Uterine agenesis

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

Epidemiology

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

Clinical presentation

Clinical presentation is characterised by primary amenorrhoea, with normal hormonal levels guaranteed by fully functional gonads.

Pathology

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

Associations

Abdominal and pelvic anatomy
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Article Information

rID: 7813
System: Gynaecology
Section: Anatomy
Tags: cases, uterus, variant
Synonyms or Alternate Spellings:
  • Class I Mullerian duct anomaly

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    Case 1: Mayer-Rokitansky-Kuster-Hauser syndrome
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    Case 2: Mayer-Rokitansky-Küster-Hauser syndrome
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