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Amenorrhea - classification

Anatomic Defects
Ovarian Failure
Chronic anovulation with estrogen present
Chronic anovulation with estrogen absent

Anatomic Defects (outflow tract)

Anatomic Defects
A. Labial agglutination/fusion
B. Imperforate hymen
C. Transverse vaginal septum
D. Cervical agenesis isolated
E. Cervical stenosis iatrogenic
F. Vaginal agenesis isolated
G. Mllerian agenesis (Mayer-Rokitansky syndrome)
H. Complete androgen insensitivity (T. fem)
I. Endometrial hypoplasia or aplasia congenital
J. Ashermans syndrome (intrauterine synechiae)

Ovarian failure
(hypergonadotropic hypogonadism)
A. Gonadal agenesis
B. Gonadal dysgenesis
1. Abnormal karyotype
a. Gonadal dysgenesis 45, X
b. Mosaicism
2. Normal karyotype
a. Pure Gonadal dysgenesis
i. 46, XX
ii. 46, XY (Swyers syndrome)
C. Ovarian enzymatic deficiency
1. 17-Hydroxylase deficiency
2. 17,20-Lyase deficiency
3. Aromatase deficiency

Ovarian failure

Ovarian failure - continued

D. Premature ovarian failure
1. Idiopathic premature aging
2. Injury
a. Mumps oophoritis
b. Radiation
c. Chemotherapy
3. Resistant ovary
a. Idiopathic
b. Mutations of FSH receptor
c. Mutations of LH receptor
4. Autoimmune disease
5. Galactosemia

Chronic anovulation, estrogen present

Chronic anovulation with estrogen present
1. Hyperthecosis
B. Adrenal disease
1. Cushings syndrome
2. Adult-onset CAH
C. Thyroid disease
1. Hypothyroidism
2. Hyperthyroidism
D. Ovarian tumors
1. Granulosa-theca cell tumors
2. Brenner tumors
3. Cystic teratomas
4. Mucinous/serous cystadenomas
5. Krukenberg tumors

Chronic anovulation, estrogen absent

(hypogonadotropic hypogonadism)
A. Hypothalamic
1. Tumors
a. Craniopharyngioma
b. Germinoma
c. Hamartoma
d. Hand-Schller-Christian disease
e. Teratoma
f. Endodermal sinus tumors
g. Metastatic carcinoma
2. Infection and other disorders
a. Tuberculosis
b. Syphilis
c. Encephalitis/meningitis
d. Sarcoidosis
e. Kallmans syndrome
f. Idiopathic hypogonadotropic hypogonadism
g. Chronic debilitating disease

Chronic anovulation, estrogen absent

A. Hypothalamic
3. Functional
a. Stress
b. Weight loss/diet
c. Malnutrition
d. Psychological eating disorders (anorexia nervosa,
e. Exercise
f. Pseudocyesis

Chronic anovulation, estrogen absent

(hypogonadotropic hypogonadism)
B. Pituitary
1. Tumors
a. Prolactinomas
b. Other hormone-secreting pituitary tumors (corticotropin, thyrotropin,
c. Nonfunctional tumors (craniopharyngioma)
d. Metastatic carcinoma
2. Space-occupying lesions
a. Empty sella syndrome
b. Arterial aneurysm
3. Necrosis
a. Sheehans syndrome
b. Panhypopituitarism
4. Inflammatory/infiltrative
a. Sarcoidosis
b. Hemochromatosis
c. Lymphocytic hypophysitis
5.Gonadotropin mutations (FSH)
6.GnRH receptor mutations


Initial workup of amenorrhea

1. History and physical examination (clinical
evaluation of estrogen status)
2. R/O pregnancy
3. Measure plasma FSH, prolactin, TSH
4. Progestin administration