Menstrual Disorders

Amenorrhoea

Amenorrhoea is the absence of menstruation; it is a symptom, not a disease in itself and is often physiological, as in pregnancy.

Primary amenorrhea refers to a patient of any age who has never menstruated.

Secondary amenorrhea refers to cession of the periods after menstruation has been established.

Cryptomenorrhoea is sometimes used where menstruation is occurring, but is concealed because the vagina is occluded by a congenital septum or atresia.

Amenorrhea may be classified as:

Physiological

¨1before puberty

¨2during pregnancy

¨3during lactation

¨4after the menopause

Pathological

¨5uterine lesions

¨6ovarian lesions

¨7pituitary disorders

¨8disorders of other endocrine glands

¨9psychiatric illness and emotional stress

¨10severe general illness

¨11drugs

¨12following surgical operations or radiotherapy.

Uterine Lesions

In general uterine causes of amenorrhoea are rare. Amenorrhoea occurs when the uterus is rudimentary and only represented by a nodule of fibromuscular tissue at the top of the vagina. In such cases the vagina may also be absent, but the ovaries and the secondary sexual characteristics are usually normal.

Ovarian Lesions

¨13Primary amenorrhoea occurs with ovarian dysgenesis.

¨14Secondary amenorrhoea may be caused by failure of the ovarian enzyme systems necessary for the production of oestrogens.

¨15There is a spectrum of such disorders, of which the polycystic ovary (Stein- Leventhal) syndrome is the one most commonly recognized. In this disorder, secondary amenorrhoea is associated with bilateral enlargement of the ovaries, which contain multiple small follicular cysts in a dense stroma.

¨16Many of these patients are obese, and have an excessive growth of facial and body hair.

¨17The secretion of FSH is within normal range and some oestrogen is produced, but not enough to cause uterine bleeding.

¨18Surgical treatment is not now usually employed, because ovulation and menstruation can usually be induced with clomiphene.

Pituitary disorders

¨19Pituitary infantilism

Rare cases of pituitary infantilism (Levi-Lorain syndrome) occur; the cause is unknown. The patients are of child-like stature and proportions, with primary amenorrhoea. Secretion of FSH is absent or low, and no oestrogens are found in the urine.

Ischaemic necrosis of the pituitary gland

This was originally described by Simmonds, but is often known as Sheehan’s disease. It is the result of thrombosis of the pituitary blood vessels after profound hypotension and hypovolaemia, most commonly caused by severe postpartum haemorrhage.

The production of gonadotrophic, thyrotrophic and adrenotrophic hormones ceases, or is very inadequate. The patients are

Þ lethargic,

Þ gain weight and

Þ have a low metabolic rate,

Þ hypotension and

Þ amenorrhoea.

Treatment with hormones is disappointing. Thyroxine and cortisone are usually given. Oestrogens may produce cyclical bleeding, but this can be of psychological benefit.

Adenomata of the anterior lobe of the pituitary gland (prolactinomata)

This may cause an excessive secretion of prolactin and consequent amenorrhoea. The tumour may be very small and difficult to demonstrate by radiography. Prolactin release is increased by hypothyroidism. Stress can cause a temporary increase in secretion of prolactin. Galactorrhoea can occur.

If a tumour is found it is treated by radiotherapy or surgery, but if no tumor is evident bromcriptine will inhibit the output of prolactin.

Disorders of Other Endocrine Glands

Þ Adrenal gland

The adrenogenital syndrome is caused by a tumour or hyperplasia of the adrenal cortex. There is excessive production of androgens and the urinary excretion of oxosteriods is increased. The symptoms and signs are those of virilism, with deepening of the voice, hirsuties, acne, amenorrhoea and enlargement of the clitoris.

In Cushing’s syndrome, hyperplasia (or less commonly a tumor) of the adrenal cortex produces an excess of glucocorticoids which stimulate the conversion of protein into carbohydrate. These patients have amenorrhoea, hypertension, polycythaemia, osteoporosis and diabetes; the abdomen often shows striae like those of pregnancy.

Amenorrhoea occurs in advanced cases of Addison’s disease when adrenal tissue is deficient.

Þ Thyroid gland

Amenorrhoea may occur in either myxoedema or hyperthyroidism, if they are severe.

Þ Pancreas

Amenorrhoea may occur in severe or badly controlled diabetes.

Psychiatric Illness And

Emotional Stress

The hypothalamus controls the output of gonadotrophins from the pituitary gland, and higher centres in the brain affect the functions of the hypothalamus.

¨ Starting new job,

¨ being away from home for the first time or

¨ emigration are examples of stressful conditions which may cause amenorrhoea until social readjustment has been made.

¨ Sudden bad news or

¨ severe emotional distress may have the effect.

The periods are likely to return spontaneously and reassurance is all that is required.

Amenorrhoea accompanies anorexia nervosa. The girl is often rejected the imagined burdens of maturity, and refusal of food is intended to stop progress from childhood to womanhood. Psychiatric help is necessary, and restoration of a diet with adequate proteins and vitamins.

Severe General Illness or Weight-Loss

Menstruation function may be temporarily suppressed during or after any severe illness, or chronic renal disease.

Secondary amenorrhoea occurs with starvation. This was seen in the inmates of concentration camps during psychological stress were also present. A less severe example is found in ballet dancers and other young women who strive to maintain a slight, slim figure by strict dieting. amenorrhoea is common and periods return when a more reasonable body mass is restored.

Drugs Causing Amenorrhoea

Amenorrhoea sometimes occurs after stopping oral contraception; it can continue for some months. Spontaneous return of menstruation is to be expected within 6 months, but in a few cases treatment by induction of ovulation with clomiphene or gonadotrophins is required. This type of amenorrhoea is more common in women who had irregular periods before starting oral contraception, and some of them are merely reverting to their previous menstrual pattern.

Þ The possibility that the amenorrhoea is due to pregnancy must not be forgotten.

Amenorrhoea Following Surgical Operations or Radiotherapy

Obviously, amenorrhoea will follow hysterectomy or removal of both ovaries. The ovaries may also be suppressed by pelvic irradiation for malignant disease.

Þ Primary Amenorrhoea

If a girl has not begun to menstruate by the age of 17, investigation is advised. The management of these cases calls for some discretion; to subject an embarrassed young woman to a barrage of investigations is not always helpful. Some or all of the following may be required, but the more invasive, such as examination under anaesthesia or laparoscopy, should not be made until the other tests show that they essential.

Þ Observation of stature and body form

Dwarfism may be the result of some long-standing metabolic disorder; for example, renal disease, a malabsorption syndrome or lack of growth hormone, as in pituitary infantilism.

In some cases of gonadal dysgenesis (Turner’s syndrome) the patients are of short stature. Webbing of the neck or wide carrying angles at the elbows suggest Turner’s syndrome.

Þ Examination of the breasts and pelvic examination

If breast development has taken place there must have been some oestrogenic activity, and in such cases it is possible that there is some abnormality of the lower genital tract such as haematocolpos causing cryptomenorrhoea, or a rudimentary uterus.

A special problem is testicular feminization. In these cases the breasts are well formed, but the vagina may be short and the uterus is absent. There may a family history of the disease. The diagnosis rests on the discovery that the patient is chromatin negative, XY.

Patients without breast development are likely to have gonadal dysgenesis, and for such cases chromosomal analysis is essential. There will be also absent of breast development in cases of pituitary infantilism.

Þ Hormone assays

The essential distinction to be made is between cases of hypothalamic or pituitary failure, in which the levels of both gonadotrophins and oestrogens will below, and cases in which the ovary fails to respond, when the level of gonadotrophins will be high and that of oestrogens low.

Þ Chromosomal studies

In all cases of primary amenorrhea in which the diagnosis is not clinically certain a full chromosomal analysis should be carried out.

Þ Radiological examination and CT scan

An intravenous urogram is carried out in all cases of uterine or vaginal malformation, as there are often associated abnormalities of the ureters and kidneys in these cases.

A lateral X-ray tomogram or a computed tomography (CT) scan of the sella turcica may show it to be expanded by a pituitary tumour; this examination should be made in all cases in which there is a raised prolactin level, or clinical reason to suspect such a tumour.

Þ Ultrasound

An ultrasound scan of the pelvis is useful for determining the size of ovaries, the presence of follicles and their size.

Þ Laparoscopy

laparoscopy and biopsy of the gonads is not indicated if the diagnosis can be made by less invasive methods. If the diagnosis is certain after clinical examination, perhaps with the addition of chromosomal or hormonal studies, little purpose is served by providing, in Turner’s syndrome.

In cases of testicular feminization, laparotomy is required to remove the tests after the secondary sexual characteristics have been established, to prevent the danger of dysgerminoma arising.

Ovarian dysgenesis and absence of the uterus cannot be altered, but in some girls morale can be improved by intermittent oestrogen therapy. This will cause breast enlargement and monthly withdrawal bleeding if the uterus is present. A low dose of oestrogen is given, such as ethinyloestradiol 20 micrograms daily for 21 days, with norethisterone 5 mg daily added on days 14 to 21. Alternatively an appropriate low-dose combined oral contraceptive pill can be used. After an interval of 7 days the cycle is repeated.

For delayed puberty in girls whose ovaries are shown to contain

oocytes, ovulation may be induced with human gonadotrophin.

A patient who has previously menstruated must have a patent lower genital tract, an endometrium which has responded to ovarian hormones and ovaries which have responded to ovarian hormones an ovaries which have responded to gonadotrophins. In every case pregnancy must be excluded. This done, a systematic management is:

Previous menstrual history

Inquiry about the patient’s previous menstrual history is made. If she has had irregular cycles for many years there is little point in investigating or treating a minor abnormality unless infertility is her problem.

Change in the patient’s environment

Inquiry should be made about any recent change in her social and emotional environment, any stress that she has undergone, or any attempt at severe dieting.

General medical examination

After a history of recent or long-standing illness is sought, a general examination is made (including observation of the body build, weight and hair distribution) to exclude any general illness. The breasts and pelvic organs are examined.

Any appropriate investigations such as tri-iodo-thyronine binding capacity and free thyroxine indices for suspected disorder of the thyroid, are performed. Prolactin levels are measured.