Amenorrhea/Oligomenorrhea

central - hypothalamic or pituitary failure

primary hypogonadotrophic - FSH is decreased

• idiopathic is number one cause
• Kallmans's syndrome:
  • X-linked
  • autosomal dominant
  • GnRH neuron deficiency
  • anosmia (no sense of smell)
• congenital hypopituitarism
• genetic: Prader-Willi, Lawrence-Moon-Beidi

secondary (or primary) is more likely environmental and reversible. FSH may be normal

• pregnancy
• hypothyroidism
• hyperthyroidism
• hypothalamic dysfunction: very common
  • drugs (ie marijuana)
  • stress
  • exercise
  • chronic disease (IBD, sickle cell, CF)
  • anorexia
• hyperprolactinemia
  • pituitary lesion
  • medications: antipsychotics, TCAs alpha methy-dopa
  • hypothyroidism
  • renal failure

• Sheehan syndrome (secondary to postpartum hemorrhage)

peripheral - ovaries

hypergonadrotrophic - FSH is increased

• ovarian agenesis/dysgenesis
  • do karyotype to look for Turner's (XO); could be mosaic

secondary amenorrhea (or primary)

• premature ovarian failure
  • 1-3% of women under 40 y
  • idiopathic number 1
  • genetic: Turner's mosaic, enzyme deficiencies
  • autoimmune (thyroid, SLE, diabetes)
  • ovarian damamge (cheomtherapy, radiation, surgical)
  • infections: mumps
  • potential toxins, such as smoking (smokers hit menopause on avg 1-2 years earlier)
• polycystic ovarian syndrome: LH/FSH ratio >2

outflow tract - (uterus, cervix, vagina)

eugonadotrophic: LH/FSH is normal

• imperforate hymen
• transverse septum in the vagina
• agenesis/dysgenesis of cervix leading to stenosis

agenesis of vagina and uterus

• Mullerian agenesis (Mayer-Rokitansky)
  • mullerian duct agenesis
  • 1/4,000= 1/10,000
  • renal and skeletal anomalies
  • normal ovaries
  • treat with vaginal dilatation or vaginoplasty
• AIS: androgen insensitivity syndrome (formerly testicular feminization)
  • X-linked recessive
  • 1/13,000 births
  • no ovaries
  • undescended testes: can become malignant
  • treat with gonadectomy, estrogen replacement, vaginal dilatation

secondary amenorrhea

• uterus: synechiae (Asherman's syndrome: adhesions from repeated D and C)

History

Primary amenorrhea: no periods by age 14 in absence of secondary characteristics, or no menses by age 16 with secondary sexual characteristics

Secondary amenorrhea: no periods for over 6 months, or over 3 cycles, after menarche

Questions will depend on possible cause:

hypothalamic or pituitary: galactorrhea, headache, visual changes

ovarian: menopausal symptoms

endocrine: weight change, signs of virilization, hypothyroid/hyperthyroid symptoms

medical history

• puberty (absent - Turner's syndrome)

family history

• delayed puberty

social history

• excesrise and diet patterns

Past menstrual history

Age, sexual activity, medical history,

• birth/development history
• pubertal changes
• symptoms: cyclical abdominal pain
• hyperprolacetemia: HA/visual disturbance, galactorrhea
• hypothyroidism (cold intolerance, dry hair, constipation, weight gain, fatigue, etc)
• PMH: chronic disease, operations, chemotherapy
• medications
• lifestyle: exercise, stress, diet, drugs, sexual activity
• family history

Physical Exam

overall appearance

• height, weight, plot BMI (anorexia)
• vital signs
• evidence of male hormones (skin: hirsuitism, acne)

thyroid exam

neuro: vision, fundus, olfaction (pituitary dysfunction)

Tanner staging

abdominal: masses, tenderness

external genitalia

• clitoromegaly, imperforate hymen, vaginal septum, vaginal agenesis

pelvic exam : tough if they are not sexually active. ultrasound instead?

Lab Investigations

Labs will should be based on history and physical findings.

• beta-hCG
• CBC: overall health
• FSH and LH, E2, TSH, PRL
• estrogen, testosterone, DHEA
• bone age
• karyotype if primary amenorrhea
• progesterone challenge to assess for withdrawal bleeding (suggests problem with ovulation)
• prolactin

With secondary amenorrhea, always begin with beta-hCG to rule our pregnancy.

Diagnostic Imaging

Ultrasound to assess anatomy and for PCOS

CT or MRI of head if FSH/LH are low