Table 1.

Malformations based upon abnormal Müllerian duct development

Malformation	Possible cause(s)
Complete absence of the uterus and upper vagina	Failure of MD development or failure of WD development.
Separate hemiuteri	Incomplete fusion of the MDs
Uterus didelphys	Incomplete fusion of the MDs
Uterus duplex	Incomplete fusion of the MDs
Uterus unicornis	Unilateral aplasia of a MD
Bilateral or unilateral doubling of the uterine tubes or uterus	Duplication of MDs
Double vagina	Failure of MD fusion
Double cervix	Failure of MD fusion
Uterus septus	Failure of regression of the septum
Vagina with septum	Failure of regression of the septum
Absence of uterine tubes, uterus, cervix and upper vagina	Complete androgen resistance in males due to Müllerian Inhibiting Substance
T-shaped uterotubal junction	Prenatal exposure to DES
Incompetent aplastic cervix	Prenatal exposure to DES
Abnormal uterine lumen	Prenatal exposure to DES
Vaginal adenosis	Prenatal exposure to DES

From (Gray and Skandalakis, 1972; Moore and Persaud, 2003; Sadler, 2004; Reed and Fenton, 2013)