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Sindrome di Rokitansky


Rokitansky syndrome, or Mayer-Rokitansky-Kuster-Hauser syndrome, is a condition which is already present at birth. Its incidence is estimated to be at least 1 out of 4000 born females.

This condition is characterised by the absence of a uterus and vagina, while
the ovaries are present and the external genital area (i.e. the pubic hair, clitoris, labia) is normal.
Women with Rokitansky syndrome have normal chromosomes and normal female hormonal function. In some cases there may be associated anomalies of other organs and systems, mainly the urinary, heart and/or skeletal systems.

syndrome has been named after its most reknown discoverer, Austrian pathologist Karl von Rokitansky (Cecoslovakia, 1804-1878), physician and professor at the University of Vienna. There is evidence of the absence of the vagina since ancient times. In the manuscript “The Nature of women”, Hippocrates describes the presence of a membranous obstruction of the vaginal canal. In 1829 Mayer and in 1838 Rokitansky, describe a syndrome that includes absence of the uterus and vagina, while subsequently Kuster will suggest an association with urologic anomalies. This explains why the condition is also named Mayer-Rokitansky-Kuster-Hauser syndrome.