The document provides an in-depth overview of amenorrhea, its classifications, causes, and mechanisms, including both primary and secondary forms. It discusses the hormonal interplay involved in menstruation, the diagnostic workup for amenorrhea, and specific syndromes such as Turner syndrome and Mayer-Rokitansky-Küster-Hauser syndrome that can lead to primary amenorrhea. Additionally, it covers the assessment and management strategies for various types of amenorrhea.

1. Amenorrhea
• DR Manal Behery
• Professor OB&GYNE
• Zagazig University
• 2014

2. Amenorrhea
Absence Of Menstruation.
ORIGIN from Greek

3. Classification of amenorrhea
AMENORRHEAAMENORRHEA
PHYSIOLOGICALPHYSIOLOGICAL PATHOLOGICAL
Pre-puberty
Pregnancy related
Menopause
Primary
Secondary

4. Events of Puberty
•1-Thelarche :
• the breast development
• Requires estrogenestrogen
•2-Pubarche/adrenarche :
pubic hair development
Requires androgensandrogens

5. Stage1 stage2
Prepupertal: elevation of
papilla- no pubic hair
Elevation of breast and papilla
on a small mount,increase in
areola – libial hair
Marshall and tunner staging

6. Stage 3 stage4
Further breast enlargment,
hair extend to mons pubis
Secondary mound of areola
and papilla ,hair extend
laterl
And increases pigmention

7. Stage5
Recession of arola to contour of the breast
Adult pubertal hair with striaght upper
borded

8. Requires:
GnRHGnRH from the hypothalamus
FSHFSH and LHLH from the pituitary
Estrogen and progesteroneEstrogen and progesterone from
the ovaries
Normal outflow tract
Events Of Puberty
3-Menarche:

9. Hypothalamo-Pituitary-Ov-Ut Axis
CNS
Hypothalamus
Pituitary
Ovary
Uterus
Outflow tract

10. The Hypothalamic-Pituitary-Ovarian Axis
http://www.shen-nong.com/eng/images/exam/missedperiods/img_mp1

11. CNS-hypothalamus-pituitary
ovary-uterus interaction
Neural control Chemical control
Dopamine
(-)
Norepinephrine
(+)
Endorphins
(-)
Hypothalamus
GnRH
Ant. pituitary
FSH, LH
Ovaries
Uterus
ProgesteroneEstrogen
Menses
–± ?

12. Functional menses
Compartment ICompartment I
outflow tract ( uterine target
organ(
Compartment ICompartment III
Ovary ( Estrogen -Progesterone(
Compartment IIICompartment III
Anterior Pituitary
Compartment IVCompartment IV
CNS ( Hypothalamic(

13. Clinically
PrimaryPrimary SecondarySecondary

14. Common causes of Amenorrhea
Primary
• Gonadal failure (45%)
• Congenital absence of uterus and vagina (20%)
• Constitutional delay (15%)
Secondary
Chronic anovulation (40%)
• Hypothyroidism / hyperprolactinemia (20%)
• Weight loss/anorexia (16%)

15. Constitutional pubertal delay
• Common cause (15%)
Positive family history
• Under stature and delayed bone
age ( X-ray Wrist joint)
•• Diagnosis by exclusion and
follow up
• Prognosis is good(late developer)
• No drug therapy is required –
Reassurance (? HRT)

16. primary amenorrhea

17. Evaluation Categories
• 1-Breast Absent – Uterus Present
• 2-Breast Present – Uterus Absent
• 3-Breast Present – Uterus Present
• 4-Breast Absent – Uterus Absent

18. 46XX

19. Typical features of Turner SyndromeTurner Syndrome
1st commen cause (45% of causes )1st commen cause (45% of causes )

20. Turner's S.

22. •A craniopharyngioma is a benign tumor that
develops near the pituitary gland.
•most commonly in childhood and adolescence
and
•in later adult life.
compresses the pituitary stalk or gland, the tumor
can cause partial or complete pituitary hormone de
ficiency.

23. • Family history: Consider watchful waiting
• Request: FSH, LH
- Raised: Karyotype: 45 XO Turner syn
46 XX Premature ovarian failure
- Low: Constitutional delay
Consider: anorexia
exercise
illness
coeliac disease
hypothalamic/pituitary
- Intermediate: Anatomical - ultrasound
Secondary sexual characteristics
absent 14y

24. Mayer-R (okitansky-Kuster-Hauser
syndrome(

25. Utero-vaginal Agenisis
Mayer-Rokitansky-Kuster-Hauser syndrome
• Second most common cause of Primary
amenorrhea.
• Normal breasts and Sexual Hair Normal
looking external female genitalia
• Karyotype 46-XX
• 15-30% renal abnormalities.
• Treatment : Vaginal creation
(Dilatation VS Vaginoplasty)

26. • Normal breasts but no sexual hair
• Normal looking female external genitalia
• Absent uterus and upper vagina
• Karyotype 46, XY
• Male range testosterone level
Androgen insensitivity
Testicular feminization syndrome

27. •Absent/abnormal then karyotype:
-46XX Mullerian agenesis
-46XY Androgen insensitivity
•Present
•+no outflow obstruction
-As for 2o
amenorrhoea
Secondary sexual characteristics
Present by 16 years
Ultrasound uterus

28. 1-Rule out pregnancy!

29. 2-Exclude cryptomenohrea

30. ✴1-Pregnancy
✴2-Cryptomenorrhea:
imperforated hymen, vaginal septum,
✴3-Causes 2nd ry Amenorrhea:
hypothalamic, pituitary ,other endocrionpathy

31. Very rare
17,20 -desmolase deficincy
agonadieisim
17 alfa-hydroxylase deficincy (46, XY )

32. Compartment I: Disorders of the Outflow
Tract
• Eugonadism

33. MRI: complete low
transverse septum with
obstruction
MRI: complete slightly
higher transverse septum
with obstruction

35. Compartment II: Disorders of the Ovary
• Hypergonadotropic
hypogonadism

36. Compartment III: Disorders of
the Anterior Pituitary
• Hypogonadotropic hypogonadism

37. Microadenoma vs
macroadenoma

38. Compartment IV: Hypothalamic
(Kallmann’s syndrome)
• Hypogonadotropic
hypogonadism
• Congenital disorder characterized
by:
• 1) Anosmia or hyposmia
• 2) Primary amenorrhea
• Caused by defect in synthesis
and/or release of gonadorelin (LH
releasing hormone)

39. History and physical examination completed for
a patient with primary amenorrhea
Secondary sexual characteristics present
No Yes
Measure FSH and LH levels
Uterus absent
or abnormal
Uterus present
or normal
Karyotype analysis Outflow obstruction
FSH and LH
< 5 IU/ L
Hypogonadotropic
hypogonadism
Hypergonadotropic
hypogonadism
Karyotype analysis 46,
XY
46,
XX
Androgen
Sensitivity
Syndrome
No
Yes
Evaluate
for
secondary
amenorrhea
Imperforat
e hymen or
transverse
vaginal
septum
Perform ultrasonography of uterus
Evaluation of Primary Amenorrhea
FSH > 20 IU/ L
and
LH > 40 IU/ L

40. Secondary amenorrhea

41. In women of reproductive age,
pregnancy is the most common
cause of secondary amenorrhea.
Pregnancy
The reality of this must be
ascertained before any
intervention is instituted for non-
obstetric amenorrhea.

42. Compartment I OCompartment I Outflow tract
( uterine target organ)
pregnancy
Asherman's Syndrome

43. Compartment IICompartment II Ovary
 PCO
Premature Ovarian Failure
 Resistance Ovarian Syndrome
Radiation & Chemotherapy .
)Hypergonadotropic Hypogonadism(

44. Polycystic ovary syndrome
• The most common cause of chronic anovulation

45. Classic 45-XO Premature ovarian failure
Turner’s syndrome
Mosaic (46-XX / 45-XO)

46. Compartment IIICompartment III Anterior Pituitary
Hyper – prolactinemia
Tumors
 Sheehan Syndrome

47. Compartment IVCompartment IV CNS ( Hypothalamic )
 Hypothyrodism
 Stress anxiety
 Anorexia
 Excessive Exercise
 Drugs

48. Anorexia Nervosa
Anorexia nervosa
• A psychological disease
characterized by
• Intense fear of gaining weight or being fat,
despite being underweight
• Disturbance in one’s experience of body
weight, size, and shape
• the refusal to maintain normal body weight,
and amenorrhea

49. V. Others
1.Thyroid
hypo or hyperthyroidism
2. Adrenal disease
Adrenogenital S
Cushing S
Addison s disease
3. Ch. illness, badly controlled DM.

50. HistoryHistory
A good history can reveal the etiologic diagnosisA good history can reveal the etiologic diagnosis
in up to 85% of cases of amenorrhea.in up to 85% of cases of amenorrhea.
ASSESSMENTASSESSMENT

51. Present History
1.Sexual activity, risk of pregnancy,
2.Type of contraceptive used.
3.Galactorrhoea
4.Androgenic symptoms: weight gain, acne, hirsutism
5.Menopausal symptoms: night sweats, hot flushes
6.Issues with eating or excessive exercise.

52. Past history
1.Drug use: Dopamine antagonists for psychiatric conditions.
Antihypertensive,anticonsulsionvant
Genital tract surgery: intrauterine instrumentation
3--radio or chmotherrapy
4-infection : mumps or TB oophritis

53. Clinical assessment
1-β - hCG
2-TSH
3-Prolactin
TOP
Bottom

54. 1. Provera 10 mg PO once daily 7-10
days or
2. Norethindrone 5 mg PO once daily for
7-10 days or
3. Progesterone 200 mg IM for one dose .
Progesterone Challenge Test:

55. Step 1

56. 1. Premarin 1.25 mg orally daily for 21 days
2. Oral Contraceptive for 2 Cycles
3. Estradiol 2 mg orally daily for 21 days and
Follow with 7-10 days of Progesterone
Estrogen progesterone challenge
test

57. Step 2

58. • Asharman syndrome(intrauterine
synechea)

59. Step 3

60. Treatment
I. Treatment of the cause.
1-if Y chromosome is present gonadectomy is
indicated
2- Create outflow tract or at least a sexually functional
vagina
II. No obvious cause:
. Conception is not required: COCP.
. Conception is required: induction of ovulation

61. Thanks