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 characterized by primary amenorrhea, 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 upper 2/3 of the vagina as well.

Associations

• renal tract anomalies including renal agenesis
• skeletal: vertebral anomalies

Siehe auch:

• angeborene Wirbelanomalien
• Fehlbildungen der Gebärmutter
• einseitige Nierenagenesie
• Müller-Gang
• Mayer-Rokitansky-Küster-Hauser syndrome

und weiter:

• Müllerian duct anomaly classification