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Amenorrhea
Jean Amoura, MD, MSc
AmenorrheaPrimary
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
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
Are there secondary sexual characteristics?
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
Hypogonadotropichypogonadism
Gonadal dysgenesis
Primary Amenorrhea Hypogonadism
30% have genetic abnormality Gonadal dysgenesis, Turner’s syndrome, mosaicism
Enzyme deficiencies Kallmann’s syndrome, CNS tumors Irradiation Chemotherapy Galactosemia
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 turner’s syndrome (45XO) - Turner variants (45XO/46XX),(46X-abnormal X) - Mixed gonadal dygenesis (45XO/46XY) Chromosomally normal - 46XX (Pure gonadal dysgeneis) - 46XY (Swyer’s 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 Is there normal development of
secondary sexual characteristics?
YESThink
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 AmenorrheaEvaluation
Pregnancy test Physical exam to determine presence of
uterus FSH Karyotype
Primary AmenorrheaTreatment
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 select 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
Secondary AmenorrheaCNS 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 Sheehan’s syndrome
Thyroid disorders Hyper- or hypothyroidism
Secondary Amenorrhea Ovulation disorders
Polycystic ovarian syndrome Premature ovarian failure
Uterine abnormalities Asherman’s syndrome Cervical stenosis
Drug-induced amenorrhea Hormonal contraceptives GnRH analogues
Asherman’s Syndrome
Secondary AmenorrheaHistory
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 bleeding
without withdrawal bleeding
hypoestrogenic compromised outflow tract
+ve.est/progest challenge test
-ve.est/progest challenge test
FSH>30-40Normal FSH
HSG OR hysteroscopy Asherman’s
FSH norm.
repeatRepeat+serum estrogen level
PreOvFailure
hypothalamic-pituitary failure
anovulation
Secondary AmenorrheaTreatment goals
Discovery and treatment of underlying disorder
Hormone replacement Menses every 1-3 months Pregnancy
Ovulation induction GnRH pump FSH/LH
Amenorrhea26 yo Gravida 0 with menarche at age
14 presents with one-year history of amenorrhea.
AmenorrheaSexually active, using condomsNo recent change in weight, skin, hairOccasional heat intoleranceNo cyclic painNo gynecologic surgeryRegular menses (every 28-30 days)
prior to past year
Amenorrhea Exam
Overweight No galactorrhea Normal hair distribution Normal pelvic exam
Pregnancy test Progestin challenge, TSH, serum prolactin Estrogen/progestin cycle, FSH
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