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AMENNORHEA

AMENNORHEA. Dr. Hazem Al-Mandeel, M.D Assistant Professor & Consultant Obstetrics & Gynecology College of Medicine King Saud University OB/GYN Rotation-course 481 GYN. Definition of Amenorrhea. 1ry Amenorrhea: is the lack of onset of menses

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AMENNORHEA

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  1. AMENNORHEA Dr. Hazem Al-Mandeel, M.D Assistant Professor & Consultant Obstetrics & Gynecology College of Medicine King Saud University OB/GYN Rotation-course 481 GYN

  2. Definition of Amenorrhea • 1ry Amenorrhea: is the lack of onset of menses • by age 16 in female with 2ry sexual characteristics OR • by age of 14 in female without 2ry sexual development • 2ry Amenorrhea: is the cessation of menses for a period of 6 months in a female who previously had initiation of menses

  3. Classification of 1ry Amenorrhea Gonadal Failure 43% Congenital Absence of the vagina 14% Constitutional delay 10%

  4. Classification of 2ry Amenorrhea Chronic anovulation 39% Hypothyroidisim/Hyperprolactinemia 20% Weight loss/anorexia 16%

  5. UterusAbsent Primary Sexual characteristics HCG + 5 -Reductase def. 17-20 Desmolase def. 17 Hydroxylase Def. (All XY Karyotype) Pregnancy Absent Present HCG -ve PE No Primary Secondary R/O Asherman’s Cx Stenosis Normal • Karyotype • XX • Y line • Turner (XO) Yes High FSH PE MullerianAbn AIS True Hemaphrodite Normal Normal • Kallman’s Syndrome • Physiologic Delay TSH, PRL, FSH

  6. TSH, PRL, FSH HypoThyroidism HyperThyroidism TSH Abnormal Normal Prolactin High Hyperprolactenemia Normal • Ovarian Failure • Radiation • Chemo • Infection • Autoimune • Galacrosemia • Idiopathic FSH High Normal Normal Low Estrogen CNS Exam PCOS Idiopathic Ovarian Neoplasms Obesity Cushing’s CAH Chronic Disease • Pituitary Hypothalamic Lesions: • Tumors • Infection • Infarction • Failure • Toxic

  7. BREAST ABSENT UTERUS PRESENT:GONADALDYSGENESIS 1-TURNER SYNDROME: 45XO 2-Mosaicism of Turner’s: 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 Amenorrhea, & streak gonads

  8. TURNER SYNDROME • 1ry amenorrhea • No breast development • Normal ♀ genital organs (external /internal) • Streak gonads (ovaries are replaced by nonfunctioning tissue) • Short stature • Webbed neck (Short broad neck) with a low hair line • Cubitusvulgus • 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: continued 4-Pure gonadaldysgenesis : 46XX, mutation in an autosomal gene  accelerated germ cell loss  streak gonads. Features: ♀ genetalia with normal mullerian structures 5- Pure gonadaldysgenesis : 46 XY. Features: normal♀ genitalia and mullerian structureswith streak gonads  ↑ risk of malignancy

  10. GONADAL DYSGENESIS: continued 6- 17-αhydroxylase 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 Failure: Kalman’s Syndrome • Anosmia & HypogonadotropicHypogonadism • 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 FILUREHypogonadotropic Amenorrhea • CNS tumors   GnRH pulses  LH & FSH   estradiol • Hypothalamic Lesions  Craniopharyngioma granuloma, aqueduct stenosis , & encephalitis • CNS tumors  interfere with the –ve feedback of Dopamine on Prolactin  ↑ Prolactin • Other causes of HypoGonadotropic Amen  hypothyroidism

  13. HYPOTHALAMIC FILUREAnorexia 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. BREAST PRESENT , UTERUS PRESENT • HYPOTHALAMIC CAUSES: CNS lesions (tumors) Stress, Excessive exercise & low body Wt • PITUITARY CAUSES: Hyperprolactinemia Hypothyroidism  ↑ TRH  ↑ prolactin • OVARIAN CAUSES: Polycystic ovarian disease/syndrome • OUTFLOW TRACT OBSTRUCTION: Imperforate hymen Transverse vaginal septum

  15. BREAST PRESENT , UTERUS ABSENTTesticular 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 peripheral conversion of androgens to estrogens • Sexual hair is absent due to absence of androgen receptors • Gonadectomy after puberty  ↑ risk of malignancy (gonadoblastoma, dysgerminoma)

  16. BREAST PRESENT , UTERUS ABSENT5 αreductase deficiency • Autosomal recessive • Formation of the ♂ external genitalia requires 5α REDUCTASE testosterone     dihydrotestosterone • Formation of the internal wolfiane structures respond directly to testosterone • External genitalia ♀ with mild musculinization • Absent uterus • At puberty   testosterone secretion  virilization

  17. BREAST PRESENT , UTERUS ABSENTMulerian 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 treatments: vaginoplasty, excision of utrinereminant (if it has fuctioningendometrium)

  18. BREAST ABSENT, UTERUS ABSENT • The least common presentation of 1ry Amenorrhea • All patients are 46 XY • Testosterone   or N • FSH/LH  ↑ • 17-20 DESMOLASE DEFICIENCY The enzyme required for the synthesis of Androgens   androgens   estrogen The testes produce MIF therefore no mullerian structures ♀ external genitalia Insufficient estrogens for breast development

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

  20. 2RY AMENORRHEA

  21. 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

  22. HYPOGONADOTROPIC AMENORRHEA “CNS / HYPOTHALAMIC ” • Stress  ↑ β-endorphins  GnRH   FSH  LH   Estrogens • Exercise  Excessive streneous exercise  e.g.: runners 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 persistent pulse frequency of “Luteal Phase ” 2ry to neurotransmitter abnormality of the CNS (? ↑ Opioid activity)

  23. 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

  24. 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 but less than athletes with regular cycles • Anorexia nervosa Pt  BMD (0.64) • Anorexia nervosa Pt may have osteoporotic fractures

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

  26. TREATMENT OF HYPOGONADOTROPIC AMENORRHEA •  In training intensity to a level where regular menses resume • HRT  Cyclic estrogen / progestrone; e.g.: Premarin continuously + Medroxyprogestrone acetate for 12 days  OCP  better compliance • Anorexia nervosa  Psychiatric Rx + HRT Long term follow up  Frequent relapses after attaining ideal body wt. • Functional Amennorhea  HRT / ovulation induction

  27. EUOGONADOTROPIC AMENORRHEA Polycystic Ovarian Disease / Syndrome • 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

  28. HYPERGONADOTROPIC AMENORRHEA WHAT IS PREMATURE OVARIAN FAILURE (POF) ? • 2ry Amenorrhea • ↑ FSH & LH •  Estrogen • Before the age of 40 years WHAT IS THE INCIDENCE OF POF ? • 1% WHAT IS THE CAUSE? • Unknown / autoimmune / genetic factors • Associated autoimmune disease 39%

  29. Premature Ovarian Failure (POF) WHAT ARE THE PATHOLOGICAL CHARACTERISTICS OF POF ? • 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 8% of women with POF on HRT

  30. 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

  31. 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

  32. 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 to normalize the menstrual cycle

  33. 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

  34. 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 • Negative progestrone chalange test • Dx  HSG / HYSTROSCOPY • Rx  Hystroscopic resection of the adhesions followed by estrogen therapy

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