Amenorrhea

Dr. Abigail Castro; Dr. Jessica Cruz | February 17, 2020 Study the algorithms and the tables J
For other info, read the book. Hehe

AMENORRHEA PUBIC HAIR GROWTH
• Absence of menses during the reproductive years
It is during the reproductive years when there is maturity of the HPO
Axis; 15-44 years of age
• Amenorrhea itself is not a pathologic entity and should not be
used as a final diagnosis. It is a symptom

PHYSIOLOGIC Pregnancy, postpartum

PATHOLOGIC Endocrine and anatomic disorders

PH1 Prepubertal: no pubic hair
Absence of menses in a woman who has never
PRIMARY PH2 Labial hair present
menstruated by the age of 16½ years

Absence of menses for an arbitrary time PH3 Labial hair spreads over mons pubis
SECONDARY
period, usually longer than 6 to 12 months
PH4 Slight lateral spread

Further lateral spread to form inverse triangle and reach

PUBERTY: Sequence of Physical Events PH5
medial thighs
EVENT AGE HORMONE

Breast development
10-11 ESTRADIOL
(Thelarche)

Appearance of pubic and

10.5-11.5 ANDROGENS
axillary hairs (Pubarche)

GROWTH
Maximal growth velocity 11-12
HORMONE

Menarche 11.5-13 ESTRADIOL

BREAST GROWTH

H-P-O INTERACTIONS
Sex Hormones during CHILDHOOD:
• Estrogen is LOW, LH & FSH are LOW
• CNS-HPO axis extremely sensitive to negative feedback effects of
low levels of circulating estrogen
B1 Prepubertal: elevation of papilla only
Sex Hormones PRIOR to puberty:
B2 Breast budding
• When the critical weight or body composition is achieved
Enlargement of breasts with glandular tissue, without o CNS–hypothalamic axis becomes less sensitive to the negative
B3
separation of breast contours effect of estrogen.
o GnRH is secreted in greater amounts
B4 Secondary mound formed by areola o This results in an increase in both LH and to a lesser extent
FSH.
B5 Single contour of breast and areola
Sex Hormones DURING puberty:
• Episodic pulses of LH during sleep and awake periods.
o The initial endocrinologic change in puberty.
o Occurs after menarche.
• Activation of positive gonadotropin response to increasing
estrogen levels.
o Last endocrinologic event in puberty.
o Results in midcycle gonadotrophic surge and ovulation.

TRANSCRIBER: Diana of
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 DELAYED MENARCHE
• Onset of menses in women older than 16.5 years who have no
reproductive abnormalities.
There is breast development with normal internal and external
female genitalia but does not menstruate ® Only requires
REASSURANCE
STRESS
• Stress per se can lead to inhibition of the GnRH axis.
• This may be the key factor influencing amenorrhea in competitive
athletes.
• Mechanism involves an increased secretion of CRH (releasing
ACTH and cortisol). CRH itself is known to inhibit GnRH

Factors Affecting Onset of Menarche:

1. Body Fat Composition
2. Strenuous exercise
3. Stress

BODY COMPOSITION
• The mean time of onset of menarche was previously thought to
occur when a critical body weight of about 48 kg (106 lb) was
reached.
SEXUAL DEVELOPMENT
• It is now believed that the ratio of fat to both total body weight
and lean body weight is probably the most relevant factor that Genetic Sex XX XY
determines the time of onset of puberty and menstruation. Gonadal sex Ovary Testes
• Moderately obese (20% & 30% above the IBW) ® earlier onset of
menarche than non-obese women (+) Wolffian Duct
Phenotypic Sex Mullerian duct
(due to MIH)
• Malnutrition (anorexia nervosa or starvation) ® delay onset of
puberty Uterus, fallopian
Internal Genital Vas deferens,
• Well-nourished individuals with prepubertal strenuous exercise tube, upper 1/3 of
Organs prostate
vagina
programs resulting in less total body fat ® delayed onset of puberty.
Labia minora and
External Genital
LEPTIN majora, mons Penis, scrotum
Organs
• Peptide secreted in adipose tissue pubis
• Circulates in the blood bound to a protein Sex of Rearing Female Male
• Acts on CNS neurons that regulate eating habits and energy
balance
• Increases during childhood until onset of puberty • Genetic Sex: established during fertilization by haploids (XX or
• ­ level of leptin – the earlier onset of menarche XY)
• Leptin is produced by adipocytes and correlates well with body • Gonad is still a Totipotential cell (still unknown) ® travels to the
weight. gonadal ridge at 5-6th weeks AOG and reach it ® absence of
• Leptin is also important for feedback involving GNRH and LH the Y ® develop into an ovary.
pulsatility and also binds to specific receptor sites on the ovary • Phenotypic sex: Presence of Y® Anti-Mullerian Hormone/
and endometrium. Substance/Factor ® Wolffian Duct ® male genitalia;
Absence of Y ® (-) Anti-Mullerian Hormone/
STRENUOUS EXERCISE Substance/Factor ® Mullerian Duct ® uterus, cervix,
• Young women with strenuous exercise programs that have fallopian tube and vagina
sufficient estrogen to produce some breast development do not • Sex of rearing should not be replaced. You have to continue on
need extensive endocrinologic evaluation if concern arises about the rearing of the patient as a female. Changing this would
lack of onset of menses. have an impact on the person, family, and community.
• They should be counseled that they will usually have a delayed
onset of menses, but it is not a health problem.
• They should be told that they will most likely have regular PRIMARY AMENORRHEA
ovulatory cycles when they either stop exercising or become • Criteria: No period by age 14 in the absence of growth or
older. development of secondary sexual characteristics
• Menarche is delayed about 0.4 year for each year of • A 14 year old showing no breast budding already needs further
premenarcheal athletic training. 3 MONTHS evaluation. FURTHER ENDOCRINOLOGIC EVALUATION
• When to evaluate: Failure to initiate breast development by age
Metabolic features of amenorrheic athletes 13
1. Elevated FSH • When PUBERTY begins, it usually lasts for about 4-5 years
2. Elevated IGFBP-1 ending in sexual maturity.
3. Lowered IGF • Mean interval is 2.3 years (SD of 1 year)

AMENORRHEA 2 of
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 BREASTS
UTERUS (-) (+)
(-) 3 2
(+) 1 4
1: FAILURE
2: (-) UTERUS = UTEROVAGINAL AGENESIS
3: ENZYME DEFICIENCY
4: DYSFUNCTIONAL

PRIMARY AMENORRHEA CLASSIFICATION

CATEGORY I: CATEGORY II: CATEGORY III: CATEGORY IV:

Breast (-) Breast (+) Breast (-) Breast (+)
Uterus (+) Uterus (-) Uterus (-) Uterus (+)

A.Hypothalamic failure secondary to inadequate GnRH release

(Hypogonadotropic Hypogonadism)
o Insufficient GnRH synthesis (Kallman’s Syndrome)
o Insufficient GnRH secretion Hypothalamic
o Congenital anatomic defects in the CNS (stenosis of the
aqueduct, absence of sellar floor)
o CNS neoplasm

B.Pituitary Failure
o Isolated Gonadotropin insufficiency (Thalassemia major,
retinitis pigmentosa)
o Pituitary neoplasia (pituitary adenoma, chromophobe
Pituitary
adenomas)
o Mumps encephalitis
o Newborn Kernicterus
o Prepubertal Hypothyroidism

C.Gonadal Failure (Hypergonadotropic Hypogonadism)

Androgen resistance Agonadism
o 45,X Anomalies (Turner’s Syndrome, Mosaicism) Ovarian
(testicular feminization) 17,20 desmolase
o Structurally Abnormal X Chromosome
deficiency
o Pure gonadal dysgenesis (46 XX, 46 XY with gonad streaks,
Congenital absence of 17α-hydroxylase
Gonadal agenesis) Uterine
uterus (MRKH) deficiency, 46 XY
o 46 XX, 17α-hydroxylase deficiency
Different anlage for the ovary and the uterus
Most common: Category I
Rarest: Category III

AMENORRHEA 3 of
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 CATEGORY I: BREAST (-), UTERUS (+)
• Lack of breast development is the most sensitive indicator that
the ovaries never secreted estradiol.
• Hypergonadotropic hypogonadism ® Gonadal Failure
• Hypogonadotropic hypogonadism ® H-P Failure
• XY ® Excise gonads (no development of breast because it’s a
testis; maybe intraabdominal)
• XX ® Look for signs of hyperandrogenism (hirsutism,
deepening of the voice, etc) ® (+) Means that there is a
gonad/testis secreting androgen® Excise
HYPERGONADOTROPIC HYPOGONADISM
• If the lack of estradiol is because of GONADAL (OVARIAN)
FAILURE: ¯Estradiol, ¯Progesterone, ­LH and FSH ®
HYPERGONADOTROPIC HYPOGONADISM
• Since estrogen is not necessary for müllerian duct development or
wolffian duct regression, the internal and external genitalia are
phenotypically normal female.
• When ovarian follicles are absent, synthesis of ovarian steroids and
inhibin does not occur. Breast development does not occur because
of the very low circulating E2 levels.
AMENORRHEA
I. 45X TURNER’S SYNDROME
• The diagnosis is usually made before puberty.
• Chances of menstruation and future pregnancy:
o Rarely may have a few follicles that develop under endogenous
gonadotropin stimulation early in puberty and may synthesize
enough estrogen to induce breast development and a few
episodes of uterine bleeding.
o However, they will have premature ovarian failure; usually
before age 25. Rarely, ovulation and pregnancy can occur.
II. MOSAICISM (X/XX. X/XXX. XXX)
• Clinical features: Primary amenorrhea and normal female external
genitalia, taller and have fewer anatomic abnormalities than
individuals with a 45,X karyotype.
• Chances of menstruation and future pregnancy:
o Occasionally may have a few follicles that develop under
endogenous gonadotropin stimulation early in puberty and may
synthesize enough estrogen to induce breast development
o 20% may have sufficient estrogen production to menstruate
o Rarely, ovulation and pregnancy may occur. However, they will
have premature ovarian failure; usually before age 25.
III. STRUCTURALLY ABNORMAL X CHROMOSOME
• 46 XX but part of X is abnormal
1. Deletion of the long arm of the X chromosome (Xq)
- These individuals have no somatic abnormalities
2. Deletion of the short arm of the X chromosome (Xp)
3. Isochrome of the long arm of the X chromosome.
4. Ring X and minute fragmentation of the X chromosome
IV. PURE GONADAL DYSGENESIS
• Genetic disorder and has been reported in siblings.
• 46 XX and 46 XY
• Manifestations: normal stature and phenotype, absence of
secondary sexual characteristics, and primary amenorrhea.
• Some may have a few ovarian follicles, develop breasts, and may
even menstruate spontaneously for a few years.
• Hyperandrogenism occurs in about 10% of women
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V. 17 a-HYDROXYLASE DEFICIENCY (46 XX) HYPOGONADOTROPIC HYPOGONADISM
• Rare Cause • If the lack of estradiol is because of HYPOTHALAMIC/
• Normal female internal genitalia PITUITARY FAILURE: ¯Estradiol, ¯Progesterone, ¯LH and FSH
• Clinical features: ® HYPOGONADOTROPIC HYPOGONADISM
o primary amenorrhea without breast development, normal
female internal genitalia • NO NEED FOR KARYOTYPING because ALL ARE XX ®
o elevated serum progesterone level (>3 ng/mL) REQUEST PROLACTIN, CT/MRI
o elevated serum deoxycorticosterone level (>17 ng/100 mL) • NO NEED for further testing if (+) Amenorrhea, galactorrhea
o low 17α-hydroxyprogesterone level (<0.2 ng/mL) BUT NORMAL ENDOCRINOLOGICALLY
o low cortisol
o high ACTH and mineralocorticoid
o hypernatremia, hypokalemia, hypertension
• Chances of menstruation and future pregnancy: If the lack of estradiol is Response to GnRH
o They have cystic ovaries and viable oocytes because of bolus/stimulation
o Individuals with 17α-hydroxylase deficiency do have
primordial follicles but cannot synthesize sex steroids. HYPOTHALAMIC FAILURE Will respond
o Pregnancies have been documented following in vitro
fertilization/embryo transfer (IVF-ET) despite low levels of PITUITARY FAILURE Will NOT respond
endogenous sex steroids

AMENORRHEA 5 of
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INSUFFICIENT GnRH SYNTHESIS (KALLMAN’S SYNDROME)
CATEGORY II: BREAST (+), UTERUS (-)
• The presence of the breasts indicates biologically active
estrogen. Thus, this assures an intact HP-gonadal axis.
However, the patient can be a male or female.
Ex. 16.5 years old presenting with primary amenorrhea. PE: (+)
breast, (-) uterus on Ultrasound. ® Category II ® Request for
Serum Testosterone and Observe pubic hair and axillary hair.
• Look at the escutcheon/distribution of pubic hair (female: inverted
triangle; male:diamond)
• Normal female range of testosterone and female escutcheon ®
Diagnosis: Congenital absence of uterus (MRKH
Syndrome/Uterovaginal agenesis) ® confirm ovulation
• Short vaginal canal, 3 cm thick (normal: 8 cm) ® Vaginal
reconstruction (McIndoe’s vaginoplasty)
• Can she bear a child? NO (No uterus)
• Can she have a biological child? YES through Surrogacy
(Present ovaries)
I. CONGENITAL ABSENCE OF UTERUS
• Uterine Agenesis, Uterovaginal Agenesis, Rokitansky Kuster
Hauser Syndrome
• Second most frequent cause of primary amenorrhea
• 46 XX: Normal endocrinologic females
• Normal FSH and LH, Female Estrogen, Progesterone and
testosterone
• Normal breast, normal pubic and axillary hair
• Shortened vagina
• Possible associated findings: Renal, skeletal, cardiac and other
abnormalities
AMENORRHEA
II. ANDROGEN INSENSITIVITY (TESTICULAR FEMINIZATION)
• Genetically transmitted: X-linked recessive or sex-limited
autosomal dominant disorder with transmission through the
mother
• Androgen receptor synthesis or action DOES NOT occur
• XY karyotype: normally functioning male gonads that produce
normal male levels of testosterone and dihydrotestosterone
• Normal male testosterone, absent pubic hair ® Diagnosis:
Androgen Insensitivity/Testicular feminization ® confirm
karyotype ® XY ® (+) Gonad ® Excise after 18 years old (to
have some hormone still functioning and the propensity for the
intraabdominal testis to develop into a malignancy occurs after
25 years, thus the need to monitor) ® Give estrogen for further
breast development
• Cannot have offspring (no ovaries)
Why (+) breast?:
o Breast development is normal or enhanced
o Estrogen levels here are in the male range but are sufficient
for breast proliferative activity.
o Testosterone inhibits breast proliferation. The absence of
androgen action allows even low levels of estrogen to cause
unabated breast stimulation.
• Testes that are intraabdominal or that occur in the inguinal canal
have an increased risk of developing a malignancy
(gonadoblastoma or dysgerminoma), with an incidence reported
to be about 20%
• Rare before age 20
• It is usually recommended that the gonads be left in place until
after puberty is completed, to allow full breast development
and epiphyseal closure to occur.
• At around age 18, the gonads should be removed.
• Patients should be informed that they have an abnormal sex
chromosome, without specifically mentioning a Y chromosome.
• Use term “gonads” instead of testes.
• They should also be informed that they can never become
pregnant because they do not have a uterus and that their gonads
(not testes) need to be removed after age 18 because of their high
potential for malignancy.
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 ANDROGEN RESISTANCE
Breast (+), Uterus (-) CONGENITAL ABSENCE OF UTERUS (RKH)
(TESTICULAR FEMINIZATION)
Karyotype 46 XY 46 XX
Maternal X-linked recessive
Hereditary 25% risk of affected child Not known
25% risk of carrier
Axillary and Pubic hair Absent to sparse, scanty Normal female body hair, ovulatory

+/- Ovulatory and Normal ovulatory female

None
PMS-like symptoms Biphasic basal temperature

Hormone levels Endocrinologically normal male Endocrinologically normal female

Other anomalies Rare Frequent

Remove gonads after breast development Mechanical dilatation of vagina

Epiphyseal closure (usually at 18 y/o) Surgical reconstruction of vagina (McIndoe)
Management Counselling No hormonal therapy needed
ERT Evaluate for additional renal, skeletal, cardiac and
Other anomalies rare. No need to evaluate other congenital abnormalities

Will never menstruate

Pregnancy and Will never menstruate
May have their own genetic children via ART using a
menstruation Cannot have children
surrogate recipient

CATEGORY III: BREAST (-), UTERUS (-)

• The absence of the breast development and uterus suggests
that we are dealing with a male phenotype.

SECONDARY AMENORRHEA
1. CNS-Hypothalamic Causes (62%): most common cause
2. Pituitary Causes (16%)
3. Ovarian Causes (12%)
4. Uterine Causes (7%)
I. 17 α-HYDROXYLASE DEFICIENCY (46 XY)

XY, (+) testes, (+) AMH/MIS, Ex. A 22 year old female, regularly menstruating suddenly stops
Why (-) uterus?
Mullerian duct regresses menstruating for 6 months
• Request for a Pregnancy test
Enzyme deficient thus no sex steroids • Evaluate uterus and ovaries (TVS) to know if patient is
Why (-) breasts? No estrogen ovulating
No breasts • If endometrium is thick on ultrasound (>5 mm) ®
Progesterone Challenge Test ® Give Progesterone for 5
II. AGONADISM days ® withdraw ® (+) bleed: endometrium is estrogen-
III. 17, 20 DESMOLASE DEFICIENCY primed; (-) bleed: endometrium is not estrogen-primed
• Request for Serum TSH ® Elevated: HYPOTHYROIDISM
CATEGORY IV: BREAST (+), UTERUS (+) • Request for Prolactin ® >100 ng/mL ®
• Genetic Female HYPERPROLACTINEMIA ® CT SCAN/MRI OF SELLA
• Second largest category TURCICA
• Profile similar to secondary amenorrhea.

AMENORRHEA 7 of
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 CNS-HYPOTHALAMIC CAUSES PCOS
Etiology:
1. Lesions in the hypothalamus
2. Drugs
3. Stress and Exercise
4. Weight loss
5. Polycystic Ovary Disease/Syndrome
6. Functional hypothalamic amenorrhea

LESIONS IN THE HYPOTHALAMUS

• Craniopharyngiomas, granulomatous diseases, etc
• Low gonadotrophin levels ® low estradiol levels

DRUGS
• Phenothiazines, some antihypertensives, other drugs
• OCPs ® persistent HP inhibition ® postpill amenorrhea
• This oral contraceptive-induced suppression should not last more
than 6 months.

STRESS AND EXERCISE

• ­ strenuous activity ® ¯ LH and FSH ® ­ b endorphins and
catechol estrogens
• Lowered body fats ® ­ catechol estrogens
• Catechol estrogen: ­ dopamine ® (-) GnRH ® no LH ® NO
OVULATION ® AMENORRHEA
• b endorphins: b endorphins ® (-) NE effect on GnRH ® (-) GnRH
® NO LH, ESTROGEN ® NO OVULATION
WEIGHT LOSS
• Amenorrhea associated with weight loss appears to be due mainly
to failure of normal GnRH release, with the lack of a pituitary
response under extreme conditions.
• Hypoleptinemia as well as GH and thyroid dysfunction contribute
to these findings.
1. Simple weight loss: Hypothalamic dysfunction
2. Severe weight loss: Possible additional pituitary disorder
ANOREXIA NERVOSA
• Severe psychiatric disorder
• Uncommon in men and rare in blacks and Asians
• Patients have a hypothalamic disorder interfering with normal
GnRH. Pituitary dysfunction also occurs when the weight loss
becomes severe
1. Normal T4, abnormally low T3
2. Elevated GH
3. High Cortisol
4. Low ACTH and DHEA-S
5. LH pattern similar to pre-pubertal girls
FUNCTIONAL HYPOTHALAMIC AMENORRHEA
• No underlying pituitary, hypothalamic or ovarian causes
• No cyclic alterations in LH pulsatility ® no pulses or only one pattern
seen throughout menstrual cycle (persistent luteal pattern)
• Possibly due to CNS neurotransmitter abnormality, increase
opioid activity
PITUITARY CAUSES
HYPOESTROGENIC AMENORRHEA
• NEOPLASMS (Chromophobe adenoma)
• NON-NEOPLASTIC LESION
1. Anoxia, thrombosis, hemorrhage ® pituitary cell damage
2. Sheehan syndrome ® hypotensive episode during pregnancy
(+) Pituitary necrosis (response of the master gland in cases of
severe hypotensive episodes)
Case: Normal delivery/D&C, massive bleeding, patient was able
to recover, followed by periods of amenorrhea. History of
hypotensive episodes during pregnancy/delivery
3. Simmonds’ disease ® hypotension unrelated to pregnancy
(+) Pituitary necrosis but patient is not pregnant
OVARIAN CAUSES
HYPOGONADOTROPHIC HYPOGONADISM
• Infection, interference of blood supply, bilateral cystectomies
that deplete follicles ® insufficient estrogen
• Cystic degeneration of the ovaries
PREMATURE OVARIAN FAILURE/INSUFFICIENCY: (<40 y/o)
1. Ovarian Sclerosis
2. Gonadal irradiation
3. Chemotherapy
4. Autoimmune associations (thyroid disease: Hashimoto’s)

AMENORRHEA 8 of
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 UTERINE CAUSES
Desires pregnancy:
• The likelihood of the diagnosis is strengthened if a sound cannot Exogenous gonadotropins
be passed into the uterine cavity.
Donor eggs
1. Intrauterine adhesions (IUAs) or synechiae (Asherman's H-P Failure (POF)
syndrome) Does not desire pregnancy:
a. Postabortal curettage (30%): overzealous curettage Estrogen progesterone
b. D&C in non-pregnant patient replacement
c. Severe endometritis or fibrosis following a myomectomy,
metroplasty, or cesarean delivery
2. Missed abortion or endometrial tuberculosis

DIAGNOSTIC EVALUATION
1. History and PE
2. Ancillary diagnostic tools
• CBC, Urinalysis
• TSH assay
• Serum E2, Progesterone challenge test, Endometrial imaging
(TVS)
• Serum FSH, Prolactin

E2 ABOVE 30-40 pg/ml

Plus PCO by
Diagnosis: PCOS
ultrasound

(+) History of drug ingestion, stress,

weight loss, exercise
NO PCO by
Diagnosis: Hypothalamic pituitary
ultrasound
dysfunction
Self-limiting, not life threatening

E2 LOW
Diagnosis: CNS lesions/HP failure
If history of drug ingestion, stress, weight
Plus LOW FSH
loss, exercise NOT present, CT/MRI is
warranted
Diagnosis: POF
Plus HIGH FSH Antithyroid & Antinuclear antibodies
Karyotype

MANAGEMENT
• Management depends on:
1. DIAGNOSIS
2. DESIRE FOR PREGNANCY

Non-prolactin secreting
Excised if possible
tumor
Pituitary adenoma Medical therapy

Women with macroadenoma

(>2 cm) who fail to respond
to medical therapy or have Surgery
poor compliance with
regimen (BROMOCRIPTINE)

Desires pregnancy:
1. Comprehensive Gynecology 7th edition
Clomiphene citrate
PCOS/H-P Dysfunction 2. PowerPoint
Does not desire pregnancy: 3. Manual
Cyclic MPA 4. Recordings
5. VM Trans

AMENORRHEA 9 of
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From Manual and book
Category I: (-) breast, (+) uterus

• Serum FSH

a. 0.625 mg Conjugated equine estrogen (CEE) for breast proliferation

Karyotyping b. Cortisol replacement + sex steroid treatment for 17a-hydroxylase
Serum electrolytes (Na, K) deficiency
Hypergonadotrophic
Serum progesterone c. Excision of the streak gonads if (+) Y chromosome
hypogonadism
17aOH progesterone, DOC d. Excision of gonads not necessary if (-) Y chromosome
BP monitoring e. Excision of gonads if (-) Y chromosome but with signs of hypergonadism
Chances of pregnancy: 50% if with normal uterus and with egg donation
Prolactin a. Estrogen-progestogen treatment to induce breast development and
Hypogonadotrophic CT Scan/MRI cause epiphyseal closure
hypogonadism TSH Chances of pregnancy: Ovulation can be induced using human
T3, T4 menopausal gonadotropins and pulsatile GnRH

Category II: (+) breast, (-) uterus

• Serum testosterone + Observation of pubic and axillary hair

Congenital absence of a. Renal scan

Normal female testosterone
uterus (MRKH b. Surgical reconstruction of an absent vagina (McIndoe procedure)
Normal pubic and axillary hair
Syndrome) Chances of pregnancy: IVF, surrogacy

Androgen insensitivity Normal male testosterone a. Excision of gonads after 18 years old
(Testicular feminization) Absent pubic and axillary hair b. Thereafter, estrogen replacement therapy should be administered

Category III: (-) breast, uterus

• Karyotype (46,XY)

Enzyme deficiency (17a-hydroxylase def with 46, XY a. Excision of gonads

karyotype b. Hormonal therapy
Agonadism (Vanishing Testes Syndrome) c. Refer to an endocrine center for the extensive evaluation necessary to
17, 20 desmolase establish

Category IV: (+) breast, uterus

• TSH, FSH, Prolactin, serum estradiol, progesteronal challenge test

Hypothyroidism Elevated TSH Thyroid hormone

Hyperprolactinemia Prolactin >100 ng/ml Bromocriptine

(+) withdrawal bleed after PCT a. Desires pregnancy: Clomiphene citrate

Anovulation - PCOS
E2 30-40 pg/ml b. Does not desire pregnancy: Cyclic MPA
c. Lesions in the hypothalamus: Excision
d. Drugs/postpill amenorrhea
e. Weight loss: gain weight
f. Stress and exercise: avoid/ reduce strenuous activities, gain weight,
estrogen supplementation administration to prevent possible
(-) withdrawal bleed after PCT
Hypothalamic development of osteoporosis
Low E2, FSH
amenorrhea g. Anorexia nervosa: gain weight, refer to psychiatrist
(+) Lesion on MRI
h. PCOS/H-P dysfunction: Ovulation induction (Clomiphene citrate or
letrozole) if desirous of pregnancy; Progesterone therapy
(Progestogen/MPA) if not desirous of pregnancy to reduce risk of
endometrial cancer associated with unopposed estrogen; metformin
therapy

AMENORRHEA 10 of
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 a. Desires pregnancy: Exogenous gonadotrophins or pulsatile GnRH; POI:
(-) withdrawal bleed after PCT Donor oocytes and priming of their endometrium with Estrogen and
H-P Failure
Low E2 Progesterone for embryo transfer
POI
High FSH a. Does not desire pregnancy: Estrogen-Progestogen replacement to
reduce risk of osteoporosis
a. Chromophobe adenoma: excision
Hypoestrogenic
a. Sheehan syndrome: hormone replacement
amenorrhea (pituitary)
a. Simmonds’ disease: hormone replacement

Hypogonadotropic
amenorrhea a. POF: hormone replacement
(Ovarian)

a. IUAs or Synechiae (Asherman’s Syndome): Estrogen therapy

Endometrial destruction
a. Endometrial TB: medical therapy

AMENORRHEA 11 of
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