Congenital utero-vaginal anomalies

Last revised by Gamal Elsayed Abdelmoamen Fares on 6 Apr 2022

There are many classification systems for congenital utero-vaginal anomalies. These include:

  • Buttram and Gibbons classification 2
  • American Fertility Society (AFS) classification
  • Modified Rock and Adam - AFS classification
Modified Rock and Adam - AFS classification

This classification divides congenital uterine anomalies into four main types:

  • class I: dysgenesis of Müllerian ducts
    • includes agenesis or hypoplasia of the müllerian duct derivatives: the uterus and upper two-thirds of the vagina
    • the most common form is the Mayer-Rokitansky-Kuster-Hauser syndrome which is the combined agenesis of the uterus, cervix, and upper portion of the vagina
  • class II: disorders of vertical fusion
    • these anomalies are due to failure of fusion of the müllerian system with the sinovaginal bulb
    • they include cervical dysgenesis and obstructive and nonobstructive transverse vaginal septa
  • class III: disorders of lateral fusion
    • describes anomalies that result in a duplicated or partially duplicated reproductive tract
    • these disorders are due to impaired fusion and/or septal resorption of fusing Müllerian ducts attempting to form the uterus, cervix, and upper vagina
    • it includes anomalies due to failure of fusion of the paired müllerian ducts (as in didelphic and bicornuate uteri) and failure of midline septum resorption after fusion (as in septate uterus)
    • disorders due to lateral fusion defects are further subclassified into 
      • a: symmetric non-obstructive forms seen in five types: unicornuate, bicornuate, didelphic, septate, and DES-related uteri
      • b: asymmetric obstructive forms seen in three types: unicornuate uterus with obstructed horn, double uterus with unilaterally obstructed horn, and double uterus with unilaterally obstructed vagina
  • class IV: unusual configurations and combinations of defects

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