Amenorrhea

Made Easy
By:

Mohammad Emam
Prof. OB & GYN
Mansoura Faculty of Medicine
EGYPT

2015

1
 Definition Of Amenorrhea

• Is complete absence of
menstruation in the
childbearing period.
Definition Of Amenorrhea

Absence of menstruation.
 Background
• Understanding normal menstruation.

• Classification of amenorrhea.

• Amenorrhea is a Symptom not a disease, so

the final diagnosis should be pathological .
Pre-requisities for normality of menstruation

• Coordinated Neuro endocrine Axis.

• Responsive ,patent Utero vaginal canal.

• Good general health .

Coordinated pituitary- ovarian –uterine Axis

VARIABLE CONSTANT
 Classifications Of Amenorrhea
• According to the onset:
– Primary amenorrhea.
– Secondary amenorrhea.
• According to the cause:
– Physiological.
– Pathological

• According to Hidden or apparant:

– False amenorrhea (Crypto menorrhea).
– True amenorrhea.

• These are complementary to each other

Primary & Secondary

Primary Secondary
pubertal changes - Marshall & Tanner

Regular sequence of events between ages of 10-16 yrs in girls

GROWTH SPURT 8 - 14 yrs (9 yrs)

6 - 10 cm / yr peak
2.5 yrs duration

BREAST GROWTH (Thelarche) 8 - 13 yrs (11 yrs)

PUBIC HAIR (Pubarche) 9 - 13 yrs

1st Pubertal Sign in 25%

AXILLARY HAIR (Adrenarche) 9.5 - 15 yrs

may follow menarche

MENSTRUATION (Menarche) 10 - 16 yrs (13 yrs)

 According to cause
Physiological. Pathological:
•Before puberty. Primary:
True
•After menarche. ( physiologic & pathologic)
False
•During pregnancy. Secondary:
•During lactation . True
( physiologic & pathologic)
•During menopause False
 Hidden or true :
Crypto menorrhea =
Obstruction of outflow tract below internal OS :
• Primary:
» Cervical atresia .
» Vaginal aplasia.
» Transverse vaginal septum
» Imperforate hymen

• Secondary:
» Cervical Cauterization.
» Cervical conization ( Leep
diathermy) .

» Vaginal synechiae
 Workup FOR DIAGNOSIS
•Exclude physiologic causes

•Exclude anatomical.

•Clinical workup
Exclude physiologic causes
 Exclude anatomical:

Cryptomenorrhea Ambiguous genitalia

 Crypto menorrhea
- Intermittent abdominal pain
- Possible difficulty with micturition
- Possible lower abdominal swelling
- Bulging bluish membrane at the
introitus or absent vagina (only
dimple)
Imperforate hymen

16
False (crypto menorrhea)

hematocolpos
hematometra

17
 Imperforate hymen
Hymenotomy or curiciate incision
 Clinical workup
Four phenotypes (Breast & uterus )

1. Absent breast + presence of uterus

2. Presence breast + absence uterus

3 A bsence breast + absence uterus

4. Presence breast + presence uterus
Breast is absent in cases with

Hypogonadism
Absent breast + presence of uterus
(Hypogonadism)
Serum FSH
LOW (less than 5 IU/l.)
HIGH (more than 20 IU/l)

Hypothalamo - pituitary PRIMARY OV. FAILURE

GnRH challenge .

LOW FSH HIGH FSH Gonadal dysgenesis

KARYOTYPE
Gonadal biopsy
PITUITARY HYPOTHALAMIC

History , exam & investigation

Gonadal dysgenesis (Turner’ s syndrome)
• Sexual infantilism and short stature.
• Associated abnormalities:
– webbed neck,
– cubitus valgus
– coarctation of the aorta,
– high-arched palate,
– broad shield-like chest with widely spaced nipples,
– short metacarpal bones
– Renal anomalies.
• Bilateral streaked gonads.
• Karyotype - 80 % 45, X0
- 20% mosaic forms (46XX/45X0)

• Treatment : HRT
Gonadal Dysgenesis ( Turner’s syndrome)
 Turner’s syndrome

(Classic 45-XO) Mosaic (46-XX / 45-XO)

Causes of hypothalmo - pituitary
• Congenital
• Traumatic
• Inflammatory
• Neoplastic
• Miscellaneous
 Hypothalamus & Pituitary
(Hypogonadotropic )
• Hypothalamus :
• Stress
• Weight changes :anorexia nervosa ,bulimia
• Exercise
• Psuedocyesis ( ?!!!) Thanks for sonar
• Syndromes

• Pituitary :
• Craniopharyngioma.
• Adenomas.
• syndromes . e.g. Sheehan's syndrome
 Emotional stress
• Emotional
shock
• Stress
Endorphins
Gn Rh
 Weight changes

• (15% of ideal wt for

Weight loss
age) whether By:

• Diet regimen
• Anorexia nervosa
• Excess weight gain :
• simple overeating
• Bulimia nervosa.

28
 Kallmann Syndrome

• Deficient secretion of GnRH + anosmia.

• Possible co-existing features include:

• Bone anomalies.
• Renal anomalies.
• Cleft lip and palate.
• Color blindness. .
Frolich syndrome
–Primary amenorrhea.
–Hypogonadism.
–Trunkal obesity.
Laurence-Moon -Biedle syndrome

• As Frolich syndrome with:

– Polydactyl.
– Syndactly.
– Mental retardation.
– Retinitis pigmentosa.
 Craniopharyngioma

• Arises from remnants of Rathke's pouch

• Compresses the hypothalamus
• Suppress GnRH secretion .
• Interrupt portal flow of GnRH in the pituitary stalk.

• Calcifications may be apparent on radiography of

the sella turcica.

• Frequent manifestations include visual field defects

and blurring vision.
 Galactorrhoea + amenorrhea.

• Chiari-Frommel syndrome
–It occurs after delivery: due to
persistent Prolactin secretion.
• Delcastello syndrome:
• It is not preceded by delivery.
 Levi- Lorian Syndrome
(Pituitary infantilism)
–Amenorrhea.
–Hypogonadism.
–Short stature (Dwarfism).
 Sheehan's syndrome &Simmonds
• Postpartum hge.
• Failure of gonadotrphic function + failure of
lactation.
• More extensive damage lead to :

• Simmonds : (Destruction of the anterior pituitary gland

due to septic emboli due to puerperal sepsis.)
 Pituitary Adenoma
• Evaluation of the sella turcica with (MRI) + radiography is
necessary.

• Vary in size.
• Micro adenomas (less than 10 mm).

• Macro adenomas (more than 10 mm).

• May be associated with:

– Visual changes.
– Galactorrhoea.
– Hypothyroidism.
– Amenorrhea
 Work up for : hypothalamic- pituitary

• History
• Exam
• Investigation…
• Then:
• Categorize as primary or secondary
• Categorize cause……..
 History in primary amenorrhea
• Developmental milestones (age of growth
spurt ,age of thelarche, adrenarche)
• Chronic illness (CRI ,TB, Bl disease).
• Weight changes

• Excessive exercise
• History of anosmia
 Examination

• General condition
• Height
• BMI
• 2ndary sex characters
 Investigations
• Bed –side:
• Visual field in suspected pituitary adenoma
• Laboratory:
• BHCG: to exclude pregnancy
• Serum prolactin
• TSH
• Imaging:
– Ultrasound : prove presence or absence of uterus, measure its size
– CT
– MRI
• Instrumental:
– Hysteroscopy: uterine synechia
– Laparoscopy
2. Presence breast + absence uterus

Sexual hair &

Karyotype
46-XY 46-XX

Andogen Mullerian
Insenitivity Agenesis
(TSF (MRKH syndrome)
syndrome)
Absent sexual
hair Presence of
sexual hair
 Utero-vaginal Agenisis
Mayer- Rokitansky- Kuster-Hauser syndrome

• Normal breasts.
• N. sexual hair development .
• Normal looking 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.

• Treatment :

STERILE? Vaginal creation : Dilatation &

Vaginoplasty)
42
 Testicular feminization syndrome

• 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
– ? Vaginal creation (Vaginoplasty )

43
 Vaginal Agenesis: Comparison of Two Syndromes

Mullerian Agenesis Androgen

Insensitivity
Syndrome

Vagina absent absent

Pubic hair present absent

Breasts present Present

Gonads ovaries Testes

Uterus absent Absent

Testestrone level Female level Male level
Karyotype 46 XX 46 XY
 3. absence breast + absence
uterus
•17, 20 desmolase deficiency
•17 a hydroxylase deficiency
•Agonadism

Very rare
all are 46
Xy
 AGONADISM

• Degeneration of the
testes (in utero) after
the production of the
MIF
4. Presence breast + presence uterus (Like secondary
amenorrhea)

PREGESTERONE
BLEEDING NO BLEEDING

CHRONIC ANOVULATION COMBINED OESTROGEN

e.g PCOS & PROGESTERONE

BLEEDING NO BLEEDING

OVARIAN FAILURE UTERINE FACTOR

( Non dysgenetic) ( Ashermann syndrome)

SERUM FSH
 Summary of Sub-phenotypes Amenorrhea

Breast UterUs
absent
UterUs Present

– 17, 20 desmolase 1. Gonadal failure turner 45X

deficiency
aBsent
17 a hydroxylase Gonadal dysgenisis
deficiency 46xy
Agonadism 17 a hydroxylase deficiency with
46XX
2. Hypothalamic failure
3. Pituitary failure
General Principles of management

• Try causative Treatment.

• Do not forget general factors
• Remember stress is common cause in
adolescents
• Pregnancy is the commonest cause of
secondary amenorrhea
General Principles of management

. HRT: (estrogen and progesterone)

In hypo-estrogenic amenorrheic women (to prevent
osteoporosis)

. Periodic progestogen:
In euestrogenic amenorrheic women (to avoid endometrial cancer)
. If Y chromosome is present: gonadectomy is indicated
. Many cases require frequent re-evaluation
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