Amenorrhea (1)

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    Amenorrhea

    Khalid A. Yarouf

    4MedStudents.com

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    Outline

    Definitions.

    Hx.

    P/E.Clinical approach to 1 amenorrhea.

    Clinical approach to 2 amenorrhea.

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    Definitions

    1 Amenorrhea:

    = No menses by age 14 + absence of 2 sexualcharacteristics.

    = No menses by age 16 + presence of 2 sexualcharacteristics.

    2 Amenorrhea:

    = No menses for 3 months if previous menseswere regular.

    = No menses for 6 months if previous menses

    were irregular

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    Hx:

    Obstetric Hx: Gravidity, parity.

    Gyne Hx: regularity of periods, duration,

    dysmenorrhea, menorrhagia, LMP.

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    P / E:

    Tanner staging.

    Breast present ?

    Uterus present ?PV exam.

    Rule out possibility of pregnancy.

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    Clinical approach to 1

    amenorrhea:No 2 sexual characteristics:

    Clinical findings:

    Absence of 2 sexual characteristics (e.g.

    breasts) must result from inadequateestrogen. Possible causes are:

    1. Gonadal Hyper-gonadotropic

    hypogonadism:

    Pathophysiology: Normal hypothalamic-

    pituitary axis (indicated by FSH), butend organ is unresponsive (absence of

    ovarian follicles no estrogen).

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    Cause is gonadal dysgenesis:

    Commonest cause of 1 amenorrhea (30%).

    Causes: Turners synd (46,X), structurally

    abnormal X chromosome, mosaicism with /

    without Y chromosome, pure gonadal

    dysgenesis (46,XX & 46,XY).

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    Cont

    2. CentralHypo-gonadotropic

    hypogonadism:

    Pathophysiology:

    a. Failure of GnRH secretion from

    hypothalamus:

    Many pts with amenorrhea also have anosmia

    (Kallmanns synd).

    b. Failure of FSH secretion from anterior

    pituitary.

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    Cont

    Causes: CNS tumor, craniopharyngioma

    FSH .

    Dx:

    FSH differentiates between gonadal & central

    causes.

    Karyotype is very useful as well.

    Brain CT / MRI to rule out a tumor in case

    of central cause.

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    Mx:

    In both conditions, give estrogen

    stimulate 2 sexual development.Cyclic progestins

    prevent endometrial hyperplasia.

    FSH Karyotype Dx

    45,X Gonadal dysgenesis

    46,XX Hypothalamic-pituitaryinsufficiency

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    2 sexual characteristics (e.g. breasts) arepresent:

    Adequate estrogen must be produced by gonads

    to stimulate breast development. Genotype isnormal 46,XX in most cases.

    Causes:

    1. Intact hymen.

    2. Transverse obstructing vaginal septum.3. Cervical agenesis: rare.

    4. Uterine absence.

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    Cont

    4. Mllarian agenesis:

    Idiopathic failure of mllarian ducts to descend

    into pelvis to form upper genital tract. Pts

    usually have bilateral rudimentary uterineanlagen, Fallopian tubes & ovaries.

    20% of cases of 1 amenorrhea.

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    Dx Testosterone level & karyotype

    should be obtained.

    Testosterone Karyotype Dx

    @ normal

    levels

    46,XX Mllarian

    agenesis

    @ male

    levels

    36,XY Androgen

    insensitivity

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    Cont

    Mx:

    Neovagina may need to be created. Its

    effective in allowing normal vaginal

    intercourse.

    Breasts developed, but no pubic and

    axillary hair 10% of cases of 1 amenorrhea.

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    Present with evidence of gonadal secretion (breast

    development) but no manifestation of androgen

    secretion reflects absence of androgen

    receptors (complete androgen insensitivity synd=testicular feminization synd is misnomer).

    Genotype is 46,XY. The Y chromosome has led to

    production of Mllarian Inhibitory Factor (MIF),

    hence pts have only vaginal dimple & no uterus ortubes.

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    Testes, which are often intra-abdominal,produce normal male levels of testosterone.Breast development is due to enzymatic

    conversion of testosterone to estrogen.Mx:

    Gonadal resection once puberty is complete.

    Creation of neovagina when pt is prepared to besexually active.

    Psychotherapy.

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    Clinical approach to 2

    amenorrhea -hCG level should be obtained:

    rule out pregnancy (commonest cause of 2

    amenorrhea).

    Progesterone challenge to assess

    estrogen status.

    Medroxy-progesterone acetate 10 mg OD

    X 1 week look for withdrawal bleeding:

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    Cont

    (+)ve test if any bleeding occurs within 2-7days always due to anovulation.

    Do S-Prolactin & TSH rule out correctable

    cause.e.g. pituitary prolactinomas / hypothyroidism.

    Mx: Treat underlying cause.

    Periodic cyclic progestins prevent endometrialhyperplasia from unopposed estrogen.

    Ovulation induction with Clomiphene citrate if pregnancy is desired.

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    (-)ve test if no bleeding occurs:

    hypo-estrogenism / outflow tractobstruction.

    Combined Estrogen-Progesterone ChallengeTest (EPCT) clarifies etiology of amenorrhea.

    EPCT should be administered to seewhether withdrawal bleeding occurs:

    Conjugated estrogen 1.25 mg PO for 21 daysfollowed by medroxy-progesterone acetate 10mg PO X 1 week.

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    (+)ve if any bleeding occurs within2-7 days

    always due to lack of estrogen.

    FSH level should be obtained todistinguish between hypothalamic-pituitary failure ( FSH) or ovarian

    failure ( FSH). In the former case,brain imaging should be obtained torule out a tumor.

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    Cont

    Estrogen should be provided to preventsequelae of estrogen deficiency, alongwith cyclic progestins to prevent

    endometrial hyperplasia, regardless ofthe specific cause.

    (-)ve test if no bleeding occurs: always due to outflow tract obstruction.

    Mx: Obtain hystero-salpingo-gram (HSG). identify site of obstruction (e.g. cervical stenosis).

    rule out endometrial adhesions (Ashermans synd).