Health Encyclopedia

Amenorrhea - primary

Definition

Amenorrhea is the absence of menstruation. Primary amenorrhea is not considered to have occurred until a girl is beyond age 16, if she has undergone other normal changes that occur during puberty.

Alternative Names

Primary amenorrhea; No periods; Absent periods; Absent menses

Causes, incidence, and risk factors

Most girls begin menstruating between ages 9 and 18, with an average around 12 years old. Primary amenorrhea is not considered to have occurred until a girl is beyond age 16, if she has undergone other normal changes that occur during puberty. Primary amenorrhea may occur with or without other signs of puberty.

There are many possible causes of primary amenorrhea:

Adrenogenital syndrome
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
Extreme weight loss
Genital abnormalities present since birth (absence of the uterus or vagina, vaginal septum, cervical stenosis, imperforate hymen)
Gonadal dysgenesis
Hypoglycemia
Hypothyroidism and hyperthyroidism
Hypogonadotropic hypogonadism
Hyperprolactinemia
Malnutrition
Obesity
Prader-Willi syndrome
Pregnancy
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 the patient's medical history. A pregnancy test will be done.

Blood tests may include:

Other tests that may be done include:

Progesterone withdrawal
Chromosome analysis
Urine chemistry, 17-ketosteroids
Head CT
Head MRI scan
Pelvic ultrasound
Laparoscopy

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), the tumor is usually treated with a drug called bromocriptine. 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.

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 possibility of correcting the amenorrhea through medication, lifestyle change, or surgery:

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

If the amenorrhea is caused by one of the following conditions, it is unlikely that the amenorrhea can be corrected by any method:

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

If the amenorrhea cannot be corrected, it is sometimes possible to create a menstrual-like situation (pseudomenstruation) with medications to help the young woman feel more like her friends or family.

Complications

Emotional distress or crisis about being different from friends or family and about possible inability to bear children can occur.

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. Abnormal Uterine Bleeding: Ovulatory and Anovulatory Dysfunctional Uterine Bleeding, Management of Acute and Chronic Excessive Bleeding. In: Katz VL, Lentz GM, Lobo RA, Gershenson DM. Comprehensive Gynecology. 5th ed. Philadelphia, Pa: Mosby Elsevier; 2007: chap. 37.

Master-Hunter T. Amenorrhea: Evaluation and Treatment. Am Fam Physician. Apr. 2006; 73(8): 1374-82.

Review Date:6/26/2008
Reviewed By:Susan Storck, MD, FACOG, Clinical Teaching Faculty, Department of Obstetrics and Gynecology, University of Washington School of Medicine; Chief, Eastside Department of Obstetrics and Gynecology, Group Health Cooperative of Puget Sound, Redmond, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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