Amenorrhoea Hisham Saleh

7/28/2019 Amenorrhoea Hisham Saleh

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Compartment I: Disorders ofthe Outflow Tract or Uterus Asherman's Syndrome

Mllerian Anomalies

Mllerian Agenesis

Androgen Insensitivity (TesticularFeminization)

Compartment II: Disordersof the Ovary Turner Syndrome

Mosaicism

XY Gonadal Dysgenesis

Gonadal Agenesis

The Resistant Ovary Syndrome

Premature Ovarian Failure

The Effect of Radiation andChemotherapy

Compartment III: Disordersof the Anterior Pituitary Treatment of Nonfunctioning

Adenomas

Pituitary Prolactin-SecretingAdenomas

The Empty Sella Syndrome

Sheehan's Syndrome

Compartment IV: CentralNervous System Disorders Hypothalamic Amenorrhea

Weight Loss, Anorexia, Bulimia

Exercise and Amenorrhea

Eating Disorders and Pregnancy

Inherited Genetic Defects

Postpill Amenorrhea

Definition of AmenorrheaBasic Principles in Menstrual FunctionEvaluation of AmenorrheaSpecific Disorders Within Compartments

Hormonal Therapy


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Primary

Absence of menses by age 16with normal secondary sexualcharacteristics

Absence of menses by age 14without secondary sexualdevelopment

Secondary

In a previously menstruatingfemale, absence of menses for alength of time equivalent to atotal of at least 3 of the previouscycle intervals or for 6 months


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Thelarche (breast development) Requires estrogen

Pubarche/adrenarche (pubic hair

development) Requires androgens

Menarche Requires:

GnRH from the hypothalamus

FSH and LH from the pituitary

Estrogen and progesterone from the ovaries

Normal outflow tract


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Neural control Chemical control

Dopamine(-)

Norepiniphrine(+)

Endorphines(-)

IV. Hypothalamus

Gn-RH

III.Ant pituitary

FSH, LH

II. Ovaries

I. Uterus

ProgesteroneEstrogen

Menses

?


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Physiological amenorrhea.

Pathological amenorrhea .

a) False (cryptomenorrhea) b) True

Ovarian 40%

Uterine 5%

Pituitary 20%

Hypothalamic 35%


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DefinitionPeriods of life at which the femaledoesn't have menses.

CausesBefore puberty.

Pregnancy (Most common cause of2ry amenorrhoea)

Lactation.

Menopause.


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Hormonalprofile

Etiology

FSH&LH

estrogen & progesterone

Immature

H.P.O

Axis

Before

puberty


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Hormonalprofile

Etiology

1- B.HCG

(pregnancy test)

2- estrogen &

progesterone

3- FS H & LH

Continuous

release of

estrogen &progesterone

without

withdrawal

Pregnancy


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Hormonalprofile

Etiology

1) PRL

2)estrogen&

progesterone

PRL suppress.

Ovarian

function &

suppress

GnRH

Lactation


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Hormonal

profile

Etiology

1) FSH >40 I.U/.L

2) estrogen < 50 pg /ml

Exhaustion

of ovarian

follicles

Menopause


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Definition:

Hidden mestruation; menstruation

occurs regularly but no bloodescapes outside due to outflowobstruction.


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Etiology:

Mainly congenital causes:1-Imperforate hymen (most

common cause)

2-Complete transverse vaginalseptum

3-Vaginal atresia with functioning

uterus4-Cervical atresia

5-Vaginal aplasia


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GalactorrheaPRL

Coned down view

Elevated TSH + Withdrawal

Hypothyroidism Normal PRLNormal TSH

II. Anovulation

PCOD

- Withdrawal

- Withdrawal

I. End organproblem

CyclicEstrogen & Progestin

Exclude pregnancyTSH, PRL

Progestational challenge

+ Withdrawal

Evalution of Amenorrhoea

Step

1

Step

2


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+Withdrawal bleed -Withdrawal bleedI. End organ

problem

Low Normal High

II. Ovarian

failure

III & IV. Pituitary

Hypothalamic

amenorrhea

CyclicEstrogen & Progestin

FSH, LH

Coned down viewCT, MRI

Evalution of Amenorrhoea

Step

2

Step

3

Step

4


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Compartment I Ashermans syndrome 7%

Compartment II Anovulation PCOS Ovarian Failure

abnormal karyotype 0.5%normal karyotype 10%

Compartment III Prolactinoma 7.5%

Compartment IV Anovulation 28% Hypothalamic suppression 10% Weight loss/anorexia 10% Hypothyroidism 1%


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Ashermans Syndrome Mullerian Anomalies

Imperforate hymen (most common) Mullerian agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome) Transverse vaginal septum

Vaginal atresia with functioning uterus Cervical atresia Vaginal aplasia Uterus present but lacking cavity Uterus present but lacking endometrium

Hystrectomy

Endometrial Ablation Uterine irradiation Androngen Insesitivity (Testicular Feminization Syndrome) Incomplete Androgen Insesitivity


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Intrauterine synchia following vigorousendometrial curettage, tuberculous

endometritis, cesarean section ormyomectomy.


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Monthly menstrual bleeding inthe presence of outflowobstruction leading tocollection of menstrual blood .

Vagina becomes distendedwith blood hematocolpos.

Later on uterus becomesdistended with blood

hematometria.

The uterus contract each cycleto overcome the obstructionwhich is the cause of cycliccolicky abdominal pain


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Clinical Picture:

Cases with false amenorrhea are usuallypresented at the age of puberty

Symptoms

-Primary amenorrhea.

-Progressive abdominal pain .

-Cyclic abdominal pain .

-Urine retention due to compression on

bladder and urethra by distended vagina


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Clinical Picture:

Signs

-General examination :

Normal Secondary sexual characters

(breast development, pupic and axillary

hair)-Abdominal examination

Presence of pelviabdominal mass (due todistended vagina and uterus with blood)

which is usually mistaken for pregnancy


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Clinical Picture:

Vaginal examination

Imperforate hymen-Intact hymen and complete

-Bulging bluish membranewith +ve

- impulse on cough-Cross fluctuation in PR

examination

Complete transverse

vaginal septum-Intact hymen, incomplete-Septum beyond level ofhymen

-No impulse on cough


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Investigations:

Pregnancy test

Ultrasound

IVU

to detect associated

congenital anomalies inurinary tract


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Treatment:

Imperforate hymen

Partial hymenectomy:Cruciate incision followed by marsupialization


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15% of primaryamenorrhea

Normal secondarydevelopment & external

female genitalia

Normal female rangetestosterone level

Absent uterus and upper

vagina & normal ovaries Karyotype 46-XX

15-30% renal, skeletal andmiddle ear anomalies


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Previously called testicular feminizationsyndrome

Genetically mediated ( X-linked trait )

This is the third most common cause ofprimary amenorrhea after gonadaldysgenesis and mllerian agenesis

Affected individuals have Karyotype 46, XYand have normal testes (undescended) withnormal production of testosterone andnormal conversion to dihydrotestosterone


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Absent cytoplasmic testosterone receptors

Due to absence of receptors in targetorgans, there is a lack of male

differentiation of the external and internalgenitalia. Therefore, external genitaliaremain female and Wolffian ductdevelopment fails to take place. Also,

Mllerian duct regression is induced by anti-mllerian hormone which is produced by theSertoli cells of the fetal testes.


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Normal female appearance No female or male internal

genitalia Normal female external

genitalia

Short or absent vaginal pouch Scanty or absent pubic/axillary

hair Normal or enhanced breasts

Treatment:

Operative removal of thetesticles after puberty due to risk of malignancy(gonadoblastoma) found in25% of patients

Surgical correction of thevagina (artificial vagina)


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MA AIS

Karyotype 46, XX 46, XY

Heredity Not known Maternal X-linkedrecessive, 25%affected, 25% carrier

Sexual Hair Normal female Absent to sparse

Testosterone level Normal female Normal to slightly

elevated male

Other anomalies Frequent Rare

Gonadal neoplasia Normal incidence 5% incidence ofmalignant tumour


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1. Gonadal Dysgenesis: Chromosomally abnormal

Classic turners syndrome (45XO)

Turner variants (45XO/46XX),(46X-abnormal X) Mixed gonadal dygenesis (45XO/46XY)

Chromosomally normal

46XX (Pure gonadal dysgeneis)

46XY (Swyers syndrome)2. The Resistant Ovary Syndrome3. Premature Ovarian Failure4. Effect of Radiation & Chemotherapy


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1. Gonadal Dysgenesis:

Isaspectrum of disorders with associated

hypergonadotropic Hypogonadism. It ischaracterized by a progressive loss ofprimordial germ cells on the developinggonads of an embryo. This loss leads to

extremely hypoplastic (underdeveloped)and dysfunctioning gonads mainlycomposed of fibrous tissue, hence thename streak gonads.


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1. Gonadal Dysgenesis:


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1. Gonadal Dysgenesis:Characters of ovarian dysgenesis: amenorrhea,

stunted growth, genital atrophy and absence of

secondary sex characters


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Classic

Turners

Turner

Variant

True gonadal

Dysgenesis

Mixed

Dysgenesis

Phenotype Female Female Female Ambiguous

Gonad Streak Streak Streak - Streak- Testes

Height Short - Short

- Normal

Tall Short

Somaticstigmata

Classical Nil

Karyotype XO XX/XO orabnormal X

46-XX(Pure)

46-XY (Swyer)

XO/XY


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Classic (45, XO) Mosaic (46, XX/45, X0)


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The term premature ovarian failure(POF) describes a stop in the normal

functioning of the ovaries in awoman younger than age 40.

This is sometimes calledpremature

menopause, but premature ovarianfailure is actually much differentthan menopause.


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Pituitary Tumors Non functioning adenomas Hormone-secreting adenoma

Prolactinoma Cushings disease Acromegaly Primary hyperthyroidism

Craniopharyngioma Meningioma Glioma

Infarction Surgical or Radiological ablation Sheehans syndrome Diabetic vasculitis


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Prolactin Secreting Adenoma

Galactorrhoea


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Prolactin Secreting Adenoma Most common pituitary tumor 50% identified at autopsy Disruption of the reproductive mechanism S/S increased PRL

Amenorrhea Galactorrhea

For macroadenomas

Headache

Visual field defect

Treatment

Medical : dopamine agonist Surgical for macroadenomas that do not respond to dopamine

agonist


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Sheehans Syndrome: Postpartum hemorrhage

Acute infarction and necrosis

Hypopituitarism= early in the PP period

Failure of lactation

Loss of pubic and axillary hair

Deficiencies : GH, Gonadotropins (FSH,LH),

ACTH, TSH (infrequency)


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Hypothalamic Amenorrhoea

Weight loss, Anorexia Nervosa,

Bulimia. Exercise Induced Amenorrhoea

Inherited Genetic Defects

Kallmanns Syndrome Adrenal Hypoplasia

Postpill Amenorrhoea


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Deficiency in GnRH pulsatile secretion.

Normal height

Normal external and internal genital organs(infantile)

Low FSH and LH

MRI to R/O intra-cranial pathology.

30-40% anosmia (Kallmanns syndrome) Sometimes constitutional delay

Treat according to the cause (HRT),potentially fertile.


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1o or 2o Amenorrhea is often first sign A body mass index (BMI)


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Common in women whoparticipate in sports (e.g.competitive athletes, balletdancers)

Eating disorders have ahigher prevalence in femaleathletes than non-athletes

Hypothalamic disordercaused by abnormalgonadotrophin-releasing

hormone pulsatility, resultingin impaired gonadotrophinlevels, particularly LH, andsubsequently low oestrogenlevels


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Post-pill amenorrhea is not an entity

Depot medroxyprogesterone acetate

Up to 80 % of women will haveamenorrhea after 1 year of use. It isreversible (oestrogen deficiency)

A minority of women taking theprogestogen-only pill may have reversiblelong term amenorrhoea due to completesuppression of ovulation

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Amenorrhoea Hisham Saleh