AMENORRHOEA
Alaa AL-Naser Assisted Professor of Obesteric. Gynae Thiqar medical college Clinical teacher Continuous medical educationobjectives
To know definition of amenorrhea(primary, secondary)& oligomenorrhea Know different cause of amenorrhea/ oligomenorhea How to diagnose Early ovarian insufficiency Able to approach& arrange suitable ix. Principle Mx.of a case of amenorrhea& oligomenorrhea & fertility treatment.PRIMARY AMENORRHOEA No menstruation by the age of 14 in the absence sec. sexual characteristics. by age of 16 when growth and sexual development are normal. SECONDARY AMENORRHOEA Secondary absence of menses for six months (or greater than 3 times the previous cycle interval) in a women who has menstruated before. Pregnancy, lactation or hysterectomy must be excluded Prepubertal and post-menopausal conditions are also to be excluded as physiological causes Oligomenorrhea defined as infrequent menstruation (duration between period more than 35 days.
CLINICAL APPROACH
There is a difference of opinion about the age at which Primary Amenorrhoea should be investigated 18 yrs. often suggested.Provided the patient has developed normal sec. sex. Characteristics and cryptomenorrhoea has been excluded.While those patient with Primary amenorrhoea and sexual infantilism should be investigated at age of 15 years or 16 years (may be earlier).Accurate, adequate history is essential to reach a firm diagnosisSpecific questioning is necessary to establish diagnosis of Primary or Secondary amenorrhoeaIs the amenorrhoea is truly secondary (e.g. prev. menses were actucally steroid – induced)Careful physical examination aids in reaching a fairly firm provisional diagnosisIn minority, there is a need to go beyond simple out-patient investigation.
CAUSES OF AMENORRHOEA amenorrhea is symptom not a final diagnosis, normal mense require coordinated interaction (hypoth,pitutary,ovaries,outflow)
A. Disorder of outflow tract and or uterus B. Disorders of ovary C. Disorders of Ant. Pituitary D. Disorders of Hypothalamus
A. DISORDERS OF OUTFLOW TRACT & OR UTERUS (normal gonadotrophin
Congenital : congenital abscnce of uterus(faulty in develop. Of Mullarian ducts (Meyer-Rokitansky-Kauser syndrome), also associated with abnormalities in the urinary tract, imporferated hymen, transverse vaginal septum. Trauma: surgical: hystrectomy, over curretage Inflamation:postpartum, postabortive infect.(surgical, inflamation= IU adhesion)A. DISORDERS OF OUTFLOW TRACT & OR UTERUS (normal gonadotrophin)
1. CRYPTOMENORRHOEAVaginal atresia or imperforate hymen prevent menstrual loss from escaping.FEATURES: Prim. Amenorrhoea in a teenage girl with normal sexual development present Complaining of: i. Intermittent lower abd. pain ii. Possible difficulty of mict. iii. Palpable lower abd. swelling (Haematometra) iv. Bulging, bluish membrane at lower end of vagina (Haematocolpus).MANAGEMENT:INCISE MEMBRANE2. ABSENCE OR HYPOPLASIA OF VAGINA:FEATURES: Growth, develop, and ovarian function are usually normal.Uterus may be normal or rudimentaryRenal anomalies (in 30%) or skeletal defects (in 10%) may be present.MANAGEMENT:Create a functional vagina by surgery or dilators
3.TESTICULAR FEMINIZATION:(Androgen Insensitivity) Phenotype is woman. Genotype is man (xy) testes are present. Inherited by an X-linked recessive gene… (familial) Resulting in absence of cytosol androgen receptor
FEATURES:Growth and develop are normal (may be taller than average).Breasts are large but with sparse glandular tissue and pale areolaInguinal hernia in 50% of casesScanty, or no axillary and pubic hairLabia minora underdevelopedBlind vagina, absent uterus, rudimentary fallopian tubesTestes in abd. or inguinal canalNormal levels of testosterone are produced.. But no response to androgens (endog. or exogen)No spermatogenesisThere is incidence of testicular neoplasia (50%)
CONSIDER THE DIAGNOSIS IN A FEMALE CHILD:With inguinal herniaWith 10 amenorrhoea and absent uterusWhen body hair is absentMANAGEMENT:These patients are female.The gonads must be removed after puberty then HRT started Rare cases of incomplete test. feminization do occur have variable degress of masculinization
4. ASHERMAN’S SYNDROME:Sec. amenorrhoea following distruction of the endomet. by over curettage multiple Synechiae show up on “Hysterography”.MANAGEMENT:Under G.A. breakdown intrauterine Adhesions through hysteroscopeinsert an IUCD to deter reformation hormone therapy (E2 + P)5.INFECTIONe.g. Tuberculosis. Ut. Schistosomiasis
DISORDERS OF THE OVARIES(normal or raised gonadotrophins)
Congenital (Turner syndromeXO, mosiac Turner syndrome, pure or mixed gonadal dysgenesis, resistant ovary syndrome. Trauma: radiotherapy, chemotherapy & surgical removal. Inflamation : severe genital TB= ovarian damage. Neoplasic : benign or malignant ov. Tumor PCOS Early ovarian insufficiency(EOI)B. DISORDERS OF THE OVARIES
1. CHROMOSOMAL ABNORMALITIES Turner’s syndrome (45 x 0) gonadal dysgenesisFEATURES:i. Amenorrhoea (10, rarely 20)ii. Short statureiii. Failure of sec. sex. Developiv. Webbing of the neckv. carrying anglevi. Shield chest vii. Coartution of aortaviii. Renal collecting syst. defects
Streak ovaries presentGonadotrophins Estrgoens Mosaic Chrom. Pattern(e.g. XO/XX) lead to various degrees of gonadal dysgenesis and sec. amen. + premature menopause If Y-Chrom is present in the genotype risk of gonadal malig. makes gonadectomy advisable
2. GONADAL AGENESIS:(Failure of gonadal develop): no other cong. abn.3. RESISTANT OVARY SYNDROME A rare condition, due to defect in gonadotrophin receptor. Normal ovarian develop and potential, no autoimmune ds. FSH It may resolve spontaneously If hot flushes Rx. With estrogen
Early ovarian insufficiency(EOI): Idiopathic 75-90% Autoimmune ds. chromosomal (mosaic Turner, galactosaemia, carrier of fragile X permutation. Cessation of ovarian function before 40, EOI should search of chromosomal abnormality. With presence of Y chromosome. Because high risk of malignancy. 5. PCOs: THIS IS DISCUSED IN MORE DETAIL
DISORDERS OF PITUITARY (normal or low gonadotrophin)
Congenital: mutation of B-subunit of FSH Trauma : surg. Removal of pitutary Neoplasm: microadenoma producing PL, macroadenoma, extra-pitutary tumor compress pitutary stalkleading to hyper prolactnaemia. Sheehan syndromeC. DISORDERS OF PITUITARY normal or low gonadotrophin
1. Pituitary Tumor causing “Hyperprolactinemia” 40% of women with hyperprolactinemia will have a pituitary adenomaPit. Fossa XR is necessary in cases of amenorrhoea – particular 20.FEATURES:In coned view: Erosion of clinoid process Enlarge of pituitary fossa Double flooring of fossaIf any of above features seenCT san or MRI + Assessment of visual fieldsMANAGEMENT:Bromocriptine (Dopamine agonist) Suppres prolactin sec. Correct estrogen deficiency Permits ovulation Size of most prolactinomasSurgical removal of tumor if extracellar manifestation (e.g. press. on optic chiasma) or if patient cannot tolerate or respond to medical Rx.
2. OTHER CAUSE OF PROLACT.♣ Drugs: e.g. phenothiazines, methyl-dopa, metclopramide, anti-histamines, oestrogens and morphine.3.CRANIOPHARYNGIOMA ♣Other intracranial tumor4.SHEEHAN’S SYNDROME ♣ Necrosis of ant. pituitary due to severe PPH, 1st hormone affected gonadotrophin & GH then ACTH& finaly TSH. Pan – or partial hypopituitarism ♣ It is rare problem today due to better obstetric care and adequate blood transfusion
D. DISORDERS OF HYPOTHALAMUS (normal or low gonadotrophins)
Congenital: Kallman syndrome Trauma : head injury, sever emotional trauma Inflamation : encephalitis, maningitis Neoplasic : hpothalamic & other brain tumor Iatragenic : chemotherap, radiotherapy for malgnant or autoimmune ds. Weight related
D. DISORDERS OF HYPOTHALAMUS
♣ Commonest reason for hypogonadotrophic sec. amenorrhoea♣ Often associated with stress e.g. in migrants, young women when leave home, university students♣Diagnosis by exclusion of pituitary lesions.♣Hormone therapy or ovulation induction is not indicated unless patient wishes to become pregnant1.WEIGHT – LOSS ASSOCIATED AMENORRHOEAA loss of > 10 kg is frequently associated with amenorrhoea In young women and teen ages girls become obsessed with their body image and starve themselves.This is seen frequently in women training for marathon racing, in ballet dancers and other form of athletes.Leptin one of adipokines( a range of protins secreted by white adiposte ts, has stimul. Signal on hypothalamus, pitutary ovarian axis)CAUSES: + redistribution between proportion of body fat mass and body muscle mass. May be also mediated by exercise related changes in -endorphins ANOREXIA NERVOSA Associated with sec. amenorrhoea .
2.AMENORRHOEA AND ANOSMIA:amenorrhoea of hypogonadotrophic – hypo-gonadism.( Kallman’s syndrome)= congenital absence of GnRH secretion from arcuate nucleus, olfactory area.POST-PILL AMENORRHOEA:♣ There is no evidence that Est. prog. Contraceptive pills predispose to amenorrhoea.. once pill taking is ceased.♣ many cases of hyperprolactinemia will be missed (1:5)♣EOI will be missed in 1:10 cases♣Once other causes are excluded, this type of ameno. Responds well to ovulation induction with Clomiphene citrate if pregnancy is desired.
INVESTIGATION OF AMENORRHOEA
Clinical exam. 1ry amenorrhea (physical k.k of Turner, out flow obstruction, secndary sex. K.k, according to result KARYOTYPING, GONADOTROPHINS MEASUREMENT).Exclusion of pregnancyS. Prolactin level and TFTProgesterone challenge test….to check endog. estrogen.e.g. Provera (medroxy-prog) if bleeding PV=reactive endom. and patent outflow tract.With drawl bleeding= anovulation Negtive with drwal=need addition of E to P =positive with drwal(ovarian hypergonadotrophic hypoganadism, or central hypogonodatrophic hypogonadism) Negative with drwal E,P= outflow cause( hystroscopy asherman ,US or MRI out flow lesion or mullarian abnormalities) Pitutary image CT, MRI if PL high