Amenorrhea

Professor
Ahmed Abdel Aziz Ismail
 Amenorrhea
Primary
Absence of menses by age 16 with normal
secondary sexual characteristics
Absence of menses by age 14 without secondary
sexual development
Secondary
Absence of menses for 6 months in a previously
menstruating female
 CNS-Hypothalamus-Pituitary
Ovary-uterus Interaction
Neural control Chemical control

Dopamine Norepiniphrine Endorphines

(-) (+) (-)

Hypothalamus
Gn-RH
Ant. pituitary
ACTH FSH, LH TSH
Estrogen Ovaries Progesterone

Uterus

Menses
 Events of Puberty
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
 Primary Amenorrhea
Is there normal development of secondary
sexual characteristcs?
NO
Think hypogonadism or hypogonadotropism
 Amenorrhea with Immature
Secondary Characteristics

FSH Serum level

Low / normal High

Hypogonadotropic Gonadal
hypogonadism dysgenesis
 Primary Amenorrhea with Immature
Sexual Characteristics
Hypogonadism (gonadal failure)
Gonadal dysgenesis
Irradiation
Chemotherapy
Galactosemia
Note: gonadotropins (FSH/LH) will be high,
similar to menopause
 Primary Amenorrhea with Immature
Sexual Characteristics
Hypogonadism (gonadal failure)
Gonadal dysgenesis
Irradiation
Chemotherapy
Galactosemia
Note: gonadotropins (FSH/LH) will be high,
similar to menopause
 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)
 Primary Amenorrhea with Immature
Sexual Characteristics
Hypogonadotropism
Hypothalamic dysfunction
Kallmann syndrome
Anorexia nervosa
Space-occupying lesion of CNS
Marijuana use
Pituitary damage (surgery/radiation)
Constitutional delay
Are there secondary sexual
characteristics?
 Primary Amenorrhea
Think
Pregnancy
Mullerian anomaly
Androgen insensitivity
 Primary Amenorrhea with Normal
Secondary Characteristics
Mullerian Anomalies
Mullerian agenesis (Mayer-Rokitansky-Kuster-
Hauser syndrome)
Imperforate hymen
Transverse vaginal septum
 Mayer-Rokitansky-Kuster-Hauser
Syndrome (utero-vaginal agenesis)
15% of primary amenorrhea
Normal secondary
development & external
female genitalia
Normal female range
testosterone level
Absent uterus and upper
vagina & normal ovaries
Karyotype 46-XX
15-30% renal, skeletal and
middle ear anomalies
Imperforate Hymen
Androgen Insensitivity
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
 Primary Amenorrhea
Evaluation
Pregnancy test
Physical exam to determine presence of uterus
FSH
Karyotype
 Primary Amenorrhea
Treatment
Cyclic estrogen/progestin
Remove gonadal streaks if XY or mosaic
Increased (52%) risk of gonadoblastomas,
dysgerminomas, and yolk sac tumors
Pulsatile GnRH for ovulation induction in selected
patients
Surgical resection of intrauterine, cervical, and
vaginal adhesions/septa
 Secondary Amenorrhea
Pregnancy!
CNS disorders
Pituitary gland
Thyroid
Ovary
Uterus
Systemic disorders
Renal failure, liver disorders, DM
Medications: anti-psychotics, reserpine, COC,Gnrh A
 Secondary Amenorrhea
CNS disorders
Chronic hypothalamic anovulation
Stress
Increased exercise levels
Anorexia nervosa
Head trauma
Space-occupying lesions
 Secondary Amenorrhea
Pituitary disorders
Hyperprolactinemia
Prolactinoma
Medications
PCOS
Renal failure
Hypoprolactinemia
Pituitary resection
Sheehans syndrome
Thyroid disorders
Hyper- or hypothyroidism
 Secondary Amenorrhea
Ovulation disorders
Polycystic ovarian syndrome
Premature ovarian failure
Uterine abnormalities
Ashermans syndrome
Cervical stenosis
Drug-induced amenorrhea
Hormonal contraceptives
GnRH analogues
Ashermans Syndrome
 Secondary Amenorrhea
History
Nutrition/exercise habits, weight change
Sexual/contraceptive practice
History of uterine/cervical surgery
Physical exam
Height/weight
Hirsutism
Galactorrhea
Estrogen status of tissues
Laboratory
BhCG PRL & TSH progesterone challenge FSH if
high karyotype
 Negative Pregnancy.test

TSH ,PROLACTIN,
Progesterone
challenge test
withdrawal without withdrawal
bleeding bleeding

hypoestrogenic compromised
anovulation outflow tract
+ve.est/progest -ve.est/progest
challenge test challenge test

Normal FSH +
Normal FSH. FSH>30-40
Normal E2
Low E2 repeat

hypothalamic- Premature Ov HSG OR hysteroscopy

pituitary failure Failure Ashermans
 Secondary Amenorrhea
Treatment goals
Discovery and treatment of underlying cause
Hormone replacement
Menses every 1-3 months
Pregnancy
Ovulation induction
GnRH pump
HMG/HCG
Thank you