What’s a unicorn uterus?

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A unicorn uterus is an unusually shaped uterus caused by errors during embryonic development. In a person with a unicorn uterus, only half of the uterus is fully formed, with a single “horn” leading to one of the fallopian tubes. This condition is very rare. People can successfully become pregnant with this uterine abnormality, although having a unicorn uterus increases the risk of pregnancy complications, including pregnancy loss.

During embryonic development, two structures known as Muller’s ducts develop in the uterus. In some fetuses, only one duct develops fully. The unicorn uterus can have a variable appearance. Sometimes, the undeveloped duct creates a sealed partial uterus. A partial second horn may be seen in some women, although it is usually not connected to the fallopian tube.

This uterine abnormality is sometimes not diagnosed until a pregnant woman experiences complications and needs a C-section. As soon as she is opened in the operating room, the unusual shape of the uterus will immediately become apparent. In other women, the abnormality may be noticed during surgical procedures in the abdomen, including procedures such as tubal ligations.

Having a unicorn uterus can lead to fertility problems. Women who have difficulty conceiving may undergo a series of tests to determine the cause. If these tests include imaging studies of the uterus, the woman’s unusually shaped uterus may become visible. However, imaging studies cannot always reliably detect a unicorn uterus.

Women with this congenital disorder are at risk for pregnancy complications because the uterus is smaller and it’s possible for the pregnancy to implant in a bad position, potentially leading to rupture. Pregnant women who know they have a unicorn uterus should make sure their obstetricians are aware of this so plans can be made to keep the pregnancy as safe as possible. Another thing to be aware of with this abnormality is that sometimes a kidney is missing or even misshapen. This may require taking some special precautions to maintain kidney health in the remaining fully functional kidney.

The causes of unicorn uterus formation are not fully understood. In some cases, environmental exposures during embryonic development can lead to anatomical abnormalities. In other cases, there are no known risk factors for the abnormalities or it is difficult to identify risk factors because the uterine abnormality is identified so late that it is difficult to obtain accurate information about environmental exposures during the patient’s embryonic development.

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