Congenital Uterine Malformations

A congenital disorder is one that occurs during fetal development and is present at birth. A woman’s reproductive organs take shape when she is still a fetus inside her mother’s uterus.  They develop from two ducts known as the müllerian ducts.  The female reproductive tract includes the fallopian tubes, uterus, cervix, and upper two thirds of the vagina. A wide variety of malformations can occur when this system is disrupted. Sometimes the uterus and fallopian tubes may not form like they should. These malformations are called müllerian anomalies or defects. Kidney problems may co-exist in women with müllerian abnormalities, mainly because the kidneys develop next to the müllerian system.

The most common types of müllerian anomalies are:

  • Müllerian agenesis, which is a failure to form the uterus and fallopian tubes
  • Disorders of fusion, which occur when the müllerian ducts fail to come together correctly

Agenesis & hypoplasia: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is most common. All or part of the müllerian tract fails to form, or is extremely underdeveloped.

Unicornuate uterus: When one müllerian duct is underdeveloped or fails to develop, a banana-shaped half-uterus is formed. A missing kidney or other kidney problems accompany this asymmetric anomaly more than they do other müllerian anomalies.

Uterus didelphys: Commonly referred to as a “double uterus”. There may be complete duplication of the vagina, cervix and uterus, and the two halves may be divided by a band of connective tissue.

Bicornuate uterus: Described as a womb with two horns. The womb is shaped like a heart, with a deep indentation at the top. This means that the baby has less space to grow than in a normally shaped womb.

Septate uterus: Occurs when the inside of the uterine cavity is divided by a wall (the septum). The septum may extend only part way into the uterus or it may reach as far as the cervix.

Arcuate uterus: The fundus of the uterus may be indented slightly both inside and outside. The shape is so slight that it is considered a variation of normal.

Müllerian anomalies are often recognized at the onset of puberty — when an adolescent begins to menstruate or when a young female fails to get her menstrual period. The condition may also be diagnosed when a woman has trouble conceiving, or maintaining a pregnancy. Some anomalies are associated with abdominal or pelvic pain, discomfort during sex, or menstrual abnormalities.

Imaging technology can help diagnose a müllerian anomaly. Diagnostic testing may include:

vaginal sonogram, magnetic resonance imaging (MRI), hysterosalpingogram (HSG), and laparoscopy/hysteroscopy.  A patient may need a combination of the above tests to reach the most accurate diagnosis.

In many cases, the condition can be left untreated, in particular when it does not significantly affect reproduction. A bicornuate uterus, for example, is associated with preterm labor and delivery but is rarely treated surgically.  Müllerian anomalies that prevent menstruation or cause significant pain are usually surgically treated. Surgery can repair the defect, eliminate discomfort during menses or sexual relations and improve fertility and pregnancy outcomes.

Some of the Müllerian anomalies, such as a septate uterus, are more associated with recurrent miscarriages than they are infertility.  The septum may be removed hysteroscopically, thus improving her chances for a successful pregnancy.

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