysis suggests Charles II may have had posterior hypospadias, monorchism, and an atrophic testicle, contributing to an intersex state with ambiguous genitalia.
- His phenotype aligns more with true hermaphroditism or XX male syndrome, possibly with Fragile X syndrome, rather than Klinefelter's syndrome.
- Congenital monorenal condition and chronic kidney failure due to nephropathy and recurrent urinary tract infections likely caused his death.
Key Insights:
- The Hapsburg dynasty's end in Spain was precipitated by Charles II's complex pluripathological condition.
- Endogamic matrimonial policies significantly contributed to the Habsburg dynasty's extinction.
- Genetic and chromosomal analysis of Charles II's remains is crucial for definitive diagnosis.
Outlook:
- Further research into Charles II's genetic makeup could provide definitive answers regarding his intersex condition.
- Understanding the genetic impact of royal inbreeding offers insights into historical health outcomes.
- The case of Charles II highlights the long-term consequences of consanguinity on dynastic survival.
Objectives:
We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death.
Methods:
We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with.
Results:
It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presented an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter's syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract.
Conclusions:
As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monastery.
Related Concept Videos
Anatomy of the Genitourinary System II: Bladder and Urethra
Urinary Tract Infection II: Pathophysiology
Urinary Tract Calculi II: Pathophysiology and Clinical Manifestations
Urine Studies I: Urinalysis
Anatomy of the Genitourinary System I: Kidneys and Ureters
Nursing Assessment of the Genitourinary System II: Inspection and Palpation

