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Submitted by Amer Hussien, M.D & Hesham Al-Inany M.D

Congenital anomalies of the uterus

Approximately 10% of infants are born with some abnormality of the genitourinary system.


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Embryology of female genital system
Gametogenesis: a basic review


Approximately 10% of infants are born with some abnormality of the genitourinary system and anomalies in one system are often mirrored by anomalies in another system. A number of genitourinary defects may result from abnormalities in the embryological process. These may occur spontaneously or are induced by agents as DES.

Although developmental anomalies of the female genital tract are not often encountered in obstetrics, minor defects may result in an increased incidence obstetric problems

Genesis and Classification

The principal groups of deformities arise from 4 types of embryological defects:

  1. Dysgenesis or complete agenesis
  2. Failure of vertical fusion
  3. Failure of lateral fusion of the mullerian ducts which may result in symmetrical or asymmetrical abnormalities
  4. Unusual configuration of vertical and lateral fusion .

Currently there is no satisfactory classification. The terminology is often complicated with latin terms. A simple classification for Mullerian duct anomalies was suggested by Buttram and Gibbons (1979) in the 19th edition of Williams obstetrics:


I. Segmental Mullerian agenesis or hypoplasia

  1. Vaginal
  2. Cervical
  3. Fundal
  4. Combined

II. Unicornuate uterus

  1. With rudimentary horn
    • With endometrial cavity
      • Communicating
      • Non-communicating
    • Without endometrial cavity
  2. Without a rudimentary horn

III. Uterine didelphys

IV. Bicornuate uterus

  1. Complete (division to the internal os)
  2. Partial
  3. Arcuate

V. Septate

  1. Complete (septum to the intrnal os)
  2. Partial

VI. Diethylstilbestrol


 The authors stressed that vaginal anomalies may exist alone or in association with other Mullerian anomalies , but vaginal anomalies were not classified because they were not associated with fetal loss. Vaginal anomalies using their scheme were most often associated with classes III and IV.  

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