Amenorrhea

Amenorrhea
Presentation lecture by:Jean Amoura, MD, MSc

Amenorrhea
* Primary
o Absence of menses by age 16 with normal secondary sexual characteristics
o Absence of menses by age 14 without secondary sexual development
* Secondary
o Absence of menses for 6 months in a previously menstruating female

Events of Puberty
* Thelarche (breast development)
o Requires estrogen
* Pubarche/adrenarche (pubic hair development)
o Requires androgens
* Menarche
o Requires:
o GnRH from the hypothalamus
o FSH and LH from the pituitary
o Estrogen and progesterone from the ovaries
o Normal outflow tract

Are there secondary sexual characteristics?

Primary Amenorrhea
Think hypogonadism or hypogonadotropism
Amenorrhea with Immature Secondary Characteristics
FSH Serum level Low / normal / High
Hypogonadotropic
hypogonadism
Gonadal
dysgenesis

* Hypogonadism
* Enzyme deficiencies
* Kallmann’s syndrome, CNS tumors
* Irradiation
* Chemotherapy
* Galactosemia
* Hypogonadism (gonadal failure)
* 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
o Hypothalamic dysfunction
+ Kallmann syndrome
+ Anorexia nervosa
+ Space-occupying lesion of CNS
+ Marijuana use
o Pituitary damage (surgery/radiation)
o Constitutional delay

Are there secondary sexual characteristics?
* Is there normal development of secondary sexual characteristics?
* Think
o Pregnancy
o Mullerian anomaly
o Androgen insensitivity
* Mullerian Anomalies
o Mullerian agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome)
o Imperforate hymen
o 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
* Evaluation
o Pregnancy test
o Physical exam to determine presence of uterus
o FSH
o Karyotype

Primary Amenorrhea
Secondary Amenorrhea
* Pregnancy!
* CNS disorders
* Pituitary gland
* Thyroid
* Ovary
* Uterus
* Systemic disorders
* CNS disorders
* Pituitary disorders
* Thyroid disorders
* Ovulation disorders
* Uterine abnormalities
* Drug-induced amenorrhea

Asherman’s Syndrome
Secondary Amenorrhea
Negative Pregnancy.test
TSH ,PROLACTIN, Progesterone challenge test
withdrawal bleeding
without withdrawal bleeding
hypoestrogenic
compromised outflow tract
* Treatment goals
* Sexually active, using condoms
* No recent change in weight, skin, hair
* Occasional heat intolerance
* No cyclic pain
* No gynecologic surgery
* Regular menses (every 28-30 days) prior to past year

* Exam
o Overweight
o No galactorrhea
o Normal hair distribution
o Normal pelvic exam
* Pregnancy test
* Progestin challenge, TSH, serum prolactin
* Estrogen/progestin cycle, FSH

Amenorrhea.ppt