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7/27/2019 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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Cont
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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Cont
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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Cont
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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Cont
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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Cont
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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Cont
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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Cont
(-)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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Cont
(+)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).