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Chapter 9 - Rokitansky Syndrome

Published online by Cambridge University Press:  28 January 2023

Naomi S. Crouch
St Michael's Hospital, Bristol
Cara E. Williams
Liverpool Women's Hospital
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Rokitansky syndrome, or Mayer–Rokitansky–Küster–Hauser syndrome (MRKH) also known as Müllerian agenesis results from embryonic underdevelopment of the Müllerian duct, resulting in agenesis or atresia of the uterus, vagina or both. The vagina is shortened and may appear as just a dimple inferior to the urethra. A single midline uterine remnant may be present, or there may be uterine horns (with or without an endometrial cavity) which do not communicate with the vagina. This condition belongs to the most severe (class 5) of the ESHRE/ESGE classification [1].

Publisher: Cambridge University Press
Print publication year: 2023

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