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Amenorrhea

Definition

Amenorrhea is defined as absence or cessation of menses

Types : Physiological - prepubertal
Pregnancy/lactation
Menopause
Pathological - Primary
Secondary


PRIMARY AMENORRHEA is defined as absence of menses by 16 years of age in the presence of secondary sexual characteristics
or by 14 years in the absence of secondary sexual characteristics
Incidence 1-2% of women
SECONDARY AMENORRHEA is defined as the absence or cessation of menses for 6 months in a women who had previously menstruated

Normal menses
  • XX chromosome
  • intact functioning hypothalamo –pituitary axis
  • functioning ovary
  • responsive endometrium
  • patent outflow tract

At puberty there are a series of changes where there is physical growth-breast development-pubic & axillary hair growth- dev of ovaries and genital organs- growth spurt and menstruation.
Average age of menarche in Indo-Pakistani girls 13.5 yrs

Causes

OUTFLOW TRACT
intact hymen
vaginal agenesis
vaginal septa
cervical agenesis
cervical os stenosis

Cryptomenorrhea
  • primary amenorrhea in a teenage girl
  • cyclic pain abdomen
  • palpable lower abdominal mass
  • possible urinary difficulty
  • Bluish bulging membrane at lower end of vagina


Uterine

Mullerian agenesis/dysgenesis:
Rokitansky Syndrome
Androgen insensitivity (XY)

Asherman Syndrome secondary to curretage /infections like TB
damaged by RT

removed surgicaly
Congenitaly absent endometrium (v rare)

Rokitansky syndrome
  • 46XX geneticaly female
  • Mullerian agenesis(absent uterus or small rudimentary bulb with blind vagina)
  • phenotypically female
  • normal ovaries
  • Presents with primary amenorrhea
  • associated with other cong anomalies :urinary 47%, skeletal 12%,heart disease

Testicular feminization syndrome
  • Genotype male 46XY
  • Absence of cytosol androgen receptors
  • phenotypicaly female : tall with long hands and feet
  • breasts developed : peripheral conversion of testerone
  • pubic and axillary hair not well developed
  • blind vagina
  • testes are in abdomen or inguinal region

Ovarian

Agenesis /dysgenesis
Turners Syndrome (XO),mosaics
Pure gonadal dysgenesis
partial deletion of X chromosome(46xx)

exposure to RT /chemo (destroys the follicles)
surgical removal
premature ovarian failure ? Autoimmune cause
Resistant Ovary Syndrome
Polycystic ovarian disease
galactosemia : galactose has toxic effect on ovarian follicles

Turners Syndrome
  • gonadal dysgenesis 45XO
  • primary amenorrhea
  • short stature
  • absence of Sec. sexual characteristics
  • streak ovaries
  • webbed neck
  • increased carrying angle
  • shield chest
  • associated with heart and renal defects
  • Growth hormone before epiphysis close; estrogen replacement therapy later



Pituitary

Tumours : non functioning adenomas
hormone producing eg prolactinomas , hyperthyroidism etc
Empty sella syndrome :hypo pituitary function
Destruction of pituitary by
necrosis : Sheehans syndrome
infarction
infiltrative lesion eg lymphocytic hypophysitis ,
granulomatous lesions
RT or surgery

Hyperprolactinaemia
  • Due to drugs: haloperidol ,metochlorpramide ,phenothiazine, reserpine, methyldopa : decrease dopamine which inhibits prolactin
  • adenomas (micro and macro)
  • Hypothyroidism : Increased TRH stimulates PL release
  • present with galactorrhea, anovulation, oligo and amenorrhea (20%), subfertility
  • CT/MRI

Hypothalamus (decrease secretion or synthesis of GnRH)
  • physiological delay (most common)
  • Kallmanns Syndrome : insufficient secretion of GnRH
  • Tumours : craniopharyngioma ,tubercular granuloma, dermoid etc
  • anorexia nervosa, extreme exercise ,stress
  • drugs : phenothiazine,reserpine,ganglion blocking agents affect hypothalamus

Congenital adrenal hyperplasia
  • congenital defect in enzymes needed for formation of cortisol – increased ACTH production
  • increased androgen : causing ambigous genitalia, amenorrhea, virilism
  • normal looking uterus and ovaries
  • can be XY or XX
  • serum levels of 17 OH progesterone levels are raised
  • supplement small doses of corticosteroid to suppress the ACTH
  • function of ovaries and uterus possible with eraly treatment

Diagnosis
Clinical history
Examination
Investigation


History

Age
detailed menstrual history
secondary sexual development
associated symptoms : pain
mass
pregnancy related symptoms
headache, visual disturbances
discharge from breast
menopausal symptoms
diet ,recent change in weight or voice

Parity , recent birth related haemorrhage,need for curretage for abortion or PPH
contraceptive history : post pill or depo related amenorrhea
Past medical : TB,DM(vasculitis can damage pituitary), psychological disorders with medications, Cushings syndrome, adrenal disorders,thyroid disorders ,cancers needing RT/Chemo
Past Surgical history : oopherectomy or hysterectomy, D/C , cranial surgeries etc

Personal : use of any drugs, exercise
Family history : mother and sisters age of onset of menses

Examination

General habitus : cachexic ,obese, truncal obesity, enlarged facial features and hands and feet
height and weight

Limbs : cubitus valgus
Secondary sexual characteristics ; breast , pubic and axillary hair
Neck : short webbed neck, LN, thyroid
Chest : shield chest ,widely spaced nipples, milky discharge from breast

PA : mass perabdominal or in inguinal region

PV: ambiguos genitalia, labial fusion, absent or short vagina, imperforate hymen(bluish bulge at introitus), absence of uterus, palpable ovarian mass

PR : confirm mass anteriorly or absence of uterus/vagina . Finger can easily feel a metal catheter in the urethra in case of vaginal agenesis ,but not in case of crptomenorrhoea

Investigation

From the examination outflow tract obstruction is evident .eg agenesis of vagina,uterus
crytomenorrhoea,haematometra
In cases of vaginal agenesis the presence of uterus and ovaries need to be confirmed .
USG - uterus ovaries presence,size
collection within uterus
collection within vagina
pregnancy
PCOD
endometrial thickness

Uterus
absent present
Mullerian agenesis vaginal agenesis
True hermaphrodite septa
cryptomenorrhea
Androgen insenstivity Cervical stenosis
Ashermans Syndrome

Karyotyping ,especially in case of ambigous genitalia

No anatomical defect, pregnancy ruled out , uterus present

Serum Prolactin functioning uterus
Serum TSH estrogenized or not

Progestin Challenge Test
5mg MPA daily for 5 days –withdrawl bleeding within a week
POSITIVE NEGATIVE
bleeding present No bleeding
Endogenous estrogen present No estrogen production

Estrogenized endometrium estrogen/progesterone
Patent outflow supplementation
No bleeding withdrawl
PCOD endometrial damage
positive withdrawl
Serum FSH,LH, PL,TSH
* Low FSH (<40iu/L) constituitional delay
Low LH (<5iu/L) Hypothalamic causes
(hypoGn,hypo gonadism)
* High FSH (>40iu/L) ovarian failure
(hyper Gn ,hypogonadism)
* PL increased hyperprolactinaemia
* TSH increased hypothyroidism

Management

Based on the cause
outflow tract obstruction :
cryptomenorrhea : hymenectomy using cruciate incision
septa : excision
haematometra : cervical dilation/drainage
vaginal agenesis : vaginoplasty





Mullerian agenesis : counseling, adoption

Ashermans syndrome : hysteroscopic breakdown of adhesions ,IUCD kept for 10-12 months to deter reformation

Ovarian dysgensis or failure : HRT supplementation in case of Y chromosome testes is to be removed,as there is 50% chance of malignancy arising from such a gonad
PCOD : medicaly ovulation induction, progesterone supplementation
Testicular feminization : treat patient as female, remove gonads , estrogen suplementation

Hyperprolactinaemia :
lateral skull Xray : enlargement of pit fossa, destruction of clinoid process - CT scan
medicaly : bromocryptine /cabergoline (D agonist)
Surgicaly : macroadenomas with pressure symptoms

Importance of amenorrhea
  • psychosocial development delays, physical sexual abnormalities
  • ? Problems with identity : ?male? Female
  • coital difficulty
  • Pain /Mass
  • infertility
  • risk of malignancy in XY cases: 50%
  • hypoestrogenic status : risk of osteoporosis
  • Hyperestrogenic status : risk of endometrial cancer