Amenorrhea

Question 1

The absence of menses in a woman who has never menstruated by the age 15 years in the presence of normal growth and secondary sexual characteristics

Girls who have not menstruated within 5 years of breast development if occurring by age 10

Answer 1

Primary Amenorrhea

Question 2

Etiologies of Primary Amenorrhea

Answer 2

Outflow tract abnormalities
End Organ Disorders
Central Disorders

Question 3

OUTFLOW TRACT ABNORMALITIES

May result from the failure of the Mullerian derived upper vagina to fuse with the urogenital sinus derived lower vagina

young woman who presents with primary amenorrhea and cyclic pelvic pain

Answer 3

Transverse Vaginal Septum

Question 4

OUTFLOW TRACT ABNORMALITIES

Have Mullerian agenesis or dysgenesis and may present with COMPLETE VAGINAL AGENESIS and absence of uterus or PARTIAL VAGINAL AGENESIS with rudimentary uterus and distal vagina

Answer 4

Mayer Rokitansky Küster Hauser (MRKH) syndrome

Diagnosis:
46xx with no patent vagina, (+) ovaries in ultrasound

Treatment:
creation of neovagina

Question 5

OUTFLOW TRACT ABNORMALITIES

Also known as Androgen Insensitivity Syndrome

Occurs in 1 in 50 000 women

Due to dysfunction or the absence of testosterone receptor

Answer 5

Testicular Feminization

phenotypically female with 46 XY chromosome

(+) testes - MIF is secreted thus Mullerian derived structures are absent

diminished testosterone – (-) pubic and axillary hair

(+) breasts
(-) uterus – amenorrhea

testicular cancer may occur

Question 6

END ORGAN DISORDERS

Ovarian Insufficiency

Answer 6

Primary Ovarian Insufficiency
Savage syndrome
Turner syndrome
17 hydroxylase deficiency

Question 7

END ORGAN DISORDERS

Defect in 17 a hydroxylase or 17, 20 desmolase

(-) testosterone produced

(+) MIF

phenotypically female (-) breast

Answer 7

Gonadal Agenesis with 46 XY Chromosomes

Question 8

END ORGAN DISORDERS

(-) testicular development
MIF not released
(+) female internal and external genitalia
(-) estrogen – no breast

Answer 8

Swyer Syndrome

Question 9

(-) breast

FSH < 5 IU/L
LH < 5 IU/L

Answer 9

Hypogonadotrophic hypogonadism

i.e. CNS tumor

Question 10

(-) breast

FSH > 20 IU/L
LH > 40 IU/L

Answer 10

Hypergonadotrophic hypogonadism

i.e. Ovarian failure (46 XX), Turner syndrome (45 XO)

Question 11

CENTRAL DISORDERS

Hypothalamic Disorders

Answer 11

Kallmann syndrome
Disruption of GnRH transport
Defects in GnRH pulsatility

Question 12

CENTRAL DISORDERS

Pituitary Disorders

Answer 12

RARE

Usually secondary to hypothalamic disorder

Question 13

(-) BREAST

(-) UTERUS

Answer 13

Gonadal agenesis in 46 XY

Question 14

(-) BREAST

(+) UTERUS

Answer 14

Gonadal failure/agenesis in 46 XX

Question 15

(+) BREAST

(-) UTERUS

Answer 15

Enzyme deficiencies in testosterone synthesis

Testicular feminization

Mullerian agenesis or MRKH

Question 16

(-) BREAST

(+) UTERUS

Answer 16

Disruption of HPA

Hypothalamic pituitary or ovarian pathogenesis similar to that of secondary amenorrhea

Congenital abnormalities of the genital tract

Question 17

Absence of menses for 3 mos in women with previously regular cycles or 6 mos in women with history of irregular cycles

Answer 17

Secondary Amenorrhea

Question 18

MCC of secondary amenorrhea

Answer 18

Pregnancy

Question 19

Causes of Secondary Amenorrhea

Answer 19

Anatomic
Ovarian Dysfunction
Prolactinoma and Hyperprolactinemia
CNS-Hypothalamic

Question 20

Secondary Amenorrhea

ANATOMIC

Obliteration of endometrial cavity

Answer 20

Intrauterine Adhesions or Synechiae (Asherman Syndrome)

Question 21

Most frequent antecedent factor of IUA

Answer 21

Endometrial curettage

Question 22

Secondary Amenorrhea

ANATOMIC

Can manifest as secondary amenorrhea and dysmenorrhea

Caused by scarring of the cervical os secondary to surgical or obstetric trauma

Answer 22

Cervical Stenosis

Question 23

Congenital absence of testes in genotypical male

Results in phenotypical picture similar to that of ovarian agenesis

(+) internal and external female genitalia

(-) breast

Answer 23

Swyer syndroem