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