Evaluation and management of a patient with amenorrhea is common in
gynecology, and the prevalence of pathologic amenorrhea ranges from 3 to 4
percent in reproductive-aged populations (Bachmann, 1982; Pettersson, 1973).
Amenorrhea is diagnosed in a female: (1) who has not menstruated by age 14
years and who lacks other evidence of pubertal development; (2) who has not
menstruated by age 16, even in the presence of other pubertal signs; or (3) who
has previously menstruated but has been without menses for a time equivalent to
a total of three previous cycles or 6 months. Although amenorrhea has
classically been defined as primary (no prior menses) or secondary (cessation
of menses), this distinction may lead to diagnostic error and should be avoided.
In some circumstances, evaluation reasonably may be initiated despite
the absence of these strict criteria. Examples include a patient with the
stigmata of Turner syndrome, obvious virilization, or a history of uterine
curettage. An evaluation for delayed puberty should also be considered before
the ages just listed if the patient or her parents are concerned. Although the
list of possible etiologies is extensive, most causes will fall into a limited
number of categories. Of course, amenorrhea is a normal state prior to puberty,
during pregnancy and lactation, and following the menopause.
Numerous classification systems for the diagnosis of amenorrhea have
been developed, and all have their strengths and weaknesses. One useful scheme
is outlined in. This is system divides the causes of amenorrhea into anatomic
versus hormonal etiologies with further division into inherited versus acquired
disorders.
As described earlier, normal menses require adequate ovarian production
of steroid hormones. Decreased ovarian function (hypogonadism) may result
either from a lack of stimulation by the gonadotropins (hypogonadotropic
hypogonadism) or from primary failure of the ovary (hypergonadotropic
hypogonadism). A number of disorders are associated with relatively normal LH
and FSH levels (eugonadotropic), however, there is loss of appropriate
cyclicity.
Anatomic abnormalities that may present as amenorrhea can broadly be
viewed as either inherited or acquired disorders of the outflow tract (uterus,
cervix, vagina, and introitus).
Amenorrhea will be observed in the presence of an imperforate hymen
(1 in 2000 women), a transverse vaginal septum (1 in 70,000 women), or isolated
atresia of the vagina (Banerjee, 1999; Parazzini, 1990; Reid, 2000). Patients
with these anomalies have a 46,XX karyotype, female secondary sexual
characteristics, and normal ovarian function. Therefore, the amount of uterine
bleeding is normal, but its normal path for egress is obstructed or absent. Th
ese patients may note moliminal symptoms, such as breast tenderness, food
cravings, and mood changes, which are attributable to elevated progesterone
levels. In addition, accumulation of menstrual blood behind an obstruction
frequently results in cyclic abdominal pain. In women with outflow tract obstruction,
an increase in retrograde menstruation may lead to development of endometriosis
with associated complications such as chronic pain and infertility.
Postoperative scarring and stenosis of the cervix may follow dilatation
and curettage (D&C), cervical conization, loop electro surgical excision
procedures, infection, and neoplasia. Severe atrophic or radiation changes can
also be causative. Stenosis most commonly involves the internal os, and
symptoms in menstruating women include amenorrhea, abnormal bleeding,
dysmenorrhea, and infertility. Postmenopausal women are usually asymptomatic
until fluid, exudates, or blood accumulates. If obstruction is complete, a
soft, enlarged uterus is palpable.
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