| What is amenorrhea? | It is the absence of menses for at least 6 months
2 types, primary (complete absence) and secondary (if it disappears after having at least 1 period per life) |
| How is the physiology of the hypothalamic-hypophyseal axis? | For hypothalamus, there is a pulsatile stable secretion of GnRH every 90 mins throughout the cycle, but difference is in gonadotrophic cells receptors according to the phase we are in (follicular or luteal) and may be influenced by activating factors (VIP and NE) or inhibiting ones (dopa, endorphins, serotonin, GABA)
For pituitary LH and FSH prodocution depends on the hormonal phase we are in (Luteal LH, Follicular FSH)
There are hormones controlling it (Sexual hormones, monamines, and most importantly Prolactin) |
| How is the physiology at ovaries? | During follicular phase estrogen secretion increases, during luteal both estrogen and progesterone are produced by corpus luteum.
There are other steroids (testosterone, delta 4 and 17-OH-P)
These control menstruation, uterine mucosa and modulate hypothalamic-hypophyseal axis. |
| What is menstruation? | It depends on sex steroids, estrogen is essential to get progesterone receptors to endometrial wall, when hormones are less this leads to shed of endometrium and thus menstruation
Permiability of the lower genital tract is also important during menstruation. |
| What are the means of investigation (gonophoric/ sex exploration) ? | Clinical exam of vagina and cervix
Hysterosalpingography/ Hysteroscopy for lesions and malformation exploration (+ synechiae uterus)
Oestro-progestative (contraceptive) test: giving estradiol for 21 days and progesterone from day 8 till 21, if we see bleeding withdrawal, then we have a functional endometrium (It is an artificial cycle) |
| What are the means of investigation of ovaries? | US (volume and structure), morning thermal curve (for luteal phase, look for monophasic amenorrhea)
Endocrine function test (smears and biopsies estimating estrogen impregnation)
Progestative test (duphaston for 10 days then progesterone, if bleeding occurs hen we have normal estrogen else we dont have)
Hormonal measurement (not very useful, use only androgens)
Karyotype (mainly turner (primary amenorrhea) |
| What are the means of investigating hypothalamic-hypophyseal axis? | PRL and gonadotropin levels (increase of PRL in case of adenoma, if PRL increases endorphins increase and thus GnRH decrease), FSH and LH levels (increase in ovarian pathology)
LHRH test (if low LH and FSH, evaluated mobilization of pituitary reserve not very imp)
clomiphene citrate test (anti-estrogen for functional pathology trigger a cycle)
Anatomic exploration (clinical and bio data) |
| What are the etiologies of primary amenorrhea? | There is with or without normal secondary sexual characteristics, sexual ambiguity and hyperandrogenism |
| What are the origins of secondary amenorrhea? | Utero-vaginal, premature ovarian follicule, hypothalamo-pituitary cause, supra-hypothalamic cause, hyperandrogenism) |
| What are the etiologies of primary amenorrhea with secondary sexual char? | First always rule out pregnancy
Then see anatomical malformations (imperforated hymen- pelvic pain, congenital aplasia of vagina- antimullerian duct growth seen by laproscopy/ceoloscopy, Post-TB uterine synechiae seen by hysterography)
Then anorexia nervosa (respond to contraceptives)
Then simple delay of menarche in case we ruled out all causes |
| What are the signals that we look for in 2ary amenorrhea without secondary sexual characteristics? | Evaluate stature-weight curve, bone age, FSH and LH test, karyotype and finally dysmorphia |
| What are the signs of primary amenorrhea without secondary char that are of gonadal origin? | Gonadal dysgenesis (turner syndrome , could be mosaic)
Bone age (normal or slightly delayed)
Dysmorphic syndrome with small size.
Partial or total estrogen deficiency with elevated gonadotropins
Seen with Coelioscopy infantile uterus, fallopian tube underdeveloped, and fibrous stripes at ovaries place (no ovaries) |
| What are the signs of primary amenorrhea without secondary characteristics that are of hypothalamic-hypophysela origin? | LH and FSH low with no response to LHRH
dx by hx (growth, headache, anosmia (Kalmann syndrome)) and exam (radio MRI mostly |
| What lesions cause primary amenorrhea without sexual characteristics? | Rarely pituitary adenoma
Mostly supra-sellar cause (craniopharyngioma -calcification, diencephalic tumors, and hydrocephalus)
Sometimes endocrine abnormalities (pan hypopituitarism with defrientation of hyperprolactinemia, eg: Olfacto-genital kalman syndrome) |
| What is functional amenorrhea in case of primary amenorrhea without secondary char? | diagnosis of elimination may be pubertal delay or psychogenic cause or malnutrition. |
| What is sexual ambiguity? | Male pseudohermaphrodism (testis but feminin)
Usually due to androgen resistance, no sexual pilosity, normal breasts and vulvula , no uterus (AMH) two palpable masses (testicles)
Dx: normal testosterone, LH and FSH, but with XY karyotype.
could be mosaic/ incomplete form |
| What are utero-vaginal causes of secondary amenorrhea? | Acquired anomaly in genital tract preventing menstruation
Characterized by ovulatory type temp curve, normal hormones and spaniomenorrhea with decreased menstrual flow
Caused by uterine synechiae (TB, trauma- myomectomy/ seen in hysteroscopy)
or stenosis of cervix (cancer, electrocoagualation, curettage) |
| What is premature ovarian insuffeciency? | Clinical signs: hot flashes, estrogen deficiency.
Hormonal expression: LH and FSH raised with explosive LHRH, estradiol decreased <30, progestogen test negative
Etiologies (induced early menopause (ovariectomy, chemotherapy, radiotherapy) , spontaneous early menopauses (autoimmune ovaritis- may be associated with other auto immune diseases)) |
| What are the spontaneous early menopauses causing premature ovarian insufficiency? | Gonadal dysgenesis (early ovarian failure (<30 yrs , chromosomal abnormalities))
Viral origin (mumps, rubella, coxsackie)
Gonadotropin-resistance (primordial follicules blocked at antral stage with normal karyotype)
Menopauses of undefined cause |
| What is hypothalamo-pituitary cause of secondary amenorrhea? | Clinical aspect (Isolated amenorrhea or pan pituitary insufficiency)
Biology (Low LH and FSH (irresponsive to LHRH), fallen estradiol (- progestogen test), prolactin increase)
Look for tumor syndrome using imaging
Etiologies (pituitary tumors, craniopharyngiomas, non tumoral origins) |
| What pituitary tumors causing secondary amenorrhea? | adenomas if <1cm micro if more macro
Prolactin adenoma (irresponsive to TRH)
GH adenoma (amenorrhea preceeds acromegaly, dx by IGF-1 level, and HGPO dose of GH)
Corticotropic adenoma (hypercortisol, dx of free cortisol in urine , midnight cortisol level, and dexamethasone decceleration level)
Thyrotropic adenoma (very rare but large)
Gonadotropic adenoma (very rare, very high FSH (not frequently LH)) |
| What is craniopharyngioma causing secondary amenorrhea? | Supra-pituitary tumor mostly at rathkes pouch, often cystic with slow evolution.
Radio: supra sellar calcification
Biology: Gonadal insufficiency with other hypophyseal deficts, hyperprolactinemia |
| What are the non-tumor origins of secondary amenorrhea by hypothalamic axis? | Hemochromatosis, glanuloma (sarcoidosis in pituitary), surgical excision, radiotherapy, empty sella turcica, sheehan syndrome (post-partum necrosis) |
| What are supra-hypothalamic origins of secondary amenorrhea? | Psychogenic, gonadotropic insufficiency due to CNS failure interaction, decrease LHRH frequency due to increase of opiods
Healed by psychogenic management. |
