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Uterine agenesis

Last revised by Dr Mohamed Saber on 01 Aug 2021

Uterine agenesis is the extreme of Mullerian duct anomalies (Class I) where there is a complete absence of uterine tissue above the vagina. 

The uterine agenesis-hypoplasia spectrum accounts for ~10-15% of all Müllerian duct anomalies.

Clinical presentation is characterized by primary amenorrhea, with normal hormonal levels guaranteed by fully functional gonads.

The complete absence of the Mullerian ducts is termed Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome which includes the absence of the upper 2/3 of the vagina as well. 

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Cases and figures

  • Case 1: Mayer-Rokitansky-Kuster-Hauser syndrome
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  • Case 2: Mayer-Rokitansky-Küster-Hauser syndrome
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  • Case 3: uterine hypoplasia
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