PRIMARY AMENORRHOEA

Prof. M.C.Bansal
MBBS., MS., FICOG., MICOG.
Founder Principal & Controller,
Jhalawar Medical College & Hospital Jjalawar.
MGMC & Hospital , sitapura ., Jaipur
 DIFFERENTIAL DIAGNOSIS OF PRIMARY
AMENORRHOEA
A. Anatomic abnormalities of the genital outflow tract
1. Müllerian dysgenesis (Rokitansky–Küster–Hauser syndrome)
2. Distal genital tract obstruction
a. Imperforate hymen
b. Transverse vaginal septum

B. Hypergonadotropic (follicle–stimulating hormone >30

mIU/mL) hypogonadism (gonadal “failure”)
1. Gonadal dysgenesis with stigmata of Turner syndrome
2. Pure gonadal dysgenesis
a. 46,XX
b. 46,XY
3. Early gonadal “failure” with apparent normal ovarian
development
• C. Hypogonadotropic (luteinizing hormone and
follicle–stimulating hormone <10 mIU/mL)
hypogonadism
• 1. Constitutional delay
• 2. Isolated gonadotropin deficiency
• a. Associated with midline defects (Kallmann
syndrome)
• b. Independent of associated disorders
• c. Prader–Labhart–Willi syndrome
• d. Laurence–Moon–Bardet–Biedl syndrome
• e. Many other rare syndromes
• 3. Associated with multiple hormone deficiencies
• 4. Neoplasms of the hypothalamic–pituitary area
• a. Craniopharyngiomas
• b. Pituitary adenomas
• c. Other
• 5. Infiltrative processes (Langerhans cell–type
histiocytosis)

• 6. After irradiation of the central nervous system

• 7. Severe chronic illnesses with malnutrition

• 8. Anorexia nervosa and related disorders

• 9. Severe hypothalamic amenorrhea (rare)

• 10. Antidopaminergic and gonadotropin–releasing

hormone–inhibiting drugs(especially psychotropic
agents, opiates)
• 11. Primary hypothyroidism
• 12. Cushing syndrome
• 13. Use of chemotherapeutic (especially alkylating)
agents
• II. Asynchronous pubertal development
• A. Complete androgen insensitivity syndrome (testicular
feminization)
• B. Incomplete androgen insensitivity syndrome
DISCUSSION
 Central signals Peripheral
HYPOTH signals
GABA, ALAMUS
NPY,GLUTAMATE Leptin, Ghrelin

Kisspeptin-
GPR54 system

GnRH

ANT. PITUITARY

FSH LH
FSH LH

OVARY

GRAN THE

Aromatisation
Androgen
of androgens
production

ESTRADIOL
INHIBIN

Follicular growth
Mid cycle LH peak
• WHO divides patients into groups based on endogenous
oestrogen production, follicle-stimulating hormone
(FSH) levels, prolactin levels, and hypothalamic-pituitary
dysfunction.
• This classification is a guide that eliminates several
diagnoses based on initial information. However, further
work-up is still required.
• Group I: low oestrogen, low FSH, and no hypothalamic-
pituitary pathology, leading to a diagnosis of
hypogonadotrophic hypogonadism.
• Group II: normal oestrogen, normal FSH, and normal
prolactin, leading to a diagnosis of polycystic ovary
syndrome.
• Group III: low oestrogen and high FSH, leading to a
diagnosis of gonadal failure.
APPROACH TO A CASE OF PRIMARY
AMENORRHOEA
HISTORY & CLINICAL EXAM

ASYNCHRONOUS IMMATURE MATURE SECONDARY

DEVELOPMENT SECONDARY SEXUAL SEXUAL
BREAST > PUBIC HAIR CHARACTERISTICS CHARACTERISTICS

ANDROGEN
FSH , PROLACTIN DISTAL GENITAL
INSENSITIVITY
TRACT OBSTRUCTION,
MULLERIAN AGENESIS
 FSH , PROLACTIN

HIGH FSH LOW OR NORMAL FSH HIGH PROLACTIN

KARYOTYPE PITUITARY FUNCTION CHECK T4, TSH

TESTING
SELLAR X-RAY
NORMAL HIGH TSH
NORMAL ABNORMAL
NORMAL ABNORMAL TSH

MRI OR CT
• 46, XX • 45,XX OR •CONSTITUIONAL
GONADAL 46,XY DELAY
HYPOTHYROIDISM
DYSGENE • MOSAIC •ISOLATED
SIS GONADAL GONADOTROPIN
• PREMATU DYSGENES DEFICIENCY
RE IS •MALNUTRITION
• HYPOPITUITARISM
OVARIAN •CHRONIC
• CNS TUMOR
FAILURE ILLNESS