What is absolute uterine factor infertility?

Infertility is a disease of the reproductive system that affects about 15% of heterosexual (a man and woman) couples in the United States. For most people, infertility is diagnosed after one year of unprotected sex without a pregnancy. It’s not always clear why infertility happens. While a third of cases are due to a female factors and a third are due to male factors, the remaining third of cases are due to unknown factors or a combination of factors.

Among the female factors for infertility, absolute uterine factor infertility is a condition where a woman is unable to get pregnant because her uterus is not present or completely non-functional. The uterus is an organ specific to the female reproductive system. It consists of the fallopian tubes (which sweep up eggs after they exit the ovaries), the main body of the uterus (which is the home of the growing fetus during pregnancy) and the cervix (which connects the uterus to the top of the vagina). The uterus is roughly the size of an upside down pear and has the ability to expand to accommodate a fetus and shrink back down in size after a delivery.

There are two types of absolute uterine factor infertility: congenital and acquired. A congenital condition is one that’s there at birth.

If you have congenital absolute uterine factor infertility, it means that you were born without a uterus, a condition known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKH).

Acquired absolute factor infertility is caused when the uterus is surgically removed. This can happen for a variety of reasons and is done through a procedure called a hysterectomy. The hysterectomy may have been done to save a woman’s life, such as in life-threatening hemorrhage or a cancer diagnosis, or because of severe pain, such as in severe forms of endometriosis.

It is possible to have uterine factor infertility and still have a uterus. This can include infertility that’s caused by fibroids, polyps, scar tissue, radiation damage or injuries to the uterus that prevent a pregnancy. Asherman’s syndrome is a rare condition where the scar tissue in the uterus creates adhesions — physical blocks — inside the uterus, preventing a pregnancy. Causes of Asherman’s syndrome include infections, radiation, and uterine surgery, such as dilation and curettage (D&C) procedures.

How is absolute uterine factor infertility different from other types of female infertility?

It can often be difficult to pinpoint the reason a woman or couple has infertility issues. With absolute uterine factor infertility, however, the problem is clear. While a physical exam is usually enough for the diagnosis, use of ultrasound and other imaging can confirm that the uterus is absent.

All causes of infertility can have a great emotional toll on the people experiencing it. Different types of infertility may cause unique kinds of challenges.

For those with acquired absolute uterine factor infertility, they may have had life-threatening bleeding requiring an emergency hysterectomy to save their life, with little or no time to think about or emotionally prepare for this new, sudden form of infertility.

For people with congenital (born with) absolute uterine factor infertility, this discovery happens during the teen years when her period doesn’t start. Before then, there is usually no reason to suspect that the uterus might be missing, so this discovery can be shocking and devastating. They then face the difficulty of knowing from an early age that they do not have a uterus. For these women, questions about when to tell a new partner about their absolute infertility or worries about how the inability to have children may impact their future family life can be difficult.

What causes uterine factor infertility?

Absolute uterine factor infertility happens when the uterus is not present, the uterus is underdeveloped, or the uterus is present but is nonfunctional. There are two main causes of absolute uterine factor infertility. These causes include:

  • Being born without a functioning uterus.
  • Having the uterus removed (hysterectomy).

Being born without a uterus.

This is a rare congenital (something you are born with) condition called Mayer-Rokitansky-Küster-Hauser syndrome (MRKH). This condition causes parts of the female reproductive system to be underdeveloped — meaning the vagina can be shorter than normal or the uterus may not be the correct shape and size. In severe cases, there’s no uterus present at birth. In MRKH, the ovaries are present and functioning, so patients may still have mood changes and other signs of a menstrual cycle, but they will not have bleeding. While the ovaries aren’t affected, MRKH is associated with kidney and skeletal problems. MRKH is often diagnosed during the teen years when menstrual cycles (periods) don’t start as expected.

Having the uterus removed (hysterectomy).

Having the uterus surgically removed during childbearing years can happen for a variety of reasons. These reasons can include:

  • Hemorrhage: A hysterectomy — the procedure used to remove the uterus — can be an emergency surgery that’s used to save a life. A woman might need an emergency hysterectomy if she has a severe hemorrhage (large amounts of blood loss). This can happen during a Cesarean section (C-section) when there’s severe bleeding that needs to be controlled. In this case, a hysterectomy is a life-saving procedure for the mother, but it results in the loss of her uterus right after childbirth.
  • Cancer: Another reason the uterus could be surgically removed is cancer. When removing the uterus could help treat and stop the spread of cancer throughout the body, a hysterectomy may be an option.
  • Severe endometriosis, fibroids, or adenomyosis: A hysterectomy may also be used in severe cases of endometriosis, adenomyosis, or fibroids. These are different benign diseases (meaning they aren’t forms of cancer) in the female reproductive system that can cause very severe symptoms, including pelvic pain, bowel and bladder issues, and heavy bleeding. There are many treatments and surgeries that do not require removal of the uterus. However, in very severe cases, these other therapies aren’t enough and the uterus must be completely removed.

What are the signs or symptoms of uterine factor infertility?

In women who have absolute uterine factor infertility (who have not had a hysterectomy), the main symptom is never having had menstrual periods. This is often the sign that leads a doctor to perform a physical exam or an imaging test and reveal the diagnosis.

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