Amenorrhea - primary

Alternative Names

Primary amenorrhea; No periods - primary; Absent periods - primary; Absent menses - primary; Absence of periods - primary

Definition

Amenorrhea is the absence of menstruation. Menstruation is a woman's monthly period.

Primary amenorrhea is when a girl has not yet started her monthly periods, and she:

Has gone through other normal changes that occur during puberty
Is older than 15

Causes

Most girls begin menstruating between ages 9 and 18, with an average of around 12 years old. Primary amenorrhea typically occurs when a girl is older than 15, if she has gone through other normal changes that occur during puberty. Primary amenorrhea may occur with or without other signs of puberty.

Being born with incompletely formed genital or pelvic organs can lead to primary amenorrhea. Some of these defects include:

Blockages or narrowing of the cervix
Hymen that has no opening
Missing uterus or vagina
Vaginal septum (a partition that divides the vagina into two sections)

Hormones play a big role in a woman's menstrual cycle. Hormone problems can occur when:

Changes occur to the parts of the brain where hormones that help manage the menstrual cycle are produced
The ovaries are not working correctly

Either of these problems may be due to:

Anorexia (loss of appetite)
Chronic or long-term illnesses, such as cystic fibrosis or heart disease
Genetic defects or disorders
Infections that occur in the womb or after birth
Other birth defects
Poor nutrition
Tumors

In many cases, the cause of primary amenorrhea is not known.

Symptoms

A female with amenorrhea will have no menstrual flow. She may have other signs of puberty.

Exams and Tests

The doctor will perform a physical exam and ask questions about your medical history. A pregnancy test will be done.

Blood tests may include:

Other tests that may be done include:

Treatment

Treatment depends on the cause of the missing period. Primary amenorrhea that is caused by birth defects may require hormone medications, surgery, or both.

If the amenorrhea is caused by a tumor in the brain:

Medications may shrink certain types of tumors.
Surgery to remove the tumor may also be needed.
Radiation therapy is usually only done when other treatments have not worked.

If the condition is caused by a body-wide disease, treatment of the disease may allow menstruation to begin.

If the amenorrhea is due to anorexia or too much exercise, periods will often begin when the weight returns to normal or the exercise level is decreased.

If the amenorrhea cannot be corrected, medicines can sometimes create a menstrual-like situation. Medicines can help the woman feel more like her friends and female family members. They can also protect the bones from becoming too thin (osteoporosis).

Outlook (Prognosis)

The outlook depends on the cause of the amenorrhea and whether it can be corrected with treatment or lifestyle changes.

Periods are not likely to start on their own if the amenorrhea was caused by one of the following conditions:

Congenital defects of the upper genital system (uterus, hymen, and other structures inside the genitals)
Craniopharyngioma (a tumor near the pituitary gland at the base of the brain)
Cystic fibrosis
Genetic disorders

You may have emotional distress because you feel different from friends or family. Or you may worry that you might not be able to have children.

When to Contact a Medical Professional

Call your health care provider if your daughter is older than 15 and has not yet begun menstruating, or if she is 14 and shows no other signs of puberty.

References

Bulun SE. The physiology and pathology of the female reproductive axis. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology, 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 17.

Lobo RA. Primary and secondary amenorrhea and precocious puberty: etiology, diagnostic evaluation, management. In: Lentz GM, Lobo RA, Gershenson DM, Katz VL, eds. Comprehensive Gynecology. 6th ed. Philadelphia, PA: Elsevier Mosby; 2012:chap 38.

Styne DM, Grumbach MM. Puberty, ontogeny, neuroendocrinology, physiology, and disorders. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology, 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 25.