Clinical Manifestations Of Some Ovarian Hormonal Disorders: Ovarian Failure And Amenorrhea

Ovarian dysfunction manifests clinically as amenorrhea, infantilism or sexual precocity. In primary ovarian failure, the levels of pituitary gonadotropins are high. Ovarian failure may be due to primary disease of the ovaries in 60% and extra-ovarian causes in 40% of cases. For proper development and functioning of the ovaries, the essential requirements are:

1. Presence of two genetically active X-chromosomes,
2. Absence of Y-chromosomes,
3. Endowement of germ cells (Oogonia),
4. Development of germ cells into primary oocytes, and
5. Adequate gonadotropin stimulation in fetal life.

Abnormalities of any or all of these factors lead to ovarian failure.

Ovarian Failure

Primary ovarian failure: the following are the causes of primary ovarian failure

1. Dysgenesis or agenesis of the ovaries.
2. Resistant ovary syndrome- the ovaries are resistant to the action of pituitary gonadotropins even though they are morphologically and genetically normal.
3. Polycystic ovaries.
4. Prepubertal infections like tuberculosis and mumps which give rise to oophoritis.
5. Systemic diseases like B thalasemia, mucopolysaccharidoses, dystrophia myotonica, and autoimmune disorders such as primary Addison’s disease.
6. Female pseudohermaphroditism.
7. Isolated enzyme deficiencies, e.g. 17-alpha hydroxylase deficiency, 21-hydroxylase deficiency.
8. Genetic disorders e.g,: Turner’s syndrome.

Secondary ovarian failure: The following are the causes of secondary ovarian failure.

1. Panhypopituitarism
2. Prolactinomas- prolactin reduces ovarian response to FSH and LH and pituitary response to LHRH.
3. Acromegaly
4. Isolated gonadotropin deficiency may occur in some cases.

Primary amenorrhea is the delay of menarche beyond 18 years of age and secondary amenorrhea denotes cessation of menstruation for 6 months or more in normally menstruating women.

Gynecological causes: Diseases of the endometrium, obstruction to the outflow tract of the uterus, imperforate hymen.

Endocrine Causes: They are: Primary or secondary ovarian hypofunction and other endocrine disorders like Cushing’s syndrome, adrenogenital syndrome, Addison’s disease and thyrotoxicosis.

Systemic diseases: Disorders such as chronic liver disease, renal disease, diabetes mellitus and malnutrition can cause amenorrhea.

Amenorrhea due to drugs: Several drugs which include hormonal and nonhormonal preparations are capable of producing amenorrhea, e.g. corticosteroids, androgens, estrogens, contraceptive pills, psychotropic drugs, hypotensives, etc.

Hypothalamic causes: Anorexia nervosa, psychiatric disorders, hypothalamic tumours and developmental neurologic defects such as Kallmann syndrome cause secondary ovarian failure.

Diagnosis

The most common physiological cause of secondary amenorrhea in the young is pregnancy. This should be excluded in all cases. Other common causes include menopause, drug therapy, systemic illnesses and psychiatric disturbances. Signs and symptoms of estrogen deficiency such as vasomotor instability and drying up of vaginal secretions should suggest the possibility of primary ovarian failure or menopause. The investigations and management of amenorrhea are the responsibilities of the gynecologist.