Primary Amenorrhea Definition
-no menses by age 13 in the absence of growth or development of secondary
sexual characteristics
OR
-no menses by the age of 15 regardless of the presence of normal growth and
secondary sexual characteristics
Amenorrhea Risks
-earliest sign of a decline in general health
-signal an underlying condition such as hypothyroidism or genetic
abnormality
-osteoporosis
-in genetic: risk of gonadal tumor
Primary Amenorrhea Etiology
-usually result of genetic or anatomic abnormality but virtually all causes of
secondary can cause primary
-chromosomal abnormalities: gondola dysgenesis (ovarian insufficiency due
to premature depletion of all oocytes and follicles)
-absence of necessary pelvic structure (mullerian agenesis)
-physiologic delay of puberty
-PCOS
-isolated GnRH deficiency
-transverse vaginal septum/imperforate hymen
-weight loss/anorexia
-pituitary disease
Amenorrhea Work Up
-rule out pregnancy
-TSH/FSH/prolactin levels
-imaging: pelvic US
-progestin challenge test
-if prolactin is elevated: imaging to evaluate for prolactinoma (MRI)
HYPOGONADOTROPHY (disorders of the anterior pituitary, prolactin
secreting adenoma)
,2
Secondary Amenorrhea Definition
-previously had menses
AND
-absence of menses more than 3 months in girls or women who previously had
regular menstrual cycles
OR
-six months in girls or women who had irregular menses
Maximal Number of Eggs At
-16 to 20 weeks
-6 to 7 million
HPA Axis Overview
-hypothalamus -> GnRH -> anterior pituitary -> LH/FSH -> ovaries ->
progesterone and estrogen -> hypothalamus
Secondary Amenorrhea Etiology
-pregnancy is MC
-pathologically causative systems: ovary, hypothalamus, pituitary, uterus
Progestin Challenge Test
-assess the level of endogenous estrogen and the competence of the outflow tract
-parenteral progesterone oil, oral micronized progesterone, oral
medroxyproesterone acetate
-within 2-7 days: does vaginal bleeding occur?
-yes -> diagnosis is anovulation; intact outflow tract; estrogen present = means
minimal function of the ovary, pituitary, and CNS
-no -> suggests hypogonadism (move to estrogen/progesterone challenge)
Anvoluatory Amenorrhea Treatment
-require treatment (if untreated unopposed estrogen can lead to endometrial
hyperplasia)
-provera for 10 days of each month
-can use OCPs if contraception is also desired
Estrogen and Progesterone Test (After Progestin Challenge)
-give estrogen and progesterone: conjugated estrogen, estradiol,
medroxygprogesterone
-if bleeding does not occur: endometrium or outflow tract problem (rare):
aggressive curettage (Asherman's syndrome), infection, genetic anomaly
-if withdrawal bleeding occurs: outflow tract and endometrium are working,
there is a problem with the body's ability to stimulate estrogen production
(move to gonadotropin assay)
Gonadotropin Assay (After Estrogen/Progesterone Test)
, 3
-if bleeding occurred with estrogen and progesterone together, there is a
problem with stimulation of estrogen production
-follicular activity or gonatotropins
-bioassay the level of gonatotropins
-must do this 2 weeks after the E/P challenge
-draw LH/FSH
-normal: FSH 5-20 with ovulatory midcycle peak about 2x the base level,
LH 5-20 with the ovulatory midcycle peak about 3x the base level
-hypergonadotropic: FSH > 20, LH > 40
-hypogonadotropic: FSH < 5, LH < 5
Hypergonadotropic Etiologies
-bilateral oophorectomy (surgical menopause)
-postmenopausal
-ovarian failure
-ectopic gonadotropin secreting tumors outside the reproductive tract
-gonadotropin secreting pituitary adenomas (associated with HA and vision
changes)
-perimenopausal period (rising FSH level)
-resistant or insensitive ovary syndrome
-autoimmune disease: developing follicles are surrounded by nests of
lymphocytes and plasma cells; also evaluate for abnormal thyroid and
adrenal function
-galactosemia: autosomal recessive; disorder of galactose metabolism due to
an enzyme deficiency; premature ovarian failure
-17-hydroxylase deficiency: absent secondary sexual development, HTN,
hypokalemia, high progesterone levels
Resistant or Insensitive Ovary Syndrome Overview
-hypergonadotropic
-amenorrhea, normal growth and development, elevated gonadotropins
-ovarian follicles are unresponsive to stimulation
-absent or defective gonadotropin receptors on the follicles
-diagnosis: laparotomy with full thickness ovarian biopsy
Hypogonadotropic Etiologies
-prepubertal
-hypothalamic dysfunction
-pituitary dysfunction
Hypogonadotropic Imaging the Sella Turcica
-start with imaging of sella turicica and PRL level (MRI pituitary specific
imaging)
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