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Understanding Amenorrhea: Causes and Classifications

Learn about the definitions of primary and secondary amenorrhea, classifications based on causes, and specific conditions including Turner Syndrome and gonadal dysgenesis. This comprehensive guide covers various factors contributing to the absence of menstrual periods.

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Understanding Amenorrhea: Causes and Classifications

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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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