v Classification:

• Primary amenorrhea:

? No menses by 16 y/o with presence of 2^o sexual characteristic, or

‚ No visible 2^o sexual characteristic by 14 y/o

• Secondary amenorrhea:

Absence of menstruation for three normal menstrual cycles or 6 months

　

v Work-up for Amenorrhea:

• see figure 24.1, p 810
• in summary:

? Hx: G?P?, LMP?, OBS/GYN Hx, medical diseases, operation Hx, etc.

‚ PE, PV, and/or PR:

2^o sexual characteristic (breast, pubic hair, axillary hair, etc.)

uterus, cervix, vagina, external genitalia, etc.

ƒ Lab. Survey: EIA, FSH, TSH, PRL, E2, etc.

„ Image Study: ultrasound, HSG, CT, MRI,etc.

　

v Amenorrhea without Secondary Sexual Characteristic:

• Causes

Abnormal Physical Examination:

5a -reductase deficiency in XY individual

17-20 desmolase deficiency in XY individual (rare)

17a -hydroxylase deficiency in XY individual

Hypergonadotropic Hypogonadism:

(disorders of ovaries, either genetic origin, enzymatic origin, or other origin)

Gonadal dysgenesis (eg. Turner’s syndrome): the most common cause

Pure gonadal dysgenesis

Partial deletion of X chromosome

Sex chromosome mosaicism

Environmental and therapeutic ovarian toxins

17a -hydroxylase deficiency in XX individual

Galactosemia

Others

Hypogonadotropic Hypogonadism:

(disorders of hypothalamic origin or pituitary origin)

Physical delay: the most common cause

Kallman’s syndrome: insufficient pulsatile secretion of GnRH

Central nervous system tumors: most commonly, craniopharyngioma

Hypothalamic/pituitary dysfunction

• Diagnosis

1. The FSH level differentiates hypergonadotropic and hypogronadotropic forms of hypogonadism.
2. See figure 24.1

• Treatment

1. Generally, estrogen and progestin

? cyclic:

premarin (0.625) 0.625 mg qd ´ 25 ds

provera (5) 5-10 mg qd ´ 12-14 ds

‚ continuous

2. for patients with 17a -hydroxylase deficiency:

Add corticosteroid replacement

3. Specific considerations:

`

v Amenorrhea with Secondary Sexual Characteristic

• Causes and Diagnosis

Mullerian anomalies:

Imperforate hymen: bulging membrane during valsalva maneuver

Transverse vaginal septum

Mayer-Rokitansky-Kuster-Hauser syndrome:

ie. hypoplasia or absence of the uterus, cervix, and or vagina

PE, ultrasound or MRI, IVP (to detect concomitant renal anomaly)

Androgen insensitivity:

male pseudohermaphrodites, 46XY

absent pubic hair and axillary hair

True hermaphrodites: Both male and female gonadal tissue is present.

Absent endometrium

Asherman’s syndrome: diagnosed by HSG or hysteroscopy

Secondary to prior uterine or cervical surgery

Currettage, especially postpartum

Cone biopsy

Loop electroexcision procedure

Secondary to infections

Pelvic inflammatory disease

IUD-related

Tuberculosis endometrial

Schistosomiasis culture

• Treatment

1. Imperforate hymen: a cruciate incision to open the vaginal orifice
2. Transverse septum: surgical removal
3. Hypoplasia or absence of the cervixa: still no effective way to correct
4. Absent or short vagina:

progressive dilation, if fails, then McIndoe split thickness graft technique

5. Complete androgen insensitivity: bilateral laparoscopic gonadectomy
6. Asherman’s syndrome:

Lysis of adhesion, IUD insertion (or pediatric Foley) 7-10 days post-operatively,

Systemic broad-spectrum antibiotics, 2-month course of high-dose estrogen

　

v Amenorrhea with Secondary Sexual Characteristic and Nonanatomic Causes:

• Causes

1. Pregnancy
2. Thyroid dysfuction and hyperprolactinemia
3. Ovarian Failure:

Genetic disorders: mosaicism of an XO or XY cell line, deletion of a portion of

X chromosome, 47XXX.

Iatrogenic causes: radiation, chemotherapy, surgical alteration of ovarian blood supply.

Infections

Autoimmune disorders

Galactosemia (mild form or heterozygote)

Savage syndrome: FSH receptors are absent or a postreceptor defect exists

Cigarette smoking

Idiopathic

4. Hypothalamic/pituitary lesions:

Hypothalamic/pituitary:

Craniopharynigioma

Germinoma

Tubercular granuloma

Sarcoid granuloma

Dermoid cyst

Pituitary:

Nonfunctional adenoma

Hormone-secreting ademonas:

Prolactinoma

Cushing’s disease

Acromegaly

Primary hyperthyroidism

Infarction

Lymphocytic hypophysitis

Surgical or radiological ablations

Sheehan’s syndrome: postpartum necrosis of the pituitary

Diabetic vasculitis

5. Abnormal hypothalamic GnRH secretion

• Diagnosis

1. Pregnancy test
2. Hormone status:

TSH and prolactin levels:

· If elevated TSH and prolactin level are found, the hypothyroidism

should be treat before hyperprolactinemia is treated

FSH level

Estrogen status: progesterone challenge test

· Provera (5) 5 or 10 mg for 10 days, or

· progesterone 100-200 mg im.

3. Assessment of the hypothalamus and pituitary

• Treatmemt (p828)

1. estrogen therapy: for protection against cardiac diseases and prevention of osteoporosis
2. gonadectomy: when Y cell line present
3. treatment of underlying diseases
4. treatment for hirsutism:

oral contraceptives: decreasing ovarian androgen production,

increasing circulating levels of sex hormone-binding globulin

spironolactone: decreasing androgen production,

competing with androgen at the androgen-receptor level

5. for conception: ® ovulation induction

Clomiphene citrate:

The usual first choice

Effective only in patients with adequate levels of estrogen, FSH, and PRL

Contraindications: pregnancy, liver disease, preexisting ovarian cysts.

Dosage: Clomiphene 50 mg qd since D5 for 5 days

HMG:

When clomiphene fails, or hypogonadotropic hypoestrogenic anovulation

GnRH:

Effective in patients with low level of estrogen and gonadotropins