Uterine agenesis
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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
CompleteThe complete absence of theMullerian ducts is termedMayer-Rokitansky-Kuster-Hauser (MRKH) syndrome which includes the absence of the vagina as well.
Associations
- renal tract anomalies including renal agenesis
- skeletal: vertebral anomalies
-<p><strong>Uterine agenesis</strong> is the extreme of <a href="/articles/mullerian-duct-anomaly-classification">Mullerian duct anomalies</a> (Class I) where there is complete absence of uterine tissue above the vagina. </p><h4>Epidemiology</h4><p>The uterine agenesis-hypoplasia spectrum accounts for ~10-15% of all Müllerian duct anomalies.</p><h4>Clinical presentation</h4><p>Clinical presentation is characterised by primary amenorrhoea, with normal hormonal levels guaranteed by fully functional gonads.</p><h4>Pathology</h4><p>Complete absence of the <a href="/articles/muellerian-duct">Mullerian ducts</a> is termed <a href="/articles/mayer-rokitansky-kuster-hauser-syndrome">Mayer-Rokitansky-Kuster-Hauser (MRKH)</a> syndrome which includes absence of the vagina as well. </p><h5>Associations</h5><ul>- +<p><strong>Uterine agenesis</strong> is the extreme of <a href="/articles/mullerian-duct-anomaly-classification">Mullerian duct anomalies</a> (Class I) where there is a complete absence of uterine tissue above the vagina. </p><h4>Epidemiology</h4><p>The uterine agenesis-hypoplasia spectrum accounts for ~10-15% of all Müllerian duct anomalies.</p><h4>Clinical presentation</h4><p>Clinical presentation is characterised by primary amenorrhoea, with normal hormonal levels guaranteed by fully functional gonads.</p><h4>Pathology</h4><p>The complete absence of the <a href="/articles/muellerian-duct">Mullerian ducts</a> is termed <a href="/articles/mayer-rokitansky-kuster-hauser-syndrome">Mayer-Rokitansky-Kuster-Hauser (MRKH)</a> syndrome which includes the absence of the vagina as well. </p><h5>Associations</h5><ul>
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Image 1 MRI (T2) ( update )
Caption
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Case 1: Mayer-Rokitansky-Kuster-Hauser syndrome
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