Amenorrhea

Khalid A. Yarouf

4MedStudents.com

Outline
Definitions.

Hx.
P/E.

Clinical approach to 1 amenorrhea.

Clinical approach to 2 amenorrhea.

Definitions
1 Amenorrhea:

= No menses by age 14 + absence of 2 sexual characteristics. = No menses by age 16 + presence of 2 sexual characteristics.
2 Amenorrhea:

= No menses for 3 months if previous menses were 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.

P / E:
Tanner staging.

Breast present ?
Uterus present ?

PV exam.
Rule out possibility of pregnancy.

Clinical approach to 1 amenorrhea:

No 2 sexual characteristics: Clinical findings:
Absence of 2 sexual characteristics (e.g. breasts) must result from inadequate estrogen. Possible causes are:

1. Gonadal Hyper-gonadotropic

hypogonadism: Pathophysiology: Normal hypothalamicpituitary axis (indicated by FSH), but end organ is unresponsive (absence of ovarian follicles no estrogen). 6

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).

Cont
2. Central Hypo-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.

Cont
FSH Karyotype 45,X 46,XX Dx Gonadal dysgenesis Hypothalamic-pituitary insufficiency

Mx: In both conditions, give estrogen stimulate 2 sexual development. Cyclic progestins prevent endometrial hyperplasia.

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Cont
2 sexual characteristics (e.g. breasts) are present: Adequate estrogen must be produced by gonads to stimulate breast development. Genotype is normal 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 uterine anlagen, 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 46,XX levels

@ male levels 36,XY

Mllarian agenesis
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 or tubes.

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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 be sexually 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 2-7

days 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 endometrial hyperplasia 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 tract obstruction. Combined Estrogen-Progesterone Challenge Test (EPCT) clarifies etiology of amenorrhea. EPCT should be administered to see

whether withdrawal bleeding occurs:

Conjugated estrogen 1.25 mg PO for 21 days followed by medroxy-progesterone acetate 10 mg PO X 1 week.
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Cont
(+)ve if any bleeding occurs within 2-7 days
always due to lack of estrogen. FSH level should be obtained to distinguish between hypothalamicpituitary failure ( FSH) or ovarian failure ( FSH). In the former case, brain imaging should be obtained to rule out a tumor.
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Cont
Estrogen should be provided to prevent sequelae of estrogen deficiency, along with cyclic progestins to prevent endometrial hyperplasia, regardless of the 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).

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