PRIMARY & SECONDARY

AMENORRHOEA

PRIMARY AMENORRHOEA
■ Definition : Absence of menstruation together
with absence of secondary sexual characteristic
by the age of 14 years or absence of
menstruation despite presence of secondary
sexual characteristics by the age of 16 years

■ Causes :
(a)Constitutional delayed puberty
(b) Outflow tract abnormalities
- Mullerian agenesis leading to absence of uterus,
vagina or both (Rokintasky syndrome)
- Transverse vaginal septum : usually due to
failure of lower 1/3 of vagina to canalize
- Androgen insensitivity (Testicular feminization
syndrome, TFS) : this is due to congenital defect
of androgen receptor despite normal circulatory
testosterone
- Imperforate hymen
- Vaginal atresia
(c) Ovarian disorder :
- Gonadal dysgenesis :
. Turner’s syndrome (45xo)
. Pure gonadal dysgenesis (46xx)
. Swyer’s syndrome (loss of germ cells
early in fetal life)
- polycystic ovarian syndrome predating
puberty

(d) Pituitary disorders :

- Pituitary infantalism (Levi-Lorain syndrome)
- Pituitary destruction by craniopharyngioma, TB,
sarcoidosis

(e) Hypothalamic disorder :

- Kallman’s syndrome (GnRH deficiency + anosmia)
- Excessive exercise : 17% of body fats by body weight are needed
for menses to occur & 20% are needed to maintain menses.
(f) Endocrine disorders :
- Hypothyroidism : Increase TRH :
increase prolactin
- Hyperthyroidism of hypothalamic origin :
increase TRH : increase prolactin
- Cushing’s syndrome
- Congenital adrenal hyperplasia

■ History
- Developmental history : head control, sitting down,
walking, talking etc
- Presence or absence of cyclical symptoms
- History of chronic illness like TB
- family history of anosmia
- Drug history e.g use of phenothiazines
■ Examination
- Height, weight, BMI : patients with Turner’s syndrome
are usually short while those with TFS are usually tall;
patients with PCOS predating puberty may have high
BMI
- Secondary sexual characteristics (breast development,
pubic & axilliary hair) : in Turner’s syndrome there is
poor breast development while in TFS the breasts are
well developed; in both Turner’s & TFS there is absent or
scanty pubic & axilliary hair
- Examine the breast for galactorrhoea
- Examine for other stigmata of dysgenesis : webbing of
neck, widely spaced nipples, wide carrying angle of
elbow, leg oedema, evidence of congenital cardiac
lesion especially coarctation of aorta
- Pelvic examination :
. Clitoral enlargement as in congenital adrenal
hyperplasia
. Patency of the vagina : in imperforate hymen &
transverse vaginal septum the vagina is not patent
. Assess the dept of vagina
. Assess for the presence of the cervix & uterus
. Examine for inguinal swelling which may be undescended testis which
is common in TFS
- Rectal examination : this can be done where V/E is not possible to
assess the presence of uterus

■ Investigations
● TH nature and extent depends on the suspected cause of the primary
amenorrhoea. They may include :
- Buccal smear & karyotype
- Serum FSH & LH
- Serum prolactin
- Thyroid function test : T3,T4, TSH, TRH
- Serum pregnanetriol & 17 hydroxyprogestreone in suspected adrenal
hyperplasia
- Serum testosterone in suspected TFS. The level will be high and in male
range
- Skull X-ray in suspected pituitary adenoma & craniopharyngioma
- CT-scan of the brain in suspected pituitary adenoma & craniopharyngioma
- Pelvic ultrasound to assess presence or absence of the uterus & ovaries
- Intravenous urography (IVU) to assess the urinary tract
for abnormalities
- Laparoscopy for direct visualization of the internal
genitilia. It also allows for gonadal biopsy.

■Treatment
●It depends on the outcome of the evaluation
- If the investigation findings are normal consider delayed
puberty & menarche. Reassure and review every 3-6
months until menarche occurs.
- Imperforate hymen & vaginal septum : excise
- Vaginal atresia : vaginoplasty
- TFS patient : should have gonadectomy to prevent the
risk of malignancy (disgerminoma)
- Endocrine disorders : should be treated accordingly :
adrenal hyperplasia (cortisol), hypothyroidism
(thyroxine),hyperprolactinaemia (dopamine agonist e.g
bromocriptine or lisuride)
- Pan-pituitarism : pituitary hormone replacement
- Hypogonadotrophic hypogonadism : give LH & FSH
injections e.g pergonal
- Polycystic ovarian syndrome : induce ovulation with
clomiphene citrate or via ovarian drilling using LASER

Prognosis
- Depends on the cause
- Good in minor disorder like imperforate hymen
- Poor in major disorders like Rokintasky syndrome & TFS.
Secondary Amenorrhoea

● Definition : absence of menstruation for at least 6 months in a woman

who had been menstruating previously
● Oligomenorrhoea : prolonged cycle length of between 5 weeks to 6
months

■Aetiology
● Physiological : pregnancy, lactation, menopause

● Pathological :
(a) Hypothalamic disorders
- Anorexia nervosa
- Stressful exercise
(b) Pituitary disorders
- Pituitary adenoma
- Sheehan’s syndrome
(c) Ovarian disorders
- Polycystic ovarian syndrome, PCOS (obesity, hirsutism, oligomenorrhoea/
amenorrhoea)
- Androgen secreting tumours of the ovary e.g androblastoma
- Premature ovarian failure (< 40 years)
 - Resistant ovarian syndrome
- Iatrogenic destruction of the ovary e.g bilateral oophorectomy &
radiotherapy
(d) Out flow tract abnormality
- Asherman’s syndrome
- TB endometritis
- Hysterectomy
(e) Other endocrine disorders
- Hypothyroidism
- Hyperthyroidism
- Cushing’s syndrome
- Addison’s disease
- Late onset adrenal hyperplasia
- Adrenal tumours producing excessive androgen
(f) Drugs
- Danazole
- Injectable contraceptives
- GnRH agonist
- Phenothiazines
- COCP (especially in pts with oligomenorrhoea before using it)
■ History
- Age of the pt, age at menarche, LMP
- Regularity of the cycle before amenorrhoea
- Presence of abnormal hair distribution
- History of thyroid and adrenal disorder
- History of galactorrhoea & visual disturbance
- History of weight loss & slimming
- Gynaecological & obstetric history including history of
D&C, hysterectomy & radiotherapy
- History of psychological or psychiatric illness
- Drug history
■ Physical Examination
- General : obesity, hirsutism, acne
- Thyroid gland enlargement. Where indicated examine for
signs of hypo or hyperthyroidism
- Examine the breast for galactorrhoea
- Examine the visual field for evidence of disturbance
which may indicate pituitary adenoma
- Examine the abdomen & pelvis to identify any abdominal
scars & presence/ size of any uterine or ovarian masses

■ Investigations
●They depend on the suspected cause and may include
- Pregnancy test
- Progesterone challenge test
- Oestrogen-progesterone challenge test
- Hysterosalpingography (HSG)
- Lateral skull X-ray
- CT-scan of pituitary fossa
- Hormonal profile : FSH, LH, prolactin, TSH,T4,T3,
cortisol, 17-ketosteriods & 17-hydroxyprogesterone
- Pelvic & abdominal ultrasound scan (polycystic ovaries
can be seen)
- Laparoscopy : stigmata of ovulation & polycystic ovaries
can be seen
- Hysteroscopy
- Endometrial biopsy when TB endometritis is suspected
■ Treatment
● Depends on the identified cause
- Pts with psychogenic or psychiatric factors : counselling
- Pts with weight loss amenorrhoea : encourage to eat and
add weight
- Those with hyperprolactinaemia : dopamine agonist e.g
bromocriptine or lusiride
- Polycystic ovarian syndrome : clomiphene citrate or
ovarian drilling
- Thyroid disorders : manage accordingly (carbamazine
for hyperthyroidism ; thyroxine for hypothyroidism)
- Adrenal hyperplasia : cortisol
- Uterine synechie (Asherman’s syndrome) : intra-uterine
adhesiolysis, insertion of Lippes loop and stimulation of
endometrial regeneration with oetrogen.