amennorhea salwa neyazi cosultant obstetrician gynecologist pediatric & adolescent gynecologist
Post on 21-Dec-2015
218 Views
Preview:
TRANSCRIPT
AMENNORHEA
SALWA NEYAZICOSULTANT OBSTETRICIAN
GYNECOLOGISTPEDIATRIC & ADOLESCENT
GYNECOLOGIST
AMENORRHEA
WHAT IS 1RY AMENORRHEA? Lack of the onset of menses by the 16 Y in
a ♀ with 2ry sexual chct or by the age of 14 Y in ♀ without 2ry sexual development
WHAT IS 2RY AMENORRHEA? Cessation of menses for a period of 6
months in a ♀ who previously had initiation of menses
CLASSIFICATION OF 1RY AMENORRHEA
A-BREAST ABSENT UTERUS PRESENT GONADAL DYSGENESIS
1-TURNER SYNDROME 45XOVariations of Turner ‘s syndrome
2-Mosaicism XO/XX not always shortThey will have menses , get pregnant then develop premature menopause
3-Structural abnormalities of the X chromosome Deletion of the short arm of the X chromosome
Short stature Deletion of the long arm normal HT, 2ry
Amen, streak gonads
TURNER SYNDROME
FEATURES1ry amenorrhea No breast development Normal ♀♀ genital organs (external /internal)Streak gonads (ovaries are replaced by white nonfunctioning tissue)Short statureWebbed neck (Short broad neck) with a low hair lineCubitus vulgusShield chest / Widely spaced nipplesHigh arched palateShort 4th metacarpalCoarctation of the aorta or VSDHorse shoe kidney or single kidneyLymphedema
GONADAL DYSGENESIS
4-Pure gonadal dysgenesis 46XX Mutation in an autosomal gene
Accelerated germ cell loss Streak gonads
♀ genetalia , normal Mullerian structures
Rarely Turner’s Stigmata 5- Pure gonadal dysgenesis 46 XY ♀ genitalia
Streak gonads ↑ risk of malignancy
N Mullerian structures
GONADAL DYSGENESIS
6- 17-α hydroxylase deficiency (rare) ovarian synthesis of estrogens 1ry Amen
Sexual immaturity
cortisol ↑ ACTH
↑ Na K ↑ BP
↑ Progestrone as it is not converted to cortisol
7-Galactosaemia (rare) galactosaemia is toxic to oocytes
HYPOTHALAMIC FILURE8-Isolated GnRH deficiency (Kalman’s
Syndrome)
Anosmia & Hypogonadotropic Hypogonadism X linked ----Mutation in the KAL geneMore common in ♂ > ♀
Midline defects Cleft lip & Palate
Somatic defects color blindness, renal agenesis, retinitis pigmentosa, neurosensory
deafness
Lack 2ry sexual chct & the ability to smell
HT & bone age appropriate for age
HYPOTHALAMIC FILURE9-Hypogonadotropic Amenorrhea
CNS tumors GnRH pulses LH & FSH estradiolHypothalamic Lesions Craniopharyngioma
granuloma, aqueduct stenosis , & the sequelae of encephalitis CNS tr interfere with the –ve feedback of
Dopamine on Prolactin ↑ Prolactin Other causes of HypoGonadotropic Amen hypothyroidism
Prader Willi & Laurence Moon Biedl syndromes
HYPOTHALAMIC FILURE10-Anorexia Nervosa, Malnutrition,
Excessive Exercise & Chronic Illness
Functional GnRH deficiency May present with or without Breast developmentPhysical stress delay menarcheEach year of athelitic training before menarche delayed menarche 5 MOsteoporosis could occur with prolonged periods of Amenorrhea, low body Wt
B-BREAST PRESENT , UTERUS PRESENT
1-HYPOTHALAMIC CAUSES CNS lesions (tumors) Stress, Excessive exercise & low body Wt
2-PITUITARY CAUSES Hyperprolactinemia Hypothyroidism ↑ TRH ↑ prolactin
3-OVARIAN CAUSES PCO
4-OUTFLOW TRACT OBSTRUCTION Imperforate hymen Transverse vaginal septum
C-BREAST PRESENT , UTERUS ABSENT1-Testicular feminization/ Androgen
insensitivity
XY Karotype produce MIF Mullerian structures are absentComplete/ Partial absence of androgen receptorsX linked recessive or dominant Female external genitalia with Short blind vaginaTestosterone normal ♂ range
Breast development due to periferal conversion of androgens to estrogens
Sexual hair is absent due to absence of androgen receptors
Gonadectomy after puberty ↑ risk of malignancy (gonadoblastoma, dysgerminoma)
C-BREAST PRESENT , UTERUS ABSENT
2- 5 α reductase deficiency
Autosomal recessive Formation of the ♂ external genitalia requiers
5α REDUCTASE testosterone dihydrotestosterone
Formation of the internal wollfiane structures respond directly to testosterone
External genitalia ♀ with mild musculinizationAbsent uterusAt puberty testosterone secretion virilization
C-BREAST PRESENT , UTERUS ABSENT3-Mulerian Agenesis/ Mayer –Rokitansky-
Kuster-Huser syndrome
Etiology ?Failure of mullerian duct development absence of the upper vagina, cx & uterus (uterine reminants may be found)The ovaries & fallopian tubes are presentNormal 46XX ♀ with normal exrenal genitaliaPt present with 1ry amenorroea47% have asociared urinary tract anomalies12% skeletal anomaliesRx psychological counseling
surgical - vaginoplasty- vaginoplasty - excision of utrine reminant (if it
has fuctioning endometrium) -vaginal dilators
D-BREAST ABSENT, UTERUS ABSENT
The least common presentation of 1ry AmenAll Pt are 46 XYTestosterone or NFSH/LH ↑
A- 17-20 DESMOLASE DEFICIENCY The enzyme required for the synthesis of
Androgens Androgens estrogen The testes produce MIF therefore no Mullerian structures ♀ external genitalia Insufecient estrogens for breast development
D-BREAST ABSENT, UTERUS ABSENT
B- 17 α HYDROXYLASE DEFICIENCYSimilar to 17-20 desmolase defCortisol synthesis also ↑ BP,
hypernatraemia & hypokalaemia
C-AGONADISM
Degeneration of the testes (in utero) after the production of the MIF
INVESTIGATIONS & TREATRMENT
Hx & Physical examination to place the Pt in one of the four
categories
CNS / HPDISORDER
Gonadal Dysgenesis
FSH ↑↑FSH
Karyotype
XY
Gonadectomy
XX
↑Na K↑Progestrone
17α hydroxylasedeficiency
XO
CT / MRIHEADCNS
TUMORS
↑TSH
ProlactinN
Wt ↑Exercise
Stress
1-BREAST ABSENT UTERUS PRESENT
TSHNPROLACTIN ↑ /N
Hypothyroidism
Kallman’sSyndrome
Wt ↑Exercise
Stress
Wt ↑Exercise
Stress
TREATMENT1-BREAST ABSENT UTERUS PRESENT
Gonadal Dysgenesis
XY
XX XO
Gonadectomy
Estrogen Progestrone
Replacement
Breast development / MensesImprove Bone Min Density
17αOH-DifCortisol
Kallman’sSyndrome
Estrogen Progestrone
Replacement
Wt ↑Exercise
Stress
PsychiatricHelp
Treat thecause
Hypothyroidism
Thyroxin
CNS Tmr
Treat accordingly
↑ProlactinTSHN
MRI/CTPituitary
Prolactin NTSH N
+Progestronechalange
Anovulatorycycle
-Progestrone chalange
FSH
Hypoth/ pituit Failure
↑FSHOvarianFailure
MRI/CTR/O
CNS TMR
Karyotyping
↑ TSHHypothyroid
2-BREAST PRESENT UTERUS PRESENT
Out flowTract
Obstruction
↑ TSHHypothyroid
↑ProlactinTSHN
↑ TSHHypothyroid
TREATMENT2-BREAST PRESENT UTERUS PRESENT
Hypoth/ pituit Failure
OvarianFailure
Out flowTract
Obstruction
↑ProlactinTSHN
↑ TSHHypothyroid Anovulatory
cycle
HRT
ProgestinD16-25
Bromocriptin
Surgery
Thyroxin
Testosterone N♀
3-BREAST PRESENT UTERUS ABSENT
↑Testosterone N♂
Karyotyping
XYTesticular
Feminization
Gonadectomy
Karyotyping
XXMullerian Agenesis
U/S PelvisU/S MRI Gonads
U/S PelvisU/S KIDNEY
IVP
3-BREAST PRESENT UTERUS ABSENT
XYTesticular
Feminization
XXMullerian Agenesis
Vaginoplasty
Vaginal dilatorsGonadectomy
HRT
4-BREAST ABSENT UTERUS ABSENT
All46 XY
Pysical ExamU/S
MRI forGonads
Gonadectomy HRT
2RY AMENORRHEA
2RY AMENORRHEA
WHAT IS 2RY AMENORRHEA?Cessation of menses for a period of 6 months or 3
consecutive menstrual cycles in a ♀ who previously had initiation of menses
WHAT IS THE PREVELANCE OF AMENORRHEA?
1.8-3%
WHAT IS THE CLASSIFICATON OF 2RY AMENORRHEA?
Hypergonadotropic
Hypogonadotrpic
Euogonadotrpic
Hperprolactinemia
Anatomic defects
CNS / HypothalamicPituitaryOvarianOutflow Uterine Cx Vaginal
HYPOGONADOTROPIC AMENORRHEA
“CNS / HYPOTHALAMIC ”Stress ↑ β-endorphins GnRH
FSH LH Estrogens
Exercise Excessive streneous exercise Runners & Ballet dancers
Mechanism Similar to stress Wt loss “Anorexia nervosa” More frequent in adolescent & young adults
0.5-1% of women aged 15 –30 years 15% < Ideal body Wt
Functional “Non of the above causes” No LH pulses or Persistant pulse frequency of “luteal phase ”
2ry to neurotransmitter abnormality of the CNS (? ↑ Opioid activity)
HYPOGONADOTROPIC AMENORRHEA
IS IT OF ANY CONCERN IF THESE YOUNG WOMEN BECOME AMENORRHEIC ?HYPOESTROGENISM is the main concern
WHY IS IT MORE WORRYING THAN THE MENOPAUSAL
WOMEN ?During adolescence estrogen plays a critical role in
determining PEAK BONE DENSITY which reached in the 2nd decade of life
HYPOGONADOTROPIC AMENORRHEA
IS THERE ANY EVIDENCE OF ITS EFFECT ON THE BONES?Amenorrheic Athletes Bone Mineral Density (BMD) in lumbar spines, femur, tibia Athletes with menstrual irregularities BMD <
athletes with regular cyclesAnorexia nervosa Pt BMD (0.64) < Normal controls (0.72)Anorexia nervosa Pt may have osteoporotic fractures
HYPOGONADOTROPIC AMENORRHEA
SHEHAN’S SYNDROMEPiuitary failure following sever post partum hemorrhageDeficiency of all pituitary hormonesFSH & LH Failure of ovarian follicular development
estrogen AmenorrheaRx HRT
hMG for ovulation induction
TREATMENT OF HYPOGONADOTROPIC
AMENORRHEA In training intensity to a level where regular menses resume HRT Cyclic estrogen / progestrone
Premarin 1.25 mg continuously Medroxyprogestrone acetate 5 mg /D for 12 D each cycle OCP better compliance
Anorexia nervosa Psychiatric Rx Meanwhile HRT Long term follow up Frequent relapses after
attaining ideal body WtFunctional HypoGt Amen HRT / ovulation induction
EUOGONADOTROPIC AMENORRHEA
PCOAmenorrhea / anovulatory cyclesEnlarged polycystic ovariesInfertilityHyperinsulinemia / ObesityHyperandrogenism / hirsutism↑ LH
Acyclic estrogen production / unopposed by progesrtrone ↑ risk of endometrial hyperplasia/Ca
Inheritable disorder with a complex inheritance pattern
TREATMENT OF PCO
AmenorrheaIrrigular cycles Hirsutism
Infertility
OCP OCPAnti
androgensCyclic
progest
-Protectendometrium
-Regulate cycle-menorrhagia
OvarianAndrogen↑SHBG
SprinolactoneCyproterone acetate
Flutamide
Bind androgen receptorsAndrogens
5αreductase activity
+
Clomid
hMG Ovariandrilling
Ovulation 70%Pregnancy 40%
Ovulation 92% Pregnancy 70%
HyperinsulinismObesity
Glucophage
Wt
Clomid
Ovulation 70%Pregnancy 40%
HYPERGONADOTROPIC AMENORRHEA
WHAT IS PREMATURE OVARIAN FAILURE (POF) ?2ry Amenorrhea ↑ FSH & LH
estrogen
Before the age of 40 Y
WHAT IS THE INCIDENCE OF POF ?
1%
WHAT IS THE CAUSE?
Unknown / autoimmune / genetic factors
Associated autoimmune disease 39%
POF
WHAT ARE THE PATHOLOGICAL CHCT OF POF ?TWO TYPES
Ovarian sclerosis & lack of folliclesResistant ovary syndrome
HOW TO MANAGE POF?R/O other autoimmune diseases RH factor
ANA, Antithyroid Antibodies, Antichromosomal Antibodies, glucose, cortisol, Ca , Ph, TSHHRT to prevent osteoprosisSpontaneous pregnancy can occur in women with
POF on HRT 8%hMG/HCG glucocorticoids have been cliamed to give
better pregnancy rates
HYPERPROLACTINEMIA
The most common pituitary cause of 2ry AmenorrheaCauses
-Pituitary adenoma -Idiopathic -Loss of inhibition by dopamine Hypothalamic or pituitary stalk lesions -Hypothyroidism -PCOS -Medications phenothiazines , haloperidol monoamineoxidase inhibitors, TCA, H2 receptors blockers
HYPERPROLACTINEMIA
Galactorrhea 1/3 of Pt Amenorrhea/ Hyperprolactinemia Pt at risk of
osteoporosis due to estrogenTREATMENT
- Hypothyroidism L-Thyroxin If still amenorrheic after RX Parlodel +
Thuroxin -If no substitute for the medications that
cause hyperprolactinemia HRT -Hypothalamic or pituitary stalk lesions Surgical excision
TREATMENT OF HYPERPROLACTINEMIA
PITUITARY ADENOMA (PROLACTINOMA) *Macroadenoma > 10 mm Respond to
medical Rx Dopamine agonist (bromocriptin) size of the tumor & prolactin level
Pt not responding to medical Rx or
not tolerating it Surgery/ Irradiation
*Microadenoma < 10mm remain stable in size Rx Bromocriptin prolactin level Normalize the menstrual
cycle
TREATMENT OF HYPERPROLACTINEMIA
IDIOPATHIC HYPERPROLACTINEMIA Rx Dopamine agonist Bromocriptin or
PergolideSide effects of dopamine agonists
-Postural hypotension -Nausea -Headache -Nasal stuffinessStarting with a low dose & gradually ↑ it helps to
avoid
The side effects
ANATOMICAL CAUSES
Uncommon cause of 2ry AmenorrheaAsherman’s Syndrome Hx of D/C for RPOC after abortion / puerperium or previous uterine infectionIntrauterine AdhesionsNormal hormones-ve progestrone chalange testDx HSG / HYSTROSCOPYRx Hystroscopic resection of the adhesions followed by estrogen therapy
top related