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DESCRIPTION
Primary amenorrhea is the complete absence of menstruation in a young woman who is at least 16 years old, or at age 14 with a lack of normal growth or absence of normal sexual development. It is a rare disorder as over 95% of girls have their first menstrual period by age 15.
FREQUENT SIGNS & SYMPTOMS
No menstrual flow. Lack of menstrual periods after puberty. Most girls begin menstruating by age 14, average age is 12 years and 8 months.
CAUSES
Usually unknown. Primary amenorrhea in the United States occurs in less than 0.1% of girls. Possible causes include:
- Delayed puberty.
- Congenital abnormalities, such as the absence or abnormal formation of female organs (vagina, uterus, ovaries). Vaginal septum, cervical stenosis, imperforate hymen.
- Intact hymen (imperforate hymen), the membrane covering the vaginal opening that has no opening to allow passage of menstrual flow.
- Gonadal dysgenesis.
- Hypoglycemia.
- Hypothyroidism and hyperthyroidism.
- Cystic fibrosis.
- Cushing's disease.
- Polycystic ovarian disease.
- Chronic (long term) illness.
- Chromosome disorders and abnormalities. Turner's syndrome (XO) or Sawyer's syndrome (XY).
- Systemic disease or infection.
- Hypogonadotropic hypogonadism.
- Hyperprolactinemia.
- Testicular feminization.
- True hermaphroditism.
- Adrenogenital syndrome.
- Congenital heart disease (cyanotic).
- Congenital adrenal hyperplasia.
- Craniopharyngioma. Pituitary tumors, ovarian tumors, adrenal tumors.
- Prader-Willi syndrome.
- Malnutrition.
- Drastic weight reduction (from poverty, fad dieting, anorexia nervosa, bulimia, strenuous exercise or other causes).
- Extreme obesity.
- Pregnancy.
RISK INCREASES WITH
Stress.
Use of drugs, including oral contraceptives, anticancer drugs, barbiturates, narcotics, cortisone drugs, chlordiazepoxide and reserpine.
Excessive exercise.
Family tendency to start menstruation late.
Excessive dieting or weight loss.
Psychological eating disorders such as Bulimia or Anorexia Nervosa.
PREVENTIVE MEASURES
Don't use drugs unless prescribed by a health care provider.
Don't use mood-altering, mind-altering, stimulant, or sedative drugs.
Reduce athletic activities if they are too strenuous. Maintain a healthy muscle-fat ratio in the female body.
Obtain medical and/or psychological treatment for any underlying disorder.
Maintain proper nutrition and body weight. A growing body during adolescence needs plenty of healthy nutritious food to grow.
EXPECTED OUTCOME
The absence of menstruation is not a health risk in itself, but the cause should be identified. If an ovarian cyst or tumor is the cause, it requires removal.
Amenorrhea is usually curable with hormone treatment or removal of the underlying cause. Treatment may be delayed to age 18 unless the cause can be identified and treated safely.
Causes which sometimes cannot be corrected include chromosome disorders and abnormalities of the reproductive system.
Overall 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 (resulting from poverty or fad dieting).
- Hypoglycemia.
- Extreme obesity.
- Hypogonadotropic hypogonadism.
- Chronic illness.
- Malnutrition.
- Congenital heart disease (cyanotic).
- Hyperthyroidism.
- Imperforate hymen.
- 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 genital system.
- Gonadal dysgenesis.
- Turner's 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 pseudomenstruation with medications to help the young woman feel more like her friends or family.
POSSIBLE COMPLICATIONS
Psychological distress about sexual development. Emotional distress or crisis about being different from friends or family can occur.
Inability to conceive.
Rare endocrinological condition may require surgical treatment in addition to hormone replacement.
TREATMENT
GENERAL MEASURES
DIAGNOSIS
Physical examination and medical history is taken and assessed.
Diagnostic tests may include a thorough physical examination, and a medical and personal history, laboratory studies of blood samples to check for hormone levels, plus thyroid and adrenal function studies.
- Urine pregnancy test.
- Progesterone withdrawal.
- Chromosome analysis.
- Serum chemistry (serum gonadotropin).
- LH
- FSH
- Prolactin
- TSH
- T3 and T4
- Urine chemistry, 17-ketosteroids.
- Head CT.
- Head MRI scan.
- Laparoscopy.
- Ultrasound, pelvic region.
TREATMENT
Treatment depends on the cause of the missing period. Primary amenorrhea caused by birth defects may require medications (hormone therapy), surgery, or both.
Treatment usually involves hormone replacement therapy. Treatment for amenorrhea not related to hormone deficiency depends on the cause.
If the amenorrhea is caused by a tumor of 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.
Surgery (minor) to create an opening in the hymen, if necessary.
Surgery to correct abnormalities of the reproductive system, if possible.
MEDICATION
You may be prescribed progesterone (hormone) treatment to induce bleeding. If bleeding begins when progesterone is withdrawn, the reproductive system is functioning. This also indicates that pituitary disease is unlikely. If progesterone withdrawal does not induce bleeding, gonad stimulants such as clomiphene or gonadotrophins may be used for the same purpose.
ACTIVITY
No restrictions. Exercise regularly, but not to excess.
Sleep at least 8 hours every night.
DIET
Eat three well-balanced meals a day.
If you are overweight or underweight, get medical advice about diets. Don't try to lose weight by crash-dieting. Avoid fad diets since they can be very nutritionally unhealthy.
MoonDragon's Nutrition Information & Index
NOTIFY YOUR HEALTH CARE PROVIDER IF...
You are 16 years old and have never had a period.
Periods don't begin in 6 months, despite treatment.
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