AMENORE PRIMER

Dwi Fatmala
Pembimbing,
dr. Steven Ridwan, Sp.OG

DIBAWAKAN DALAM RANGKA TUGAS KEPANITERAAN KLINIK

BAGIAN OBSETRI DAN GINEKOLOGI FAKULTAS KEDOKTERAN UNIVERSIITAS
HALUOLEO
KENDARI
2018
 BATASAN
SPEROFF ( 1994 )
I. GADIS USIA 14 TH TANDA SEKS
SEKUNDER NEG. & BLM MENARKE

II. GADIS USIA 16 TH TANDA SEKS

SEKUNDER POS. TETAPI BELUM MENARKE
 PEMBAGIAN
BRENNER PAUL F. ( 1999 )
KATEGORI PENOTIP AMENORE PRIMER
GRUP BUAH DADA UTERUS

I. TIDAK ADA
II. ADA TIDAK
III. TIDAK TIDAK
IV. ADA ADA

MISHELL DANIEL R. Jr , ATLAS OF CLINICAL GYNEKOLOGY.

KLASIFIKASI SEKS SEKUNDER : TANNER
 TANNER STAGING
BREAST PUBIC HAIR
STAGE 1 ELEVATION OF NO PUBIC HAIR
( PREPUBERTAL ) PAPILA ONLY
ELEVATION OF BREAST AND
STAGE 2 SPARSE, LONG, PIGMENTED
PAPILA AS SMALL MOUND,
HAIR CHIEFLY ALONG LABIA
AREOLA DIAMETER ENLARGED
MAJORA. MEDIAN AGE 10,5 YRS
MEDIAN AGE: 9,8 YEARS
STAGE 3 FURTHER ENLARGEMENT
DARK, COARSE, CURLED HAIR
WITHOUT SEPARATION OF
SPARSELY SPREAD OVER MONS
BREAST AND AREOLA.
MEDIAN AGE 11,4 YEARS
MEDIAN AGE: 11,2 YEARS

STAGE 4
SECONDARY MOUND OF AREOLA ADULT TYPE HAIR, ABUNDANT
AND PAPILA ABOVE THE BREAST BUT LIMITED TO THE MONS.
MEDIAN AGE: 12,1 YEARS MEDIAN AGE: 12,0 YEARS

STAGE 5
RECESSION OF AREOLA ADULT TYPE SPREAD IN
TO CONTOUR OF BREAST QUANTITY AND DISTRIBUTION.
MEDIAN AGE : 14,6 YEARS MEDIAN AGE : 13,7 YEARS
 GRUP I
( BUAH DADA NEG, UTERUS POS. )

PROD. ESTROG.
NEG.

B. HIPOTAL. – PITUITARY
A. OVARIUM GAGAL
GAGAL
HIPER - HIPOG
HIPOG - HIPOG

TINGGI PX. FSH SERUM RENDAH

 A.OVARIUM GAGAL
HIPER - HIPOG
KEL. KROMOSOM

NON ANDROGENIK ANDROGENIK

-45 XO ( S. TURNER ) -45X/ 46 XY
-46 ABNORMAL X DELESI -45X/ 46X ( Yq )
LENGAN PENDEK/ PANJ. -45X (TESTICULAR
-MOSAIK ( X/XX, X/XX/XXX, DETERMINANT POSITION )
X/XY )
-46 XX ATAU 46 XY ( MURNI
GONADAL DISGENESIS )
-46 XX DG DEF. 17 α HIDROK-
SILASE
SINDROMA TURNER
KLINIS
-PENDEK
-WEBBING OF THE NECK
-JARAK NIPLE JAUH
-UDEMA KAKI & TANGAN
-KUBITUS VALGUS
-MULTIPLE PIGMENTED
NEVI
-RECURRENT OTITIS MEDIA
-KEL. JANTUNG, GINJAL
DSB
DEF. 17 α - HIDROXYLASE

1. GENITALIA EXTERNA WANITA

2. RETENSI NATRIUM
HIPOKALEMI
HIPERTENSI

TX.
- HRT
- CORTISONE
 TEST ACTH
DARAH BASAL 60 MENIT

PERIKSA DARAH

COSYNTROPIN
0, 25MG IV INTERVAL
30 – 60 DETIK
8 – 9 PAGI
( PUASA SEMALAM )

DEF. 17 α - HIDROKSILASE BILA :

PROGESTERON
17 α - HIDROKSI PROGESTERON SEDIKIT / TETAP.
 BLOOD PRESSURE MEASUREMENT IN DIAGNOSIS
GROUP I PRIMARY AMENORHEA

SERUM FSH LEVEL

ELEVATED

HYPERGONADOTROPIC
HYPOGONADISME

BLOOD PRESSURE MEASUREMENT

NORMAL BLOOD PRESURE HYPERTENSION

GONADAL 17α- HYDROXYLASE

DYSGENESIS DEFICIENCY 46, XX

45,X
46,X/ ABNORMAL X
KARYOTYPE MOSAICISM
PURE GONADAL DYSGENESIS
45,X/ 46, XY
45,X / 45, X ( Yq )
45,X / TESTICULAR
DETERMINANT POSITION
CT OR MRI IN DIAGNOSIS OF GROUP I
PRIMARY AMENORRHEA
SERUM FSH

LOW / NORMAL

HYPOGONADOTROPIC
HYPOGONADISM

CT OR MRI SELLA TURCICA

NO LESION LESION

GnRH STIMULATION TEST

NORMAL, FSH, LH ABSENT

RESPONSE RESPONSE

HYPOTHALAMIC PITUITARY
FAILURE FAILURE
 TEST GnRH
100µg GnRH IV
BOLUS 30 “
100 µg GnRH/ HR IM 30’ 60’
1 MINGGU
DRH PX PX
BASAL LH FSH
2X TIAP 15’

-30’ LH PITUITARY
KEL.
HIPOTALAMUS BILA -60 ‘ FSH BAIK
 COMPLETE DIAGNOSTIC EVALUATION
OF WOMEN WITH GROUP I PRIMARY AMENORRHEA

LOW OR NORMAL ELEVATED

SERUM FSH
HYPOGONADROTROPIC
LEVEL HYPERGONADOTROPIC
HYPOGONADISM
HYPOGONADISM

CT OR MRI, PROLACTIN, TSH BLOOD PRESSURE

NORMAL BLD
NO LESION LESION HYPERTENSION
PRESSURE

GYNADAL 17 -HYDROX.
DYSGENESIS DEFICIENCY 46,XX
GnRH STIMULATION TEST
KARYOTIPE
NORMAL FSH, LH ABSENT
RESPONSE RESPON 45,X 45,X TESTICULAR
46,X/ ABNORMAL X DETERMINANT
MOSAICISM POSITION
HYPOTHALAMIC PITUITARY
PURE GONADAL DYSG.
FAILURE FAILURE
45,X/ 46 XY
45, X/ 45, Xi ( Yq )
 GRUP II AMENORE PRIMER
( BUAH DADA POS., UTERUS NEG )

ESTROG.
POS.
GGAN
RKH PERTUMB. TFS
ORGAN
REPROD.

RKH = MAYER ROKITANSKY KUSTER HAUSER SYNDROME

TFS = TESTICULAR FEMINIZATION SYNDROME

SKEMA PERTUMBUHAN DUKTUS MULLER
 KLASIFIKASI KELAINAN
PERTUMBUHAN DUKTUS MULLER

I. AGENESIS ( RKH )
II.GANGGUAN FUSI VERTIKAL
A. OBSTRUKSI
B. NON OBSTRUKSI
III. GANGGUAN FUSI LATERAL
A. OBSTRUKSI
B. NON OBSTRUKSI
 CLASSIFICATION OF MULLERIAN ANOMALIES

HIPOPLASIA/
AGENESIS
VAGINAL SERVICAL

FUNDAL TUBAL COMBINED

UNICORNUATE
COMMUNICATING NON COMMUNICATING

NO CAVITIY NO HORN

DIDELPHYS
CLASSIFICATION OF MULLERIAN ANOMALIES
BICORNUATE

SEPTATE

ARCUATE

DES RELATED
 RKH
DASARNYA GENOTIPE WANITA
AGENESIS DUKTUS MULER
( OVARIUM NORMAL )

KLINIS

•H – P – O  BAIK  OVULASI POS.

•TESTOSTERON “ N “ WANITA
•GANGGUAN PADA GENITALIA :
VAGINA & UTERUS
 NORMAL DEVELOPMENT OF INTERNAL
AND EXTERNAL GENITALIA
UNDIFFERENTIATED GONAD

XY
XX  OVARIAN KARYOTIPE
DEVELOPMENT TESTICULAR
DEVELPMENT

TESTOST. AND
MIS
DEHYDROTESTOST.

WOLFIAN DUCT REGRESSION T DHT

+
MULLERIAN DEVELOPMENT
OF OVIDUCTS AND UTERUS WOLFIAN DEV. OF
+ DUCT MALE EXT
FEMALE EXTERNAL GENITALIA DEV. GENITAL

MULLERIAN SEMINAL VES. LAB. SCROTAL

DUCT AND PROSTAT FUSION +
REGRESSION PHALLIC
DEV.
 PERBEDAAN RKH & TFS
PEMERIKSAAN RKH TFS
KARYOTIPING 46 XX 46 XY

HEREDITER ? MATERNAL X LINKED

RECESSIVE 25% ANAK
TERKENA 25% CARIER

RAMBUT SEKS “N” WANITA NEG. / SEDIKIT

TESTOSTERON “N” WANITA “N” / PRIA

KELAINAN LAIN SERING * JARANG

5% KEMUNGKINAN
KEGANASAN GONAD NORMAL
GANAS
SPEROFF 1994
* 1/3 PEND. ADA KEL. URINARIUS ECTOPIC KIDNEY RENAL AGENESIS
HORSE SHOE KIDNEY DSB.
* 12 % KELAINAN TULANG ( TULANG BELAKANG )
 DIFFERENTIATION OF THE CONGENITAL ABSENCE OF
THE UTERUS FROM THE ANDROGEN INSENSITIVITY SYNDROME

SERUM TESTOSTERONE &

OBSERVATION OF PUBIC AND AXILARY HAIR

NORMAL FEMALE TESTOSTERON NORMAL MALE TESTOSTERONE

LEVEL AND NORMAL PUBIC LEVEL AND ABSENT PUBIC
AND AXILARY HAIR AND AXILARY HAIR

CONGENITAL ABSENCE ANDROGEN INSENSITIVITY

OF UTERUS ( TESTICULAR FEMINIZATION )

CONFIRM OVULATION CONFIRM WITH

WITH A BBT OR WEEKLY KARYOTIPE
SERUM PROGESTERONE X 4
 GRUP III AMENORE PRIMER
( BUAH DADA & UTERUS NEG. )

A.GANGGUAN ENZIM :
DEFISIENSI DARI:
- 17.20 - DESMOLASE*
- 17α – HYDROKSILASE*
B. TESTICULAR AGONADISM.

* KARYOTIPING XY  GONADEKTOMY
EVALUATION OF WOMEN WITH GROUP III
PRIMARY AMENORRHEA

KARYOTYPE ( 46, XY)

ENZYME
AGONADISME
DEFICIENCY
 GRUP IV AMENORE PRIMER
( BUAH DADA & UTERUS POS. )

EVALUASI

SAMA DENGAN AMENORE

SEKUNDER
 GARIS BESAR EVALUASI AMENORE PRIMER
SEKSI FERTILITAS & ENDOK. REPROD. LAB
SMF OBGYN. FK UNAIR RSUD DR SOETOMO
SURABAYA
I. BD-, UT + II. BD +, UT - III. BD & UT - IV. BD & UT +
FSH TESTOST. KARYOTIPING
TINGGI RENDAH “N” WNT “N” PRIA LIHAT.
AMENORE
KEL. OV. FOTO SELLA XY SEKUNDER
RKH* TFS
KARYO KEL
TIPING VAGINO KARYO GONADEKTOMI
+ - PLASTI TIPING & HRT

Y POS. PIT. H.TAL

GONADEKT. GONADEKTOMI
& HRT
-TX G. TROPIN
-HRT *BILA ADA TANDA
HRT KLINIS ANDROGEN
CORTISONE
( BILA TENSI NAIK )  PX KARYOTIPING