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Chapter 9 - Rokitansky Syndrome

Published online by Cambridge University Press:  28 January 2023

By
Gail Busby
Edited by
Naomi S. Crouch  and
Cara E. Williams
Naomi S. Crouch
Affiliation:
St Michael's Hospital, Bristol
Cara E. Williams
Affiliation:
Liverpool Women's Hospital
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Summary

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].

Type
Chapter
Information
Paediatric and Adolescent Gynaecology for the MRCOG , pp. 67 - 76
Publisher: Cambridge University Press
Print publication year: 2023

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References

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