AMENORRHOEA

Dr. R . Mbu
Senior Lecturer

DR. R.E. MBU 2006

PLAN

Introduction
Objectives
Causes of primary amenorrhoea
Causes of secondary amenorrhoea
Management
Primary amenorrhoea
Secondary amenorrhoea
DR. R.E. MBU 2006

INTRODUCTION

Amenorrhoea is the absence of

menses
Could be primary ( the absence of
menses by the age of 18 )
Or secondary ( the absence of
menses for six months in a woman
in whom normal menstruation has
been established )
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INTRODUCTION contd

Menarche is between 9 and 16

years in Cameroon
With an average of 13 years
97.5% of females menstruate
before 18 yrs.
The incidence of primary
amenorrhoea is, therefore, 2.5%
DR. R.E. MBU 2006

CAUSES OF PRIMARY
AMENORRHOEA

Testicular feminization
Pure gonadal dysgenesis
Testicular steriod enzyme defects
Defects in 6( 17-hydroxysteroid
dehydrogenase)
Mullerian dysgenesis
Vaginal agenesis
Transverse vaginal septume
DR. R.E. MBU 2006

CAUSES contd

Imperforate hymen
Hypothalamic defects
Ovarian failure

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CHROMOSOMAL
ABNORMALITIES
1 : TESTICULAR
FEMINIZATION
( 46, xy karyotype )

Because of the y
chromosome
Mullerian inhibiting
factor( MIF) is
secreted
All Mullerian-derived
structures are
absent( uterus,uppe
r 2/3 of the vagina)

DR. R.E. MBU 2006

TESTICULAR FEMINIZATION
contd

They are phenotypic females

But lack the uterus and complete
vagina
They produce some estrogens
Therefore they develop breasts
They are reared like girls
DR. R.E. MBU 2006

CHROMOSOMAL
ABNORMALITIES contd

2 : Turners Syndrome ( 45, xo

caryotype) or Mosaicism ( 45 ,
xo/xx )
The primitive ovary ( oogonia )
undergo rapid atresia
So that there are no oogonia at
puberty

DR. R.E. MBU 2006

PURE GONADAL
DYSGENESIS

The gonads are not well developed

They are rudimentary ( streak )
The streak gonad neither secrets MIF
nor estrogens
They have normal female internal and
external genitalia
No estrogen is produced
They do not develop breasts
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TESTICULAR STEROID ENZYME

DEFECTS

A testis with defective enzymes

The testis produces MIF but no
testosterone
Female external genitalia exist
and no mullerian structures

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DEFEECT IN 6(17HYDROXYSTEROID
DEHYDROGENASE

Ambiguous genitalia
Virilization at puberty

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MULLERIAN
DYSGENESIS

Absence of the uterus

Absence of the 2/3 of the vagina
Affected individuals have a 46,xx
karyotype
The ovaries function very well
Well developed breasts, lower of the
vagina and the vulva
Called ROKITANSKY-CAUSER-HAUSER
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VAGINAL AGENESIS

Failure of the vagina to deveop

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TRANSVERSE VAGINAL
SEPTUM

Failure of fusion of the mullerian

And urogenital sinus-derived
portions of the vagina

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HYPOTHALAMIC
DEFECTS

The arcuate nucleus releases

LHRH into the hypophyseal portal
system hourly
LHRH causes the release of LH
and FSH from the pituitary
LH and FSH stimulate ovarian
follivular growth and ovulation
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HYPOTHALAMIC
DEFECTS contd

1 : Defects of LHRH transport

2 : Defects of LHRH pulse
production
3 : Congenital absence of LHRH

( KALLMANNS SYNDROME)

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OVARIAN FAILURE

PRIMARY OVARIAN
FAILURE; :
Elevated
gonadotropins
and low estradiol(
Hypergonadotropi
c hypogonadism)

SECONDARY
OVARIAN FAILURE
Normal or low
gonadotropins
Low estradiol
( hypogonadotrop
ic
hypogonadism )

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OTHER OVARIAN
FAILURES

Steroid enzyme
defects
Ovarian
dysgenesis

Ovarian
resistance( SAVAGE
S SYNDROME)
Defects in the LH
and FSH receptors
in the ovaries
Ovaries contain
primordial germ
cells
Elevated LH and
FSH levels

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CAUSES OF SECONDARY
AMENORRHOEA

Hypothalamic defects as in primary

amenorrhoea
Ovarian failure
Steroid enzyme defects
Ovarian resistance (SAVAGES
SYNDROME)
ASHERMANS SYNDROME( synechia)
Acquired gynatresia
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CAUSES contd

Sheehans syndrome
Post partum pituitary necrosis
Secondary to PPH

Polycystic ovarian disease( PCOD)

STEIN-LEVENTHAL SYNDROME
Multiple small follicles , thick capsule

Obesity
Ovarian tumoours
Adrenal tumours
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WORKUP FOR PATIENTS WITH

PRIMARY AMENORRHOEA

No uterus
Karyotype
( testicular feminization, mullerian
agenesis ,steroid enzyme defects , pure
gonadal dysgenesis)
Uterus present but no patent vagina:
Imperforate hymen
Transverse vaginal septume
Vaginal agenesis
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WORKUP contd

FSH is over 40 mIU/ml( gonadal

failure)
FSH under 40 mIU/ml ( severe
hypothalamic dysfunction)

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WORK UP FOR PATIENTS WITH

SECONDARY AMENORRHOEA

Progestin challenge:
Positive and patient hirsute ( PCOD ,
ovarian or adrenal tumours)
Positive and patient not nonhirsute ( mild
hypothalamic dysfunction
Progestin challenge negative
Rule out Ashermans syndrome
If FSH is over 40 mIU/ml( gonadal failure)
FSH under 40mIU/ml ( severe
hypothalamic dysfunction)
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PATIENTS WITH AMENORRHOEAGALACTORRHOEAHYPERPROLACTINAEMIA

TSH elevated ( treat

hypothyroidism)
TSH normal, con view normal PRL
under 50-100ng/ml ( repeat PRL
every six months and cone view 12 yrs.
TSH normal , PRL over 50100ng/ml ( microadenoma)
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TREATMENT OF PRIMARY
AMENORRHOEA

Depends on the etiology

Nothing is done for ( testicular
feminization, pure gonadal dysgenesis,
mullerian dysgenesis, vaginal
dysgenesis)
Transverse virginal septume could be
excised
Stimulate for:
Hypothalamic defects
Ovarian failure
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TREATMENT FOR SECONDARY

AMENORRHOEA

Breakdown of intra-uterine synechia for

Ashermans syndrome
Surgical repair for acquired gynatresia , ovarian
and adrenal tumours
Bromocriptine for hyperprolactinaemia
Stimulate for:
Hypothalamic defects
Ovarian failure
Ovarian resistance
Stein-leventhal syndrome
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