Amenorrhea - primary

Also known as: Primary amenorrhea, No periods - primary, Absent periods - primary, Absent menses - primary or 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, incidence, and risk factors

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

Being born with poorly formed genital or pelvic organs (missing uterus or vagina, vaginal septum, cervical stenosis, or imperforate hymen) can lead to primary amenorrhea.

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

These problems may be due to:

Genetic defects
Infections that occur in the womb or after birth
Other birth defects
Tumors

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

Conditions associated with primary amenorrhea include:

Adrenogenital syndrome
Anorexia
Chromosomal abnormalities such as Turner syndrome or Sawyer syndrome
Congenital heart disease (cyanotic)
Congenital adrenal hyperplasia
Craniopharyngioma
Chronic (long-term) illnesses
Cushing's disease
Cystic fibrosis
Gonadal dysgenesis
Hypothyroidism and hyperthyroidism
Hypogonadotropic hypogonadism
Hyperprolactinemia
Obesity
Prader-Willi syndrome
Polycystic ovarian disease
Testicular feminization
True hermaphroditism
Tumors of the pituitary or adrenal glands
Tumors of the ovaries

Symptoms

A female with amenorrhea will have no menstrual flow with or without other signs of puberty.

Signs 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 caused by birth defects may require medications (hormones), surgery, or both.

If the amenorrhea is caused by a tumor in the brain (pituitary tumor):

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

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

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

Expectations (prognosis)

Overall the outlook is good, depending on the cause of the amenorrhea. If the amenorrhea is caused by one of the following conditions, there is a good chance that it can be corrected with medication, lifestyle changes, or surgery:

Adrenogenital syndrome
Chronic illness
Congenital heart disease
Drastic weight reduction
Hypogonadotropic hypogonadism
Imperforate hymen or vaginal septum
Malnutrition
Normal delay of onset (up to age 14 or 15)
Obesity
Overactive thyroid

Periods are unlikely to start on their own if the amenorrhea was caused by one of the following conditions:

Congenital abnormalities of the upper genital system
Craniopharyngioma
Cystic fibrosis
Gonadal dysgenesis
Prader-Willi syndrome
Testicular feminization syndrome
True hermaphroditism
Turner syndrome (XO)

If the amenorrhea cannot be corrected, it is sometimes possible to create a menstrual-like situation (pseudomenstruation) with medications. Medicines can help you feel more like your friends or family, and will also protect the bones from becoming too thin (osteoporosis).

Complications

Emotional distress due to feeling different from friends or family, or worrying you might not be able to have children
Osteoporosis and increased risk of fractures

Calling your health care provider

Call your health provider if your daughter is older than age 16 and has not yet begun menstruating, or if she is 14 and shows no other signs of puberty.

References

Lobo RA. Primary and secondary amenorrhea and precocious puberty: Etiology, diagnostic evaluation, management. In: Katz VL, Lentz GM, Lobo RA, Gershenson DM, eds. Comprehensive Gynecology. 5th ed. Philadelphia, Pa: Mosby Elsevier; 2007:chap 38.

Master-Hunter T, Heiman DL. Amenorrhea: evaluation and treatment. Am Fam Physician. 2006;73(8):1374-1382.

Disease

Hyperthyroidism

Hyperthyroidism is a condition in which the thyroid gland makes too much thyroid hormone. The condition is often referred to as an “overactive thyroid.”
Disease

Cystic fibrosis

Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is one of the most common chronic lung diseases in children and young adults, and may result in early death. See also:
Disease

Prader-Willi syndrome

Prader-Willi syndrome is a congenital (present from birth) disease that involves obesity, decreased muscle tone, decreased mental capacity, and sex glands that produce little or no hormones.
Disease

Anorexia nervosa

Anorexia nervosa is an eating disorder that involves an inability to stay at the minimum body weight considered healthy for the person’s age and height. Persons with this disorder may have an intense fear of weight gain, even when they are underweight. They may use extreme dieting, excessive ...
Disease

Polycystic ovary syndrome

Polycystic ovary syndrome is a condition in which there is an imbalance of a woman’s female sex hormones. This hormone imbalance may cause changes in the menstrual cycle, skin changes, small , trouble getting pregnant, and other problems.
Disease

Bulimia

Bulimia is an illness in which a person binges on food or has regular episodes of significant overeating and feels a loss of control. The affected person then uses various methods — such as vomiting or laxative abuse — to prevent weight gain. Many (but not all) people with bulimia also ...
Disease

Hypothyroidism

Hypothyroidism is a condition in which the thyroid gland does not make enough thyroid hormone. See also: Chronic thyroiditis ()
Disease

Turner syndrome

Turner syndrome is a genetic condition in which a female does not have the usual pair of two X chromosomes.
Disease

Congenital adrenal hyperplasia

Congenital adrenal hyperplasia refers to a group of inherited disorders of the .
Disease

Intersex

Intersex is a group of conditions where there is a discrepancy between the external genitals and the internal genitals (the testes and ovaries). The older term for this condition, hermaphroditism, came from joining the names of a Greek god and goddess, Hermes and Aphrodite. Hermes was a god of ...
Disease

Androgen insensitivity syndrome

Androgen insensitivity syndrome (AIS) is when a person who is genetically male (has one X and one Y chromosome) is resistant to male hormones called androgens. As a result, the person has some or all of the physical characteristics of a woman, despite having the genetic makeup of a man.
Disease

Hypoglycemia

Hypoglycemia is a condition that occurs when your blood sugar (glucose) is too low. Blood sugar below 70 mg/dL is considered low. Blood sugars at this level can harm you.
Disease

Crohn's disease

Crohn’s disease is a form of inflammatory bowel disease (IBD). It usually affects the intestines, but may occur anywhere from the mouth to the end of the rectum (anus). See also:
Disease

Congenital heart disease

Congenital heart disease refers to a problem with the heart’s structure and function due to abnormal heart development before birth. Congenital means present at birth.
Disease

Hypogonadotropic hypogonadism

Hypogonadotropic hypogonadism is absent or decreased function of the male or the female ovaries. It is considered a form of secondary hypogonadism, which means the condition is due to a problem with the pituitary or hypothalamus gland. See also:
Disease

Craniopharyngioma

A craniopharyngioma is a benign that develops near the pituitary gland (a small at the base of the brain).

Review date:: June 16, 2010
Reviewed by:: Susan Storck, MD, FACOG, Chief, Eastside Department of Obstetrics and Gynecology, Group Health Cooperative of Puget Sound, Redmond, Washington; Clinical Teaching Faculty, Department of Obstetrics and Gynecology, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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