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Amenorrhea

Dr.Aliya Waheed
Senior Registrar
Department of Obstetrics and Gynaecology
Ziauddin University & Hospitals

Complete absence or cessation of menstrual


bleeding for greater than 6 months (not due
to pregnancy)

Puberty

It is the time when one becomes


functionally able to reproduce.

Physiology of puberty
Onset: 10-16 years
Hypothalamus matures pulsatile release
Gonadotrophin releasing hormone
(GnRH) Pituitary gland FSH & LH
release Ovaries Oestradiol
Secondary sexual characteristics

Signs Of Puberty
Breast development
Pubic & axillary hair
Growth spurt
Onset of menstruation

Physiology of menstruation and


ovulation
GnRH FSH & LH one or more follicles
mature estrogen negative feedback on
pituitary and hypothalamus FSH large LH
and small FSH surgeovulationcollapsed
follicle luteinized secretes progesterone and
estrogentheca lutein cells degenerate
progesterone and estrogen negative feedback
FSH endometrial shed menstruation

Physiological amenorrhea
Before menarche
Pregnancy
Lactation
After menopause

Classification
Primary

Amenorrhea
Secondary Amenorrhea

Primary Amenorrhea

Primary Amenorrhea
Failure of spontaneous onset of menstruation
by the age of 16 years.

Primary Amenorrhea

If menarche delayed beyond 16 years then


it should be investigated

With normal secondary sexual


characteristics
Without secondary sexual characteristics

Secondary sexual characteristics


normal
Imperforate hymen
Transverse vaginal septum
Absent vagina and functioning uterus
Absent vagina and non-functioning uterus
XY female
Resistant ovarian syndrome
Constitutional delay

Secondary sexual characteristics


absent (normal stature)
GnRH deficiency
Weight loss/Anorexia
Excessive exercise
Hyperprolactinaemia
Gonadal agenesis/dysgenesis
Ovarian failure (rare)

Secondary sexual characteristics


absent (short stature)
Congenital or neonatal infections
damaging hypothalamus
Trauma
Empty Sella syndrome
Tumors
Turner syndrome(45XO)

Case 1:
17 years old brought by her mother for
complaints of failure to start menstrual
cycle.
Management?

History:
Withdrawl bleed, cyclical pain, urinary
retention
Past medical and surgical history (cranial,
thyroid, genital tract, chemotherapy,
radiotherapy)
Family history (mother and other sisters)
Personal history: athlete, vigorous exercise

Examination
Height and weight (BMI)
Secondary sexual characteristics, breast and
hair distribution (axillary and pubic)
Thyroid
Systemic examination (perabdominal mass)
Genital tract inspection

Normal secondary sexual


characteristics
N o rm a l s e c o n d a ry s e x u a l c h a ra c te ris ti c s
U ltra s o u n d

U te ru s a b s e n t

U te ru s p re s e n t

K a ry o ty p e

O u t flo w o b s tu c tio n

46XX

46XY

A b s e n t u te ru s a n d v a g in a

X Y fe m a le

N o rm a l a n a to m y

F S H /L H
P ro la c tin

N o rm a l

L H /F S H

F S H /L H

P ro la c tin

H Y p o th a lm ic

PCO

R e s is ta n t o v a ry

P ro la c tin o m a

No secondary sexual
characteristics
N o s e c o n d a r y s e x u a l c h a r a c t e r is t ic s
H e ig h t

N o rm a l

S h o rt

F S H /L H

F S H /L H

Low

H ig h

Low

H ig h

H y p o g o n a d o t r o p h ic
h y p o g o n a d is m

K a ry o ty p e

I n t r a c r a n ia l le s io n

K a r y o t y p in g

* P r e m a t u r e o v a r ia n fa ilu r e
* R e s is t a n t o v a r y
* G o n a d a l a g e n e s is

* X Y a g e n e s is
* X Y e n z y m a t ic fa ilu r e

X O o r v a r ia n t s

Normal secondary sexual characteristics


Imperforate Hymen

Early childhood--bulging hymen (mucocele)


--hymenectomy, no subsequent problem.

At puberty cyclical intermittent abdominal pain,


haematocolpos, haematometra, difficulty with
micturation and defecation.

On exam: abdominal swelling, tense bulging


bluish membrane which is hymen ------cruciate
incision in the hymen allows drainage of retained
menstrual blood.

Normal secondary sexual characteristics


Transverse Vaginal septum
Vagina fails to cannulate, the upper and lower part of the
vagina are separate.
Septum occurs at three levels lower, middle or upper third
septum
Cyclical abdominal pain (haematocolpos)
Palpable abdominal mass
On inspection blind ending vagina, bulging but pink.
Separate hymenal remnants.
No introital swelling and mass on rectal examination
Special reconstruction to create a vagina

Normal secondary sexual characteristics


Absent vagina and functioning uterus
Embriologically the uterine body has
developed normally, but there is failure of
development of the cervix and upper vagina
Cyclical abdominal pain but no pelvic mass
Small haematometra, retrograde
menstruation ------ endometriosis and pelvic
adhesions.

Normal secondary sexual characteristics


Absent vagina and non-functioning uterus
(Meyer-Rokitansky-kuster-Hauser syndrome)
Second most common cause of primary amenorrhoea
(second only to Turner syndrome)
Ovarian function is unaffected
Normal female external genitalia but blind ending vaginal
dimple 1.5 cm in depth and uterine development is usually
very rudimentary.
Small uterine remnants found on the lateral pelvic walls
40% renal anomalies, 15% major e.g. absent kidney

Normal secondary sexual characteristics


XY FEMALE

Female phenotype
Failure of testicular development, enzymatic failure of the
testis to produce androgens (testosterone), and androgen
receptor absence or failure of function.
Androgen insensitivity ---- structural abnormality with
androgen receptors, due to abnormalities of androgen
receptor genes---non-functional receptors.
Peripheral conversion of androgen to estrogen and
subsequent stimulation of breast growth

Scanty pubic hairs


Normal vulva but short vagina
Absent uterus ovaries and fallopian tubes
Testes --- lower abdomen or hernial sac

Normal secondary sexual characteristics


RESISTANT OVARY SYNDROME
Rare condition
Elevated gonadotrophins, appearently
normal ovarian tissues
Absence or malfunction of FSH receptors in
the ovarian follicles and are unable to
respond properly to FSH

Normal secondary sexual characteristics


Constitutional delay
No anatomical anomaly
Normal endocrine levels
Immature pulsatile release of GnRH
Spontaneous menstruation

No secondary sexual characteristics


(normal height)
Isolated GnRH deficiency (Kallman syndrome)
Hypothalamus lacks the ability to produce GnRH
(Hypogonadotrophic state)
Pituitary gland is normal and stimulation with
GnRH leads to normal release of gonnadotrophins
Maldevelopment of neurons in the arcuate nucleus
of hypothalamus (derived embriologically from
olfactory bulb and some have anosmia)

Case 2:
35 years old para 3+0 complaint of absent
menstrual cycles for 6 months.
Diagnosis?
Management?

History

Previous menstrual cycles, L.M.P


Previous obstetrical history
Hot flushes, insomnia
Last delivery (PPH)
Lactation
Weight gain or loss (exercise)
Headache, galactorrhoea, vision
Drug history (contraception, radiotherapy
antidepressants,Chemo)
Surgery (diagnostic D&C)

Examination
General (BMI, thyroid, Galactorrhoea,
hirsuitism, galactorrhoea)
Systemic (mass)
Local examination

Secondary Amenorrhea

Absence of menstruation for at least 6 consecutive


months or longer if the patient has previous
regular periods and for 12 months or more when a
patient has oligomenorrhea.

Classification of Secondary
Amenorrhea
Uterine causes:

Ashermans syndrome
Cervical stenosis

Ovarian causes:

PCOS
Premature ovarian failure (genetic, autoimmune,
infective, radio and chemotherapy)

Hypothalamic causes
(hypogonadotrophic hypogonadism):

Weight loss
Exercise
Chronic illness
Psychological distress
Idiopathic

Pituitary causes

Hyperprolactinaemia
Hypopituitarism (Sheehan syndrome)

Causes of hypothalamic/pituitary damage


(hypogonadism):

Tumors (craniopharyngioma, gliomas


Germinomas)

Cranial irradiations

Head injuries

Tuberculosis

Systemic causes:

Chronic illness
Weight loss
Endocrine disorders
(thyroid disease, cushings disease)

Investigations and treatment


I n v e s t ig a t io n s

P r o la c t in

FSH

H ig h

N o rm a l / L o w

A s s e s s R x ta k e n
C T s c a n o f p it u it a r y fo s s a

A b n o rm a l

N o rm a l

N e u r o lo g ic a l
o p in io n

C heck TS H
le v e l

N o rm a l o r
Low

H ig h

E s tr o g e n le v e l
I f p r e g n a n c y r e q u ir e d , e v a lu a t e p r o g e s t e r o n e
s t im u la t io n t e s t

H ig h

R e fr a c t o r y
o v a ry
b le e d in g + v e

H ig h

N o rm a l

T h y r o x in e

B r o m o c r ip t in e

C lo m ip h e n e

I f fa ilu r e

b le e d in g - v e

F o llit r o p in /
hC G or G nR H

Low

O v a r ia n
fa liu r e

POLYCYSTIC OVARY
SYNDROME (PCO)
Is the commonest endocrine disorder in
women (prevalence15-20%)
PCO runs in families and affects 50% of
first-degree relatives.

POLYCYSTIC OVARY
SYNDROME
Oligo- and/or anovulation
Hyperandrogenism(clinical and/or
biochemical)
Polycystic Ovaries (ovary with 12 or more
follicles measuring 2-9 mmin diameter and
increased volume(>10cm3 )on TVS.

Symptoms:
Hyperandrogenism(acne,hirsutism,alopecia)
Menstrual disturbances
Infertility
Obesity
Asymptomatic with PCO on U/S

Serum Endocrinology
Fasting Insulin
Androgens
Leutinizing hormone,normal FSH
Sex hormone binding globulin(SHBG)
Oestradiol,oestrone
Prolactin

Late Sequele of PCOs


Diabetes Mellitus
Dyslipidaemia
Hypertension,Cardiovascular disease
Endometrial cancer
Breast cancer

Case:3
34 years old female married since 5 yrs
para 0+2,presented in OPD with secondary
amenorrohea.
a.Differential diagnosis.
b.What additional information will you obtain
about her complaint?
c.Briefly outline the investigation you will
undertake on her?

KEY(a)
Wt loss,stress anrexia
Hyperprolactinoma-galactorrhea,headaches
Drugs-steroids,antihypertensive
Feature of hyperandrogenismacne,hirsutism,wt gain voice change
Exclude Ashermans syndrome, infection
Symptoms of thyroid dysfunction
Premature menopause-hot flushes,mood
changes

KEY(b)
Exclude pregnancy
Hormonal profile
U/S of pelvis
C.T of brain to rule out prolactinoma
Hysteroscopy

THANKYOU