Amenorrhea

Amenorrhea
Dra. Maria de Lurdes Silva

Amenorrhea
TÍTULO DA APRESENTAÇÃO
1. Definition
Primary Amenorrhea
• No menarche by 16 years in presence of
normal thelarche
• No menarche by 14 years with no thelarche
Secundary Amenorrhea
• Abcence of menstrual period for ≥ 3
consecutive cycles or 6m (if irregular cycles) in
a woman who was previously menstruating
Amenorrhea
2. Normal menstrual cycle

Menstrual Cycle
• Normal gonadotrophin-
releasing hormone (GnRH)
• Normal gonadotrophins
prodution (FSH, LH)
• Steroid hormones (ovary)
• Normal uterus
• Intact genital outflow tract
Outflow
Amenorrhea
2. Normal menstrual cycle

Menstrual Cycle
• 6-8w of gestation: rapid

multiplication of germ cells
• Max 16-20w: 6-7millions of oocytes
• Progressive decline until ±50 years
• Some chromosomal anomalies
could accelerate this process
Amenorrhea
3. Evaluation
Is essencial to exclude pregnancy
• Pregnancy is the most common cause of amenorrhea
Patient history
• Date of last menstrual period, history of amenorrhea

• Disorders development / growth
• Past medical illnesses
• Exercise, dietary history, eating disorders
• Medications, illicit drugs use
• Stress; psychiatric history; history of congenital anomalies
Physical examination
• Secondary sex characteristics (Tanner staging)

• Evaluation of genital outflow tract
• Hiperandrogenic features: hirsutism, acne, obesity (BMI); galactorrhea
Amenorrhea
3. Evaluation
Laboratory studies
• βHCG (β-human chorionic gonadotropin) exclude

pregnancy)
• TSH (thyroid-stimulatin hormone)
• PRL (prolactin)
• FSH (follicle-stimulating hormone)
Ginecologic Ultrassound
• Presence ou absence of the uterus and/or vagina

Amenorrhea
3. Gonadal Axis
WHO definition
SNC
• Hypogonadotrophic Hypogonadism
• FSH N/ Estrogen 
I Hypothalamus
Eugonadism GnRH
FSH N Estrogen N PRL N
Pituitary
II
FSH LH
• Hypergonadotrophic Hypogonadism Ovary

• FSH Estrogen 
III Estrogen Progestogen
Uterus
Menstruation
Amenorrhea
3. Outflow tract disorders

SNC
Hypothalamus
GnRH
Pituitary
FSH LH
Ovary
Estrogen Progestogen
FSH N
Uterus Estrogen N
PRL N
Menstruation
Amenorrhea

FSH N
Absent uterus Estrogen N
PRL N
karyotype
Primary
amenorrhea
46, XX 46, XY
Müllerian agenesis Complete androgen

Mayer-Rokitansky-Hüster- insensitivity syndrome
Hauser Syndrome
X-linked recessive disorder
Congenital absence of the Normal female phenotype.
vagina with variable uterine Hypoplasia or absence of vagina with
development absence uterus
Resistance to Testoterone due a defect in
the androgen receptor
Testes should be surgically excised after
puberty because of the increased risk of
Adapt by UptoDate developing testicular cancer after age 25
years
Amenorrhea

FSH N
Estrogen N
Uterus present PRL N
Primary Secundary
amenorrhea amenorrhea
Transverse Imperforate Asherman
vaginal septum hymen Syndrome
One or more transverse An imperforate hymen is Scarring of the uterine cavity is the most
vaginal septae can occur at any the simplest defect that anatomic cause of secondary
level between the hymenal ring results in primary amenorrhea
and the cervix. After menarche, amenorrhea. Causes: endometial instrumentation
the major symptoms are similar It may be associated with (curetage), uterine artery embolization,
to those associated with an cyclic pelvic pain and a uterine surgery or infections
imperforate hymen. perirectal mass from
sequestration of blood in
the vagina (hematocolpos)
Adapt by UptoDate
Amenorrhea
4. Ovarian dysfunction
SNC
Hypothalamus
GnRH
Pituitary
FSH LH
 Hypergonadotropic
Ovary Hypogonadism
WHO III
FSH 
Uterus Estrogen 
PRL N
Menstruation
Amenorrhea
Primary ovarian FSH 
insufficiency Estrogen 
PRL N
Primary ou
Abnormal karyotype secondary
amenorrhea
45, X0 gonadal Mosaic 46,XY gonadal

dysgenesis 45,X/46,XX dysgenesis
Turner Syndrome
Amenorrhea occurs because the oocytes and follicles
undergo accelerated apoptosis.
Spontaneous puberty and menstruation occur more
commonly in women with a mosaic karyotype (45,X/46,XX) short stature
but can occur in women with a 45,X karyotype. lymphoedema
Women with Turner syndrome whose karyotype includes a thick neck tissue
Y chromosome (such as 45,X/46,XY mosaicism) are at swelling of the neck
(cystic hygroma)
increased risk for gonadoblastoma. heart conditions
kidney abnormalities
Cubitus valgus
Amenorrhea
PRL N
Primary ou
Abnormal karyotype secondary
amenorrhea
45, X0 gonadal Mosaic 46,XY gonadal

dysgenesis 45,X/46,XX dysgenesis
Swyer Syndrome
In complete 46,XY gonadal dysgenesis, the fibrous

streak gonad cannot secrete anti-müllerian hormone
(AMH). This results in persistent müllerian structures
and a Female phenotype.
The phenotype of partial gonadal dysgenesis can
range from genital ambiguity to an undervirilized
male.
Amenorrhea
PRL N
Normal Primary ou
karyotype secondary
amenorrhea
46,XX gonadal
46 XX
dysgenesis
Primary ovarian Perrault Syndrome

insufficiency (POI)
Some cases of gonadal dysgenesis result from

POI is defined as the development of clinical autosomal recessive inheritance.
menopause before the age of 40 years in women who There are syndromic forms associated with
have normal karyotype. Causes:Gonadotropin-resistant hearing loss
ovary syndrome(SavageSyndrome);Genetic; Autoimune
disorders;RT/QT; Uknown causes.
Amenorrhea
5. Hypothalamic-Pituitary Disfunction
SNC
Hypothalamus Hypogonadotrophic
Hypogonadism
GnRH WHO I
Pituitary FSH /N
Estrogen 
FSH LH PRL N
Ovary
Uterus
Menstruation
Amenorrhea
FSH /N
Hypothalamic-
Pituitary Disfunction
Estrogen 
PRL N
Primary ou
secondary
Normal MRI amenorrhea
Functional
Isolated GnRH
hypothalamic Systemic illness
deficiency
amenorrhea
Functional hypothalamic amenorrhea

Abnormal hypothalamic GnRH secretion  decreased gonadotropin pulsations
Is a disorder that,by definition, excludes pathologic disease.
Role of leptin deficiency.
Causes:
Gonadotropins  ou N
MRI N
- eating disorders (such as
Absence of menses anorexia nervosa, bulimia)
- exercise
- stress
Amenorrhea
Hypothalamic- FSH /N
Pituitary Disfunction Estrogen 
PRL N
Primary ou
secondary
Functional
Isolated GnRH
hypothalamic Systemic illness
deficiency
amenorrhea
Idiopathic hypogonadotropic Kallman Syndrome

hypogonadism
Complete congenital GnRH deficiency
Although rare, primary amenorrhea can be associated with anosmia.
due to complete congenital gonadotropin-
releasing hormone (GnRH) deficiency.
Amenorrhea
5. Hypothalamic-Pituitary Disfunction FSH /N

Hypothalamic- Estrogen 
Pituitary Disfunction PRL N
Primary ou
secondary
Functional
Isolated GnRH Systemic illness
hypothalamic
deficiency
amenorrhea
Sistemic Illness
Systemic illness can cause secondary or primary

amenorrhea when it is severe enough to result
in a decrease in hypothalamic GnRH secretion.
Hypothalamic tumors ; infiltrative lesions
Examples include:
- celiac disease
- type 1 diabetes mellitus
- inflammatory bowel disease
Amenorrhea
Hypothalamic- SNC
FSH /N Primary ou

Estrogen 
PRL  Abnormal MRI
secondary
amenorrhea
 Hypothalamus
GnRH
PRL
Pituitary
Other diseases of the Empty Sella
Hyperprolactinemia
pituitary Syndrome
FSH LH
Among pituitary disorders that cause secondary amenorrhea, lactotroph Ovary

adenomas (prolactin-secreting pituitary adenomas) are the most common.
Hyperprolactinemia has a similar presentation to functional hypothalamic Estrogen Progestogen
amenorrhea, except for the additional finding of galactorrhea in some women.
Prolactin release is mostly controlled by inhibition,primarily by hypothalamic
dopamina.Disruption by trauma or a large tumor, leads to hyperprolactinemia.
As a general rule, the serum prolactin concentration correlates with pituitary
adenoma size.
Amenorrhea
Estrogen 
PRL 
Primary ou
secondary
Abnormal MRI amenorrhea
Other diseases of Empty Sella

Hyperprolactinemia
the pituitary Syndrome
Uncommon causes of gonadotropin deficiency:
- Sheehan syndrome
- Radiation
- Infarction
- Infiltrative lesions of the pituitary gland, such as
hemochromatosis and lymphocytic hypophysitis
Amenorrhea
Estrogen 
PRL 
Primary ou
secondary
Abnormal MRI amenorrhea
Other diseases of the Empty Sella

Hyperprolactinemia
pituitary Syndrome (ESS)
Primary ESS happens when a small anatomical defect above the

pituitary gland increases pressure in the sella turcica and causes the
gland to flatten out along the interior walls of the sella turcica cavity.
Secondary ESS is the result of the pituitary gland regressing within the
cavity after an injury, surgery, or radiation therapy
Amenorrhea
6. Eugonadism
SNC
Hypothalamus Eugonadism
WHO II
GnRH
FSH N
Pituitary Estrogen N
PRL N
FSH LH
Ovary
Uterus
Menstruation
Amenorrhea
6. Eugonadism
FSH N
Eugonadism Estrogen N
PRL N
Polycystic ovary
Thyroid disease
syndrome (PCOS)
PCOS - the most common reproductive disorder in women

its pathogenesis is complex
20% of cases of amenorrhea
50% of cases of oligomenorrhea
The principal features of PCOS include androgen excess, ovulatory
dysfunction, and/or polycystic ovaries.
In addition: overweight/obese, insulin resistence, acantose nigricans.
Amenorrhea
6. Eugonadism FSH N
Estrogen N
Eugonadism
PRL N
Polycystic ovary
Thyroid disease
syndrome (PCOS)
Menstrual disturbances were common in women with

hypothyroidism (35 and 10% for severe and mild-moderate
hypothyroidism, respectively).
TSH /
In women with severe hyperthyroidism, amenorrhea and
hypomenorrhea occurred in 2.5 and 3.7%, respectively.
Amenorrhea
7. Post-pill amenorrhea
Subjectively, post-pill amenorrhea is the failure to resume menstruation within
6 months after discontinuation of oral contraceptives or 12 months after
discontinuation of progestogen-only injectable contraceptives.
Prior to 6 months, detailed laboratory evaluation is not indicated, but after 6

months of amenorrhea, the history and physical status should again be carefully
evaluated.
Pregnancy should always be excluded before further testing.

Amenorrhea
Dra. Maria de Lurdes Silva

Amenorrhea

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Amenorrhea

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Amenorrhea

Dra. Maria de Lurdes Silva

2. Normal menstrual cycle

2. Normal menstrual cycle

• 6-8w of gestation: rapid

• Pregnancy is the most common cause of amenorrhea

• Date of last menstrual period, history of amenorrhea

• Secondary sex characteristics (Tanner staging)

• βHCG (β-human chorionic gonadotropin) exclude

• Presence ou absence of the uterus and/or vagina

• Hypergonadotrophic Hypogonadism Ovary

3. Outflow tract disorders

3. Outflow tract disorders

Müllerian agenesis Complete androgen

3. Outflow tract disorders

45, X0 gonadal Mosaic 46,XY gonadal

45, X0 gonadal Mosaic 46,XY gonadal

In complete 46,XY gonadal dysgenesis, the fibrous

Primary ovarian Perrault Syndrome

Some cases of gonadal dysgenesis result from

Functional hypothalamic amenorrhea

Idiopathic hypogonadotropic Kallman Syndrome

5. Hypothalamic-Pituitary Disfunction FSH /N

Systemic illness can cause secondary or primary

FSH /N Primary ou

Among pituitary disorders that cause secondary amenorrhea, lactotroph Ovary

Other diseases of Empty Sella

Uncommon causes of gonadotropin deficiency:

Other diseases of the Empty Sella

Primary ESS happens when a small anatomical defect above the

PCOS - the most common reproductive disorder in women

Menstrual disturbances were common in women with

Prior to 6 months, detailed laboratory evaluation is not indicated, but after 6

Pregnancy should always be excluded before further testing.

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