Mayer-Rokitansky-Küster-Hauser syndrome - uterine agenesis

Case contributed by Dr Dalia Ibrahim

Presentation

Amenorrhea

Patient Data

Age: 15 years old
Gender: Female

Complete absence of the uterus and vagina.

Normal both ovaries.

Right renal agenesis.

 

Absent right kidney.

Absent uterus and vagina.

Normal ovaries.

Case Discussion

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

Pathology

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

Associations

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is an anomaly that belongs to class I Mullerian duct anomalies.

There are two different form of this syndrome

  • the typical form (type A) of this syndrome is characterized by congenital absence of the uterus and upper vagina with normal ovaries and fallopian tubes.
  • the atypical form (type B) of the syndrome includes associated abnormalities of the ovaries and fallopian tubes and renal anomalies.
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Case information

rID: 28855
Case created: 16th Apr 2014
Last edited: 14th Mar 2016
System: Gynaecology
Inclusion in quiz mode: Included

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