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AMENNORHEA

This article provides an overview of primary and secondary amenorrhea, including their definition, classification, evaluation, diagnosis, and management. It covers various causes such as gonadal dysgenesis, hypothalamic failure, androgen insensitivity, and more.

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AMENNORHEA

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  1. AMENNORHEA SALWA NEYAZI ASSISTENT PROFESSOR KSU/COSULTANT OBSTETRICIAN GYNECOLOGIST PEDIATRIC & ADOLESCENT GYNECOLOGIST

  2. OBJECTIVES • 1-Definition of 1ry and 2ry amenorroea

  3. OBJECTIVES • 2-Classification of primary amenorrhoea: Breast Uterus example ---------------------------------------------------------- A- absent present gonadal dysgenesis/ turner syndrome / Pure gonadal dysg, hypothalamic failure B-present present hypothalamic, pituitary ovarian, outflow C-present absent androgen insensitivity, mullarian agenesis D-absent absent XY karyotype enzyme deff. agonadism

  4. OBJECTIVES • 3-Evaluation and investigations • 4-Management of primary amenorroea • 5-Secondary amenorroea classification, evaluation, diagnosis and management

  5. 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

  6. CLASSIFICATION OF 1RY AMENORRHEA

  7. A-BREAST ABSENT UTERUS PRESENTGONADAL DYSGENESIS 1-TURNER SYNDROME 45XO • Variations of Turner ‘s syndrome  2-Mosaicism XO/XX  not always short • They 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

  8. TURNER SYNDROME FEATURES • 1ry amenorrhea • No breast development • Normal ♀ genital organs (external /internal) • Streak gonads (ovaries are replaced by white nonfunctioning tissue) • Short stature • Webbed neck (Short broad neck) with a low hair line • Cubitus vulgus • Shield chest / Widely spaced nipples • High arched palate • Short 4th metacarpal • Coarctation of the aorta or VSD • Horse shoe kidney or single kidney • Lymphedema

  9. 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

  10. 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

  11. HYPOTHALAMIC FILURE8-Isolated GnRH deficiency (Kalman’s Syndrome) • Anosmia & Hypogonadotropic Hypogonadism • X linked ----Mutation in the KAL gene • More 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

  12. HYPOTHALAMIC FILURE9-Hypogonadotropic Amenorrhea • CNS tumors   GnRH pulses  LH & FSH  estradiol • Hypothalamic 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

  13. HYPOTHALAMIC FILURE10-Anorexia Nervosa, Malnutrition, Excessive Exercise & Chronic Illness • Functional GnRH deficiency • May present with or without Breast development • Physical stress delay menarche • Each year of athelitic training before menarche delayed menarche 5 M • Osteoporosis could occur with prolonged periods of Amenorrhea, low body Wt

  14. 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

  15. C-BREAST PRESENT , UTERUS ABSENT1-Testicular feminization/ Androgen insensitivity • XY Karotype  produce MIF  Mullerian structures are absent • Complete/ Partial absence of androgen receptors • X linked recessive or dominant • Female external genitalia with Short blind vagina • Testosterone  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)

  16. C-BREAST PRESENT , UTERUS ABSENT2- 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 musculinization • Absent uterus • At puberty   testosterone secretion  virilization

  17. 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 present • Normal 46XX ♀ with normal exrenal genitalia • Pt present with 1ry amenorroea • 47% have asociared urinary tract anomalies • 12% skeletal anomalies • Rx  psychological counseling surgical - vaginoplasty - excision of utrine reminant (if it has fuctioning endometrium) -vaginal dilators

  18. D-BREAST ABSENT, UTERUS ABSENT • The least common presentation of 1ry Amen • All Pt are 46 XY • Testosterone   or N • FSH/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

  19. D-BREAST ABSENT, UTERUS ABSENT • B- 17 α HYDROXYLASE DEFICIENCY Similar to 17-20 desmolase def Cortisol synthesis also   ↑ BP, hypernatraemia & hypokalaemia • C-AGONADISM Degeneration of the testes (in utero) after the production of the MIF

  20. INVESTIGATIONS & TREATRMENT Hx & Physical examination to place the Pt in one of the four categories

  21. 1-BREAST ABSENT UTERUS PRESENT ↑↑FSH FSH 17α hydroxylase deficiency Kallman’s Syndrome Wt ↑Exercise Stress Wt ↑Exercise Stress Wt ↑Exercise Stress CNS / HP DISORDER Gonadal Dysgenesis ↑Na K ↑Progestrone ↑TSH ProlactinN TSHN PROLACTIN ↑ /N XX Karyotype CT / MRI HEAD Hypothyroidism XY CNS TUMORS Gonadectomy XO

  22. TREATMENT 1-BREAST ABSENT UTERUS PRESENT Hypothyroidism  Thyroxin Gonadal Dysgenesis Wt ↑Exercise Stress 17αOH-Dif Cortisol XX XO XY CNS Tmr Psychiatric Help Treat thecause Kallman’s Syndrome Gonadectomy Treat accordingly Estrogen Progestrone Replacement Estrogen Progestrone Replacement Breast development / Menses Improve Bone Min Density

  23. 2-BREAST PRESENT UTERUS PRESENT ↑ TSH Hypothyroid ↑ TSH Hypothyroid ↑Prolactin TSHN ↑ TSH Hypothyroid ↑Prolactin TSHN MRI/CT Pituitary Karyotyping Prolactin  N TSH  N Ovarian Failure ↑FSH +Progestrone chalange -Progestrone chalange Out flow Tract Obstruction Hypoth/ pituit Failure FSH MRI/CT R/O CNS TMR Anovulatory cycle

  24. TREATMENT 2-BREAST PRESENT UTERUS PRESENT ↑ TSH Hypothyroid Anovulatory cycle Ovarian Failure Hypoth/ pituit Failure Out flow Tract Obstruction ↑Prolactin TSHN Thyroxin Bromocriptin HRT Surgery Progestin D16-25

  25. 3-BREAST PRESENT UTERUS ABSENT Testosterone N♀ ↑Testosterone N♂ Karyotyping Karyotyping XX Mullerian Agenesis XY Testicular Feminization Gonadectomy U/S Pelvis U/S MRI  Gonads U/S Pelvis U/S KIDNEY IVP

  26. 3-BREAST PRESENT UTERUS ABSENT XX Mullerian Agenesis XY Testicular Feminization HRT Vaginoplasty Gonadectomy Vaginal dilators

  27. 4-BREAST ABSENT UTERUS ABSENT All 46 XY Pysical Exam U/S MRI for Gonads HRT Gonadectomy

  28. 2RY AMENORRHEA

  29. CNS / Hypothalamic Pituitary Ovarian Outflow  Uterine Cx Vaginal 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

  30. 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)

  31. 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

  32. 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 cycles • Anorexia nervosa Pt  BMD (0.64) < Normal controls (0.72) • Anorexia nervosa Pt may have osteoporotic fractures

  33. HYPOGONADOTROPIC AMENORRHEA SHEHAN’S SYNDROME • Piuitary failure  following sever post partum hemorrhage • Deficiency of all pituitary hormones • FSH & LH  Failure of ovarian follicular development  estrogen  Amenorrhea • Rx  HRT  hMG for ovulation induction

  34. 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 Wt • Functional HypoGt Amen  HRT / ovulation induction

  35. EUOGONADOTROPIC AMENORRHEA PCO • Amenorrhea / anovulatory cycles • Enlarged polycystic ovaries • Infertility • Hyperinsulinemia / Obesity • Hyperandrogenism / hirsutism • ↑ LH • Acyclic estrogen production / unopposed by progesrtrone  ↑ risk of endometrial hyperplasia/Ca • Inheritable disorder with a complex inheritance pattern

  36. TREATMENT OF PCO Infertility Amenorrhea Irrigular cycles Hyperinsulinism Obesity Hirsutism Clomid Clomid hMG Gluco phage Wt  Ovarian drilling Anti androgens Cyclic progest OCP OCP + Ovulation 70% Pregnancy 40% Ovulation 70% Pregnancy 40% Sprinolactone Cyproterone acetate Flutamide Ovarian Androgen ↑SHBG Ovulation 92% Pregnancy 70% -Protect endometrium -Regulate cycle -menorrhagia Bind androgen receptors Androgens 5αreductase activity

  37. 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%

  38. POF WHAT ARE THE PATHOLOGICAL CHCT OF POF ? TWO TYPES • Ovarian sclerosis & lack of follicles • Resistant ovary syndrome HOW TO MANAGE POF? • R/O other autoimmune diseases  RH factor ANA, Antithyroid Antibodies, Antichromosomal Antibodies, glucose, cortisol, Ca , Ph, TSH • HRT  to prevent osteoprosis • Spontaneous pregnancy can occur in women with POF on HRT 8% • hMG/HCG glucocorticoids have been cliamed to give better pregnancy rates

  39. HYPERPROLACTINEMIA • The most common pituitary cause of 2ry Amenorrhea • Causes -Pituitary adenoma -Idiopathic -Loss of inhibition by dopamine Hypothalamic or pituitary stalk lesions -Hypothyroidism -PCOS -Medications  phenothiazines , haloperidol monoamineoxidase inhibitors, TCA, H2 receptors blockers

  40. HYPERPROLACTINEMIA • Galactorrhea  1/3 of Pt • Amenorrhea/ Hyperprolactinemia Pt  at risk of osteoporosis due to  estrogen • TREATMENT - 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

  41. 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

  42. TREATMENT OF HYPERPROLACTINEMIA • IDIOPATHIC HYPERPROLACTINEMIA Rx  Dopamine agonist  Bromocriptin or Pergolide • Side effects of dopamine agonists -Postural hypotension -Nausea -Headache -Nasal stuffiness Starting with a low dose & gradually ↑ it helps to avoid The side effects

  43. ANATOMICAL CAUSES • Uncommon cause of 2ry Amenorrhea • Asherman’s Syndrome  Hx of D/C for RPOC after abortion / puerperium or previous uterine infection • Intrauterine Adhesions • Normal hormones • -ve progestrone chalange test • Dx  HSG / HYSTROSCOPY • Rx  Hystroscopic resection of the adhesions followed by estrogen therapy

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