Sclerocystic ovaries are considered to be ovaries, in which there are many small cysts ranging in size from wheat grain to large cherries. In this case, as a rule, both ovaries are enlarged. For the first time, the clinical picture of the disease was described by Stein and Leventhal (1935). The following set of symptoms is characteristic: bilateral ovarian enlargement due to their polycystic degeneration; menstrual irregularities (amenorrhea, sometimes followed by prolonged uterine bleeding); infertility; hirsutism; sometimes hypoplasia of the uterus, external genitalia and mammary glands; fat hormonal imbalance. Stein-Leventhal syndrome accounts for up to 3% of all gynecological diseases and up to 30% of menstrual irregularities and infertility.
Etiology and pathogenesis of Stein-Leventhal syndrome
In the development of Stein-Leventhal syndrome, an important role is played by hormonal dysfunctions of the ovaries and other endocrine glands involved in the regulation of the menstrual cycle (the papillary region, pituitary gland, adrenal glands, pineal, thyroid, thymus, pancreas, and also the nervous system).
The etiology of the disease has not yet been elucidated. According to supporters of the theory of the pituitary origin of the syndrome, the disease develops as a result of a violation of the pituitary gonadotropic function.
Most authors believe that the overproduction of FSH plays a leading role in this, as a result of which there are no cyclic changes in the ovaries and a large number of immature follicles are formed. At the same time, the activity of the inner lining of the lid of the follicles and stromal cells increases markedly, which leads to the appearance of cysts, thickening of the protein membrane and sclerotherapy of the ovarian stroma. Other proponents of this theory attach great importance to the luteinizing hormone (LH) of the pituitary gland.
Many authors believe that polycystic ovary is the result of an ovarian hyperfunction of the cortical substance of the adrenal glands (with the syndrome, hyperplasia of the cortical substance of the adrenal glands is often observed, and some clinicians have seen successful results of treatment of this disease with corticosteroid drugs when surgical treatment is ineffective).
In recent years, the pathogenesis of Stein-Leventhal syndrome has been considered from the perspective of impaired steroid synthesis in the ovary with an estrogen deficiency that changes the pituitary-ovarian balance, resulting in a violation of steroidogenesis, which leads to anovulatory cycles in the ovaries, virilization and infertility. It is believed that the defect in the synthesis of estrogen in the ovaries is congenital. Many researchers attach great attention to the development of this syndrome to various disorders of the function of the submandibular region. There are supporters of the genetic theory, according to which the sclerocystic ovary is formed as a result of ovarian dysgenesis during chromosomal abnormalities.As a result, ovaries arise in the ovaries, which after the puberty period appear as a change in steroidogenesis and increased reactivity of the primary ovarian follicles to pituitary gonadotropic hormones. According to L. I. Tychinsky, polycystic ovary develops as a result of violations in the gene apparatus that regulates the synthesis of sex hormones.
In the pathogenesis of polycystic ovaries, an important role is played by inflammatory processes in them. Typically, polycystic ovary is bilateral, observed in both women and girls. In our opinion, with this pathology, the production of FSH in the body decreases as a result of blocking it with an excessive amount of androgens formed in polycystic ovaries. In this case, the amount of androgens (sometimes corticosteroids) increases sharply. Along with this, the content of pregnandiol (a progesterone metabolite) and luteinizing hormone decreases sharply. Stein-Leventhal syndrome can develop against the background of enlarged (polycystic) and against the background of shrunken (reduced) ovaries. In the first case, the ovaries are enlarged p 3-4 times, have an ovoid shape, dense, gray in color, small cysts are located under a thick protein coat. Often, the protein membrane is less thick, as a result of which the primary ovarian sclerocystically regenerated follicles protrude on the surface of the ovaries. In the second case, the ovaries are mostly covered with a dense thick fibrous protein membrane with a completely smooth white surface. In the context of such an ovary, the follicles are in the stage of atresia, their number is reduced, they are small in size, not exceeding the size of the wheat grain. At the same time, the most persistent forms of amenorrhea, infertility are observed, and strong hair growth is noted. The thick albumen of the sclerocystic degenerated ovaries prevents the normal growth of follicles, their rupture (ovulation) and the formation of the corpus luteum. In this case, there is significant hyperplasia and hypertrophy of the inner lining of the follicle tire, which intensively produces androgens. Both in the first and in the second case, the anovulatory cycle takes place, which leads to infertility. Usually, with this pathology, structural changes in the ovaries precede a menstrual cycle disorder, most often by the type of amenorrhea, less often by hemorrhagic metropathy.
Histological picture
The ovarian capsule is 6-10 times thickened, the abundance of primary ovarian follicles and the almost complete absence of luteal tissue; ovarian stroma is hyperplastic, multiple cystically altered follicles at different stages of development, undergoing atresia. There are very few primary ovarian follicles, no mature ones. Thickening of the capsule (shell) of the ovary is the result of a high concentration of androgens in them.
Clinic and diagnosis of Stein – Leventhal syndrome
Sclerocystic ovary is usually accompanied by obesity. However, as a result of the increased content of androgens and corticosteroids in the body, a violation of the general condition is possible: headache, insomnia, lethargy, weakness, general weakness, irritability or apathy (signs of neurasthenia), decreased sexual desire. In a bimanual vaginal examination, along with a normal, slightly enlarged or reduced uterus, enlarged, dense, painless, polycystic ovaries are determined (sometimes on one side). In some cases, the ovaries on both sides are of normal size, and sometimes even less than normal (these forms of ovarian pathology are most unfavorable, because the ovaries are covered with a thick protein membrane that interferes with ovulation).
Tests of functional diagnostics (measuring basal temperature, studying colpositograms, determining the symptoms of the “pupil”, examining endometrial scrapings) indicate that the changes in the ovaries are single-phase (anovulatory cycles).
For the diagnosis of sclerocystic ovaries, the method of ultrasound ultrasound imaging, gas pelvography is used. On the pelvogram, the uterus is usually slightly smaller than in healthy women, and the ovaries are enlarged, round, oval or bean-shaped. The coincidence of the radiological data with the data obtained during the operation reaches 90-95%. The absence of ovarian enlargement on the pelvogram shows no reason for excluding the diagnosis.
In doubtful cases, laparo- and culdoscopy are used. In this case, enlarged ovaries are visible, covered with a smooth grayish-white capsule and translucent cysts of various sizes.
For the purpose of diagnosis, some laboratory tests are used (the study of the secretion and excretion of FSH, LH, LTH, estrogens, pregnandiol, androgens and 17-ketosteroids (17-KS). To exclude hyperplasia of the adrenal cortex with increased excretion of 17-KS, suprarenography is performed, and for exceptions for the pituitary tumor-X-ray of the Turkish saddle region.In spite of the presence of hirsutism (up to 70% of cases), the content of 17-KS in the daily amount of urine is often normal or slightly elevated. Hirsutism therapy uses a cortisone or prednisolone test. Within 8 days, the patient is given 50 to 100 mg of cortisone per day (or 20 mg of prednisolone for 5 days). Before and after the test (blockade of corticotropin excretion), determine the amount of 17-KS. Since the cortical substance of the adrenal glands is hyperplastic, blocking the excretion of corticotropin leads to a decrease in 17-KS excretion, but if the pathology comes from the ovaries, the cortisone test should not affect the excretion of 17-KS. For the same purpose, samples with dexamethasone and corticotropin are used.
With Stein-Leventhal syndrome, these samples do not reduce the content of 17-KS. Data on the estrogenic activity of the ovaries in this disease is extremely controversial. With opso- and amenorrhea, estrogen excretion is often reduced, and with anovulatory bleeding increased. The level of pregnanediol in daily urine is usually reduced.
Sclerocystic ovary syndrome in 80% of cases occurs during puberty and in 20% of cases in the post-puberty period.
The most common (sometimes the only) symptom is amenorrhea. As a rule, amenorrhea is preceded by normal menstrual function in adolescence and even during the first years of marriage. Then the cycle of menstruation is disturbed, the pauses between them are lengthened, opsenomenorrhea passes into amenorrhea. The duration of amenorrhea ranges from 3 months to several years. The second common clinical symptom of the syndrome is infertility. In this case, from 50 to 90% of patients suffer from primary or secondary infertility. The third characteristic symptom is hirsutism, which occurs in 50-70% of cases. There are minor (moderate) and significant hirsutism. With slight hirsutism, hair growth is observed on the upper lip, cheeks, around the breasts. With significant hirsutism, thick hair covers large areas of the limbs, trunk, face and other areas of the body. In 50-60% of cases, hirsutism is accompanied by a violation of the menstrual cycle. Despite excessive androgen production, clitoral enlargement and coarsening of the voice are extremely rare. Less persistent symptoms of the syndrome include obesity (up to 60% of cases), uterine hypoplasia (up to 50%), mammary gland hypoplasia (up to 30%). With a duration of the syndrome of ten years or more, atrophy of the uterus and mammary glands is possible.