EVALUATION AND MANAGEMENT OF

AMENORRHEA

Mazen Freij, MBBS MRCOG

Assistant Professor at JUH

EVALUATION AND MANAGEMENT OF

AMENORRHEA

Mazen Freij, MBBS MRCOG

Assistant Professor at JUH

Objectives

Know the definition of Amenorrhea and Oligomenorrhea

Understand the endocrine, genetic and anatomical basis for these disorders

Definitions

Primary amenorrhea

No menses by age 14, absence of 2º sexual characteristics.

No menses by age 16 , presence of 2º sexual characteristics.

Secondary amenorrhea

No menses for 3 months if previous menses were

regular.

No menses for 6 months if previous menses were irregular

Oligomenorrhea

Interval of more than 35 days between periods

Neural control Chemical control

Dopamine (-)

Norepiniphrine (+)

Endorphines (-)

Hypothalamus

Gn-RH

Ant. pituitary

FSH, LH

Ovaries

Uterus

ProgesteroneEstrogen

Menses

–± ?

AMENORRHOEA AN APPROACH FOR DIAGNOSIS

• HISTORY• PHYSICAL EXAMINATION• BLOOD TESTS• ULTRASOUND EXAMINATION

Exclude PregnancyExclude Cryptomenorrhea

Cryptomenorrhea

Outflow obstruction to menstrual blood

- Imperforate hymen- Transverse Vaginal septum with functioning

uterus- Isolated Vaginal agenesis with functioning

uterus

- Isolated Cervical agenesis with functioning uterus

Imperforated hymen

Amenorrhea and no breast development.

FSH Serum level

Low / normal

High

Hypogonadotropichypogonadim

Gonadal dysgenesis

Amenorrhea and normal breast development.

- FSH, LH, Prolactin, TSH- Provera 10 mg PO daily x 5 days

+ Bleeding No bleeing Prolactin TSH

FurtherWork-up(Endocrinologist)

- Mild hypothalamic dysfunction - PCO (LH/FSH) Review FSH result

And history (next slide)

Amenorrhea Utero-vaginal absence

Karyotype

46-XX

Rokitansky syndrome)

Andogen Insenitivity

(TSF syndrome)

46-XY

Normal breasts& sexual hair

Normal breasts& absent sexual

hair

Amenorrhea

PRIMARY AMENORRHEA

. Ovarian failure

. Hypogonadotrophic Hypogonadism.. PCOS . Congenital lesions (other than dysgenesis) . Hypopituitarism

Hyperprolactinaemia . Weight related

SECONDARY AMENORRHEA

. Polycystic ovary syndrome

. Premature ovarian failure

. Weight related amenorrhoea

. Hyperprolactinaemia

. Exercise related amenorrhoea

. Hypopituitarism

Classic turner’s syndrome (45XO) - Turner variants (45XO/46XX),(46X-abnormal X) - Mixed gonadal dygenesis (45XO/46XY)

Gonadal dysgeneis

• Sexual infantilism and short stature.• Associated abnormalities, webbed

neck,coarctation of the aorta,high-arched pallate, cubitus valgus, broad shield-like chest with wildely spaced nipples, low hairline on the neck, short metacarpal bones and renal anomalies.

• High FSH and LH levels.• Bilateral streaked gonads.• Karyotype - 80 % 45, X0 - 20% mosaic forms (46XX/45X0)• Treatment: HRT

Turner’s syndrome

Mosaic (46-XX / 45-XO) (Classic 45-XO)

Turner’s syndrome

Ovarian dysgenesis

Hypogonadotrophic Hypogonadism

Normal hight

Normal external and internal genital organs (infantile)

Low FSH and LH 30-40% anosmia (kallmann’s

syndrome)Treat with HRT

Constitutional pubertal delay

• delayed bone age ( X-ray Wrist joint)• Positive family history• Diagnosis by exclusion

and follow up

Weight-related amenorrhoeaAnorexia Nervosa 1o or 2o Amenorrhea is often first sign

A body mass index (BMI) <17 kg/m² menstrual irregularity and amenorrhea

Hypothalamic suppression

Low estradiol risk of osteoporosis

Treatment : body wt. (Psychiatrist referral)

Utero-vaginal Agenisis Mayer-Rokitansky-Kuster-Hauser syndrome

Second most common cause of Primary amenorrhea.

Normal breasts and Sexual Hair Normal looking external female genitalia

Karyotype 46-XX

15-30% renal abnormalities.

Treatment : Vaginal creation (Dilatation VS Vaginoplasty)

Normal breasts but no sexual hair

Normal looking female external genitalia

Absent uterus and upper vagina

Karyotype 46, XY

Male range testosterone level

Treatment : gonadectomy after puberty + HRT

Androgen insensitivityTesticular feminization syndrome

Endocrine causes.

Genetic causes.

Anatomic causes.

Amenorrhea

TEST

A. History of leukemia during infancy B. Short stature C. History of delayed puberty in the family D. All of the above E. None of the above

A 17-year-old girl presents to the clinic for the evaluation of primary amenorrhea.Which would be an important aspect of her clinical history?

FSH of 60 IU/L (normal 0.33–10.54) B. Estradiol of 100 pg/ml (normal 40–410) C. LH of < 0.2 IU/L (normal 0.69–7.15) D. All of the above E. None of the above

After receiving Chemotherapy, which of the following labs results would make you consider the diagnosis of ovarian failure?

A. Turner Syndrome B. CAH C. Rokitansky Syndrome D. Imperforsted Hymen E. PCOS

The commonest cause for primary amenorrhea is

A. Imperforated hymen. B. Turner Syndrome C. Androgen insensitivity. D. Rokitansky syndrome E. Hypogonadotropic hypogonadism

One of the following can be the cause for primary amenorrhea with normal secondary sexual development.

What is the definition of Primary Amenorrhea?

19 year old presented with primary amenorrhea, normal breast development but no pubic hair, absent uterus. The most likely diagnosis is:

A. Rokitansky syndrome B. Turner Syndrome C. Androgen insensitivity D.Hypogonadotropic Hupogonadism

Thank You