INVESTIGA

TING A
CASE OF
PRIMARY
AMENORR
PRESENTED BY- DR.SHAIK AFRAH NAAZ
HEA3 YEAR PGT
rd

DEPT OF OBSTETRICS AND

GYNAECOLOGY
GUIDE- DR.G PRASANNA
DEFINITIONS:
Primary Amenorrhea(Incidence-approx 2.5%)
• Absence of menses by age 15 years.
• Absence of breast development or menses by age 13 years.
Secondary Amenorrhea(Incidence from 3% to 100%-conditions of extreme
physical exercise or emotional stress.
• Ceasation of established menses for more than three months (regular menstrual
cycles) or six months in who had irregular menses.

ALL WOMEN WITH AMENORRHEA SHOULD HAVE A THOROUGH

EVALUATION THAT BEGINS WITH A PREGNANCY TEST!
https://www.fogsi.org/analysing-amenorrhea
ETIOLOGY
• Primary amenorrhea - a genetic or anatomical abnormality.
• Secondary amenorrhea can also present as primary amenorrhea.
• The most common etiologies are:
I. Gonadal dysgenesis, including Turner syndrome – 43 %
II. Mullerian agenesis (absence of vagina, sometimes with absence
of uterus) – 15%
III. Physiological delay of puberty (constitutional delay of puberty,
chronic systemic disease, acute illness) – 14 %
IV. Polycystic ovary syndrome (PCOS) – 7 %
I. Isolated gonadotropin-releasing hormone (GnRH) deficiency – 5 %
II. Transverse vaginal septum – 3 %
III. Weight loss/anorexia nervosa – 2 %
IV. Hypopituitarism – 2 %
• The least common etiologies (≤1 % each)
 Imperforate hymen,
Complete androgen insensitivity syndrome,
 Hyperprolactinemia/prolactinoma, other pituitary tumors,
Congenital adrenal hyperplasia,
Hypothyroidism,
Central nervous system defects, craniopharyngioma, and Cushing's disease.
https://www.uptodate.com/contents/evaluation-and-
management-of-primary-amenorrhea?2021
AMENORRHEA IS A SYMPTOM NOT A
DISEASE.

The final diagnosis should be a pathological diagnosis.

DIAGNOSTIC WORKUP

• History

• Physical examination

• Imaging studies

• Hormonal evaluation

• Karyotyping
History
• Developmental milestones(age of growth spurt, age of thelarche and adenarche)
• Any symptoms of hyperandrogenism (acne, hirsutism) or virilization
• Cyclic symptoms of menstruation
• History of Anosmia, Galactorrhea, Drugs
• Weight changes, Excessive exercise
• Symptoms of other hypothalamic-pituitary disease, including headaches, visual
field defects, fatigue, or polyuria and polydipsia
• Neonatal and childhood health normal? Neonatal crisis suggests congenital
adrenal hyperplasia.
• Family history(delayed or absent puberty/short stature)
Physical Examination

Height,Weight, Blood pressure.

Breast development/Pubic
and axillary hair: Tanner staging
Arm span/upper segment and
lower segment ratio
Features of Turner's syndrome
Tanner Staging
• Stage 1: Prepubertal.
• Stage 2: Breast bud stage with elevation of breast and papilla;
enlargement of areola.
• Stage 3: Further enlargement of breast and areola; no separation of
their contour.
• Stage 4: Areola and papilla form a secondary mound above level of
breast.
• Stage 5: Mature stage with projection of papilla only, related to
recession of areola.
Per Abdominal Examination

Abdominal mass(Cryptomenorrhea)
Inguinal hernia, gonad in labia/inguinal region(AIS)

Per Vaginal Examination

External genitalia(Pubic hair)
Clitoromegaly/signs of virilization
Depth of vagina/bulging bluish membrane.
•Imaging studies
USG: Presence/absence of uterus
HSG/SIS : Uterine synechiae
CT/MRI : Hypothalamic/pituitary cause.
•Hormonal evaluation
LH, FSH, Total T
Serum prolactin
TSH.
•Karyotyping
MRKH-46XX
Turner's-45XO
AIS-46XY
Gonadal dysgenesis-46XX, 46XY
Initial Approach
• Presence or absence of breast development (a marker of estrogen
action and therefore function of the ovary)

• Presence or absence of the uterus (by ultrasound, or in more complex

cases by magnetic resonance imaging [MRI])

• Follicle-stimulating hormone (FSH) level

http://dx.doi.org/10.18203/2320-1770.ijrcog20174981-2017
 Treatment of Amenorrhea
Address the underlying causes!
Mullerian Agenesis
Involves creation of neovagina.
• Nonsurgical creation of a vagina using serial vaginal dilators
(Franks/Ingrams)
• McIndoe procedure involves the creation of a cavity by dissection
between the urethra and bladder anteriorly and the perineal body
and rectum posteriorly. The cavity is lined by a split-thickness skin
graft overlying a plastic or soft silicone mold.
• Laparoscopic vaginoplasty (Vecchietti procedure)
https://www.fogsi.org/analysing-amenorrhea
Cont..

• PITUITARY TUMOR ® Cabergoline/Bromocriptine/Surgery

• ANDROGEN producing tumor of ovary ® Surgery

• ANDROGEN INSENSITIVITY ® removed gonads + HRT

• TURNER’S syndrome ® HRT/possibly egg donation

• IMPERFORATE HYMEN ® surgical incision

Cont..
• THYROID disease– appropriate medical treatment
• POI – HRT/egg donation
• EATING DISORDERS referred to psychiatrist
• EXCESSIVE EXERCISE – counseling/stress management/HRT
• PCOS appropriate treatment- fertility rx/ ovarian drilling/ OCPS/
Monthly P withdrawal/weight reduction/lifestyle
• ASHERMAN’s syndrome ® breaking down adhesion+insert
IUD/uterine stent +estrogen.
 Conclusion

• The overall prognosis for amenorrhea is good.

• One must remember and reiterate the patient and her relatives that
it is usually not a life-threatening clinical event and with proper
evaluation the precise etiology can be diagnosed and treated.
THANK YOU....