ADAM Health Encyclopedia

Amenorrhea - primary

Definition

Amenorrhea is the absence of menstruation. It refers to missing periods in a female age 16 or older.

Alternative Names

Causes

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:

• Drastic weight reduction (from poverty, fad dieting, anorexia nervosa, bulimia, very strenuous exercise, or other cause)
• Malnutrition
• Extreme obesity
• Chronic (long term) illnesses
• Genital abnormalities present since birth (absence of the uterus or vagina, vaginal septum, cervical stenosis, imperforate hymen)
• Gonadal dysgenesis
• Hypoglycemia
• Hypothyroidism and hyperthyroidism
• Cystic fibrosis
• Cushing's disease
• Polycystic ovarian disease
• Chromosomal abnormalities such as Turner's syndrome (XO) or Sawyer's syndrome (XY)
• 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
• Pregnancy

Symptoms

No menstrual flow

Exams and Tests

• Physical examination and medical history
• 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
• Ultrasound, pelvic region
• 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.

Outlook (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 (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

Emotional distress or crisis about being different from friends or family can occur.

When to Contact a Medical Professional

Call your health provider if your daughter is older than age 16 and has not yet begun menstruating.

Stenchever A. Comprehensive Gynecology. 4th ed. St. Louis, Mo: Mosby; 2001:1109-1119.

Rakel P, ed. Conn’s Current Therapy 2005. 57th ed. Philadelphia, Pa: WB Saunders; 2005:1291-1293.