PRIMARY AMENORRHEA

the absence of menses at age 15

PRIMARY years in the presence of normal
AMENORRHEA growth and secondary sexual
characteristics.

However, at age 13 years, if no menses have occurred and

there is a complete absence of secondary sexual
characteristics such as breast development, evaluation for
primary amenorrhea should also begin
KLASIFIKASI SEKS SEKUNDER TANNER
 TANNER STAGING
BREAST PUBIC HAIR
STAGE 1 ELEVATION OF PAPILA ONLY NO PUBIC HAIR
(PREPUBERTAL)

STAGE 2 ELEVATION OF BREAST AND SPARSE,LONG,PIGMENTED

PAPILA AS SMALL MOUND,
HAIRLY CHIEFLY ALONG LABIA
AREOLA DIAMETER ENLARGED
MAJORA,MEDIAN AGE 10,5 YRS
MEDIAN AGE:9,8 YRS
STAGE 3 FURTHER ENLARGEMENT DARK,COARSE,CURLED HAIR
WITHOUT SEPARATION OF SPARSELY SPREAD OVERMONS
BREAST AND AREOLA MEDIAN AGE 11,4 YRS
MEDIAN AGE 11,2 YRS
STAGE 4 SECONDARY MOUND OF AREOLA ADULT TIPE HAIR,ABUNDANT
AND PAPILA ABOVE THE BREAST
BUT LIMITED TO THE MONS
MEDIAN AGE 12,1 YRS
MEDIAN AGE 12,0 YRS
STAGE 5 RECESSION OF AREOLA ADULT TYPE SPREAD IN
TO CONTOUR OF BREAST QUANTITY AND DISTRIBUTION
MEDIAN AGE 14,6 YRS MEDIAN AGE 13,7 YRS
All causes of secondary However, primary
amenorrhea can also amenorrhea is usually
present as primary the result of genetic or
amenorrhea anatomic abnormalities
 Chromosomal abnormalities causing gonadal dysgenesis

The most
(ovarian insufficiency due to the premature depletion of all
oocytes and follicles) 50 percent
Hypogonadotropic hypogonadism, including functional
common hypothalamic amenorrhea 20 percent
Absence of the uterus, cervix, and/or vagina, mllerian

causes of agenesis 15 percent

Transverse vaginal septum or imperforate hymen 5
percent
primary Pituitary disease 5 percent
The etiology in the remaining 5 percent of cases includes a
amenorrhea combination of disorders, such as androgen insensitivity
due to mutations in the androgen receptor, congenital
adrenal hyperplasia, and polycystic ovary syndrome (PCOS)
 Primary amenorrhea is
evaluated by determining
the presence or absence of
a uterus, the presence or
absence of breast
development (a marker of
estrogen action and
therefore function of the
ovary, except in complete
androgen insensitivity
syndrome), and the serum
follicle-stimulating hormone
(FSH) level
Treatment of primary
amenorrhea is directed at
correcting the underlying
pathology (if possible), helping
the woman to achieve fertility (if
desired), and prevention of
complications of the disease
process (eg, estrogen
replacement to prevent
osteoporosis).
Mayer-Rokitansky-Kster-Hauser
(MRKH) syndrome
This syndrome is subdivided in two types: type
I (isolated) or Rokitansky sequence (OMIM
277000), and type II or MURCS association
(Mllerian duct aplasia, Renal dysplasia and
Cervical Somite anomalies) (OMIM 601076)
The MRKH syndrome is also referred to as
CAUV (Congenital Absence of the Uterus and
Vagina), MA (Mllerian Aplasia) or GRES
(Genital Renal Ear Syndrome)
DIAGNOSTIC CRITERIA

congenital aplasia of
the uterus and the
upper part (2/3) of the
vagina in women
showing normal
development of
secondary sexual
characteristics and a
normal 46, XX
karyotype.
 Renal (unilateral agenesis, ectopia of
kidneys or horse-shoe kidney)
Skeletal and, in particular, vertebral
(Klippel-Feil anomaly; fused vertebrae,
mainly cervical; scoliosis)
Other associated
Hearing defects
malformations include
(type II or MURCS More rarely, cardiac and digital
association): anomalies (syndactyly, polydactyly)
 Isolated utero-vaginal
aplasia is referred to as
Rokitanskybsequence or
to type I (isolated) MRKH
syndrome. Incomplete
aplasia and/or associated
with other
malformations,is
generally referred to as
MURCS association (or
type II MRKH syndrome)