Presentation
Amenorrhea.
Patient Data
Age: 15 years
Gender: Female
From the case:
Mayer-Rokitansky-Küster-Hauser syndrome
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Axial T2
Complete absence of the uterus and vagina. Normal both ovaries. Right renal agenesis.
From the case:
Mayer-Rokitansky-Küster-Hauser syndrome
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
- renal tract anomalies including renal agenesis
- skeletal - vertebral anomalies.
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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