METHODICAL INSTRUCTIONS for practical lesson « Endometriosis. Gynaecological diseases of children and teenagers. Abnormalities of location and development of female genital organs. » MODULE 3: Diseases of the Female Reproductive system. Family planning. CONTEXT MODULE 4: Endocrine disorders the female reproductive system. I. Scientific and methodical grounds of the theme Recently the problem of endometriosis has become especially actual due to the increase of frequency of this pathology, implementation of modern diagnostic and therapeutic methods in to practice that’s why the aim of the practical lecture is the study of etiopathogenesis of endometriosis, methods of its diagnostic and treatment II. Aim: A student must know: 1. Classification of endometriosis of female genitalia . 2. Main clinic symptoms for endometriosis of female genitalia. 3. Methods of diagnosis of endometriosis of female genitalia. 4. Main principles of therapy of endometriosis of female genitalia. 5. Indications for surgery of endometriosis of female genitalia. 6. Possible developmental anomalies of uterine tubes, ovaries. 7. Clinic, diagnosis and treatment infantilism. A student should be able to: 1. Collect general and specific gynecologic anamnesis. 2. Make up a plan of examination and treatment of endometriosis. III. Recommendations to the student ENDOMETRIOSIS Endometriosis involves deposits of endometrium outside the uterine cavity. Its manifestations are very variable and often bear no relation to the extent of the disease. Pathology The gross appearance shows ectopic deposits which can very in number from a few in one locality to large numbers distributed over the pelvic organs and peritoneum. The commonest sites of these deposits are: The commonest appearance of a typical lesion is that of a round protruding vesicle which shows a succession of colours from blue to black to brown. The variation in colour is due to haemorrhage with subsequent breakdown of the haemoglobin. Ultimately the area of haemorrhage heals by the formation of scar tissue. The result is a puckered area on the peritoneum. Commonly however the haemorrhage results in adhesion to surrounding structures. These adhesions are more apt to form between fixed structures such as the broad ligament, ovary, sigmoid colon or the posterior surfaces of the vagina and cervix. The ectopic deposits of endometrial tissue vary in size from pin-point to 5 mm or more. It is these larger deposits which tend to rupture leading to adhesions. These adhesions over the ovary can lead to the formation of quite large haemorrhagic cysts due to continued bleeding from deposits, the blood being unable to escape. Investigation has shown that many lesions do not have a 'typical' appearance. The following is a list of other appearances which have been described. White, slightly raised opacities due to retro-peritoneal deposits. Red flame-like or vascular swellings, more common in the broad ligament or utero-sacral ligament. Small excrescences like the surface of normal endometrium. Adhesions under the ovary or between the ovary and the ovarian fossa peritoneum. Cafe-au-lait patches often in the Pouch of Douglas, broad ligament or peritoneal surface of the bladder. Peritoneal defects on utero-sacral ligament or broad ligament. Areas of petechiae or hypervascularisation usually on the bladder and the broad ligament. Secondary pathology This is due to the adhesions between the endometriotic deposits and adjacent organs. In longstanding cases the pelvic cavity is obliterated by these adhesions. Retroversion of the uterus can be produced. Clinical findings The incidence of endometriosis has been estimated at 3 to 7% of women but the true incidence is unknown. Quite often deposits are found incidentally in women who have no symptoms of endometriosis and are undergoing laparoscopy or laparotomy for some other condition. In addition, as indicated in the section on pathology, many peritoneal changes now known to be due to endometriosis were undiagnosed in the past. The prevalence of endometriosis peaks between the ages of 30 and 45 years. Since ectopic endometrium is stimulated by the same ovarian steroid hormones as the endometrium lining the uterine cavity, endometriosis is almost never found outside the reproductive years. Symptomatology A. Pain affects more than 80% of women with endometriotic deposits. The pain tends to begin premenstrually reaching a peak during menstruation and subsiding slowly. The character of pain may vary as does its apparent origin. It may be generalised throughout the abdomen and pelvis like the pain of severe dysmenorrhoea. Alternatively, pain may be localised to a particular site within the pelvis. Deep dyspareunia affects around 40% of women with endometriosis. B. Menstrual disturbance. Menstrual disturbance affects around 20% of women with endometriosis. It may take the form of premenstrual 'spotting', menorrhagia or infrequent periods. Lesions in the wall of the bladder may result in 'menstrual haematuria'. C. Infertility. Endometriosis is found more commonly in women undergoing investigation for infertility than in the 'normal' population. It is not clear which condition arises first. Approximately 30% of patients with endometriosis complain of infertility. When endometriosis is extensive, and both fallopian tubes are occluded, the mechanism by which endometriosis prevents conception is obvious. However, milder forms of endometriosis are also associated with subfertility, and here the pathophysiology is less clear. The most likely mechanism appears to be that immunological factors within the peritoneal cavity inhibit normal gamete function, thus reducing fertilisation rates. Physical examination Endometriosis cannot be diagnosed by physical examination alone. However, enlargement of the ovaries, fixed retroversion of the uterus and tender nodules within the pelvis may each raise the suspicion of the disease. Endometriosis should always be considered when patients have symptoms referable to the pelvic cavity. Laparoscopy Laparoscopic examination is the only way of making a positive diagnosis. The lesions can be seen and their number and location estimated. Endometriosis of long standing may be very difficult to diagnose due to obliteration of the pelvic cavity by adhesions. Histological confirmation must be obtained if feasible. Imaging techniques Ultrasound, computerised tomography and magnetic resonance imaging may suggest the presence of endometriosis (e.g. by the demonstration of a particular type of ovarian cyst) but are by themselves insufficiently reliable to make the diagnosis. Differential diagnosis Due to the mixture of symptoms and the variation in appearance of the pelvic structures, conditions such as pelvic inflammatory disease and tumours of the ovary and bowel must be considered and eliminated. Histogenesis. There are three theories. Retrograde spill of menstrual debris through the tubes. Retrograde menstruation takes place in most women, but it is unclear why some women should develop endometriosis while others are unaffected. Metaplasia of embryonic cells. These are derived from the primitive coelom and may remain in and around the pelvis and differentiate into Mullerian duct tissue. Emboli of endometrial tissue may travel by lymphatics or blood vessels and become established in various sites. The first of these theories is most favoured. TREATMENT. Medical treatment. Any treatment must be aimed at treating symptoms. Since ovarian hormones are responsible for growth and activity in endometrium many medical therapies are designed to reduce ovarian steroid production or oppose their action. 1. Progestogens Progestogens in a relatively high dose (e.g. medroxyprogesterone acetate 10 mg tid) induce decidualisation, and sometimes resorption of ectopic endometrium. Side effects include weight gain, bloating and irregular vaginal bleeding. 2. Combined contraceptive pill The combined oral contraceptive pill also induces decidualisation of ectopic endometrium. It may be given continuously for up to 3 months. 3. Danazol Danazol is a steroid hormone closely related to testosterone, which inhibits pituitary gonadotrophins, is anti-oestrogenic, anti-progestational, slightly androgenic and anabolic. The dose of danazol given can be titrated to the patient's symptoms up to a maximum of 800 mg daily. If danazol can be tolerated, symptoms and objective signs of disease can be alleviated in the majority of patients. However, androgenic side effects including amenorrhoea, weight gain, acne, hirsutism and deepening of the voice may limit acceptability of the drug. 4. Gestrinone Gestrinone is a derivative of 19-nortestosterone. It has slight androgenic activity and is markedly anti-oestrogenic and anti-progestogenic. It interacts with the pituitary steroid receptors and decreases gonadotrophic secretion resulting in diminished follicular growth and anovulation. A bi-weekly oral dose of 2.5 to 5.0mg for 6 months induces amenorrhoea, disappearance of pain and regression of the endometrial deposits. Side effects include weight gain, acne, seborrhoea and mild hirsutism. Gonadotrophic releasing hormone analogues (GnRH analogue) GnRH analogues are administered by depot injection or nasal spray. Their mode of action is shown above. Although these drugs are generally effective in treating symptoms, menopausal side effects, in particular bone loss, may preclude long term use. In the future, use of 'add back' regimens which include small supplementary doses of oestrogen may prove to be effective in treating the symptoms of endometriosis without the complications of total oestrogen deprivation. Conclusion As with medical therapies for other conditions, the optimum treatment is dictated by the side effect profile which is most acceptable to the patient. None of the drug treatments described will prevent recurrence of endometriosis once therapy has been stopped, although there may be a period of some months between stopping treatment and the re-emergence of symptoms. No medical treatment has been shown to improve subsequent fertility. Notwithstanding, none of the above, with the exception of the combined pill, is a proper contraceptive agent and patients should be advised to use barrier contraception to avoid the potential teratogenic effects of drugs such as danazol if they are at risk" of becoming pregnant. Surgical treatment Where infertility is not a problem radical surgery to remove both ovaries is said to be a lasting cure for endometriosis, since it removes the oestrogenic stimulus to endometrial growth. In many cases the patient wishes relief from pain but also desires to retain the possibility of future pregnancy. In these circumstances only conservative surgery can be employed. The intentions in conservative surgery are: To ablate as many endometrial deposits in the pelvic cavity as possible. To restructure the pelvic anatomy by destroying adhesions which interfere with ovarian and tubal function. To destroy endometrial deposits in the ovaries. To deal with sensory nerve pathways. In view of the many vital structures such as the bladder, rectum, colon and ureters in close proximity to each other, conventional open surgery is not always feasible. Laser surgery under laparoscopy, with its almost microscopic accuracy, may be employed. Endometrial deposits and adhesions can be vaporised easily without damaging tissue outside a radius of a fraction of a millimetre from the target. Similarly the laser destruction of ovarian lesions can be carried out without destroying any of the functional tissue. The question of dealing with sensory nerve pathways is difficult to answer. Severe pain is a feature of a number of gynaecological conditions, especially those related to malignancy. Elsewhere in this book operative techniques are described which involve interfering with sensory conductivity centrally, i.e. at the spinal cord level. Recently, a local operative procedure, paracervical uterine denervation, has been recommended. This consists of vaporising the utero-sacral ligaments by laser at their attachment to the posterior aspect of the cervix where the sensory fibres emerge from the uterus. Two difficulties are associated with this procedure. First, the ureters must be avoided and, secondly, veins lying lateral to the ligaments must not be injured. Unfortunately severe pain is often associated with severe endometriosis and adhesions may make the operation very difficult. Reports in the literature record complete relief from pain in 50% of patients followed for more than a year and another 41% obtained moderate relief. IV. Control questions and tasks 1. Frequency of endometriosis pathology. 2. Classification of endometriosis. 3. Laboratory methods of endometriosis diagnosis. 4. Conservative methods of treatment. 5. Surgical methods of treatment. V. List of recommended literature 1. Danforth’s Obstetric and gynaecology.-Seventh edition.-1994.-P.845-853 2. Gynecology.-Stephan Khmil, Zina Kuchma, Lesya Romanchuk.-2003.-P.263-267 3. Gynaecology illustrated. David McKay Hart, Jane Norman.-Fifth Edition.-2000.-P.122128 DEVELOPMENT OF FEMALE GENITALIA IN PRENATAL PERIOD On the 3-4th weeks of embryo development on internal surface of primary kidney a gonad germ is generated. Primary gonad has an indifferent structure (identical for both genders) and consists of celomic epithelium cells (external layer), mesenchyme (internal medullar layer) and gamete cells — gonocytes. Sexual differentiation of indifferent glands is induced by sexual chromosomes. Y-chromosome presence determines testicle development, and X-chromosome presence determines ovarian development. External genitals of fetus also goes through the different stages of development. They are germinated on the 6-7th weeks of the development in the form of genital prominence and urethral fissure, bordered by urethral and labioscrotal folds. Forming of masculine sexual glands begins from the 7th week, and masculine genitals — from the 8th week of fetal development. Differentiation of female reproductive system takes place in later terms. Forming of female-type gonads begins from 8-10th week of pregnancy. Presence of 2 X-chromosomes in a zygote is necessary for ovarian development. A gene inducing ovarian development is localized in long shoulder of X-chromosome. Under its influence gonocytes are transformed into ovogonies, then — into ovocytes, around which the primary granulous cells are generated from mesenchymal cells. They are situated in the cortex of sexual gland and intensively reproduce themselves by means of mitotic division. On the 5th month of embryonal development a number of primary follicles reaches 4 mln., till the birth time of a girl their amount is reduced to 1 mln. The ovary is morphologically formed. Internal genitalia — uterine tubes, body, uterine cervix, upper 1/3 of vagina are formed from paramesonephral ducts. Process starts on the 5-6th and finishes to 18th week of pregnancy. From upper one-third of paramesonephral ducts uterine tubes are formed. Lower and middle parts uniting together form a body and uterine cervix. Lower department of paramesonephral ducts forms the upper one-third of vagina, lower 2/3 are formed from urinogenital sinus Common organ cavity is formed to 21-22 week of gestation. The rest of mesonephral channels are preserved as paraovophorone, epiovophorone and Gartner's passages on the lateral walls of vagina. External female genitals are formed since the 17th week of gestation. At first major labia are formed from labioscrotal folds, from urethral folds minor labia are generated. Clitoris is formed from genital prominence DEVELOPMENT ANOMALIES OF EXTERNAL GENITALIA Most frequently defects of external genitalia development are common at androgeny and adrenogenital syndrome. These defects of external genitalia development are manifestations of genital glands development violations and they will be discussed in the corresponding chapter UTERINE AND VAGINAI DEVELOPMENT ANOMALIES Genitalia formation in female fetus takes place during the first months of gestation from the middle embryonic layer (mesenchyme). From the same layer the organs of urinary system are generated, that's why the uterine and vaginal anomalies can be combined with urinary organs anomalies. The ovaries are formed on the first weeks of gestation from indifferent (identical for both genders) genital gland. On the third month of gestation their differentiation starts. The ovaries dislocate down and draw into the small pelvis. Uterus, uterine tubes and vagina are developed from mesodermal germs (Muller's ducts). One uterine tube, a half of uterus and vagina are generated from each of them. The middle and lower third of these ducts are united on the second month of gestation, forming the external organ contour, but on all the length uterus and vagina are parted by membrane. During the 3rd month of fetal development this membrane is dissolved, uterine cavity and vagina are generated. Uterine tubes are formed from the upper parts of Muller's ducts that didn't join. External genitalia are generated from urino-genital sinus. If during the act of sexual organs forming harmful factors, specifically medicinal ones (uncontrolled medicines reception), would affect a pregnant woman, a differentiation process of the genitals can be broken. Agenesy is the absence of the organ and even of its rudiment. Aplasia is the absence of the organ's part. Atresia is underdevelopment in the result of the prenatal cause. Proceeding from mechanism of genitalia forming, such variants of uterine and vaginal development defects are possible: Both mesonephral ducts are formed properly, but they are not joined together along the whole length. A full uterus and vagina doubling (uterus didelfus) is generated: the patient has two vaginas divided by a thin membrane. Uterine cervix opens into each vagina. There are two uteruses (unicornous), in each uterus there is one tube and one ovary. Both uteruses can function. In the patients with such pathology pregnancy loss is more frequent. In most cases one half of sexual apparatus is developed better than the other one. Both mesonephral ducts are formed properly, but their uniting takes place only at any interval. Other parts of uterus and vagina are divided by a membrane. There can be following variants: membrane in vagina (vagina septa); presence of one vagina, into which two uterine cervices open (uterus bicornus bicollis); membrane in uterine cavity (uterus septa), twohorned uterus (uterus bicornus); saddle-like uterus (uterus arcuatus). At such anomalies genitals can function normally, pregnancy can occur, but frequently pregnancy loss takes place. When there is a saddle-like uterus the irregular fetus positions are usually diagnosed. One of Muller's ducts develops properly, and the other one does not develop at all. Vagina, single-horn uterus with one ovary and one tube is formed (uterus unicornus). In such patient one should inspect urinary system, because such defect correlates with the absence of kidney on the affected side. Unicornous uterus can function, menstrual function is usually for the type of hypomenstrual syndrome. The woman can become pregnant, however there exists high frequency of pregnancy loss on the early terms. One of the mesodermal ducts develops properly, the other—insufficiently. The uterus with rudimentary horn is formed. These cavities can be joined, that's why pregnancy in rudimentary horn is possible. It develops as ectopic pregnancy. During its interruption a considerable bleeding takes place (horn rupture) that's why the surgical intervention is necessary. At presence of closed cavity of rudimentary horn during menstruation blood can deposit inside, that needs the removal of the horn. If uterine development anomalies are combined with underdevelopment of genitals it is followed by violation of menstrual cycle, infertility. Diagnosis is made after examination of external genitals, uterine cervix examination in specula, bimanual examination. Ultrasound examination, sounding, hysterosal-pingography or contrasting sonography (contrasting substance "Echovist" is used) are necessary for specification of diagnosis. Treatment of the development anomalies is surgical. Doubling of uterus and vagina, which does not disturb woman's sexual and reproductive functions, doesn't need intervention. Operative treatment is necessary at presence of ectopic pregnancy or agglomeration of menstrual blood in rudimentary horn. Membranes in vagina are usually diagnosed during pregnancy or delivery; if they prevent child's birth, they are lanced. Absence of vagina (aplasia vagina) is a serious defect, which makes impossible the realization of menstrual, sexual and reproductive functions. It develops primarily (in fetus) or secondly in the result of healing after the carried difficult inflammatory processes in babyhood (smallpox, diphtheria, scarlet fever). It can rarely appear in women after serious labour traumas. Treatment is only surgical. It is a plastic operation with vagina formation from allotment of sigmoid bowel, recently allopl'asty is common. Gynatresy — violation of genital channel permeability in some of its departments. Most frequently atresia of hymen, vagina and uterine cervix are present. Primary gynatresy develops in fetus in the result of embryonic development defects. Secondary gynatresy (acquired) develops in the result of inflammatory processes, carried in childhood. In mature age vaginal atresia can occur in the result of labour traumas, uterine cervix atresia after diathermocoagulation, atresia of uterine cavity or adhesion in it after surplus uterine curretage because of abortion. Primary atresia of hymen ought to be diagnosed by medical personnel or by girl's mother still in newborn period. Then all the further complications can be avoided. If gynatresy is not found in time, then with the beginning of the first menses blood begins to accumulate in vagina, straining it (haematocolpos). Girls complain of pain. After finishing of such "latent menses blood gemolizes, liquid part of it is absorbed, volume is decreased, pain stops until the following menses begin. If the patient does not apply for medical help, then blood, accumulating more and more, gathers in the uterine cavity (haematometra), and in the uterine tubes (haematosalpinx). Diagnosis consists of examination of external genitals, during which one can see obstructed hymen and blood, that has accumulated behind it. During the rectal examination one can palpate tumorous formation in allotment of vagina, uterus and uterine tubes. Treatment. Surgical incision of hymen is necessary. Hymen is crosswise incised. Thick, brown-colored blood is removed from vagina. In order to prevent secondary atresia they put several stitches in dissection edges. Prognosis depends on the interna diagnostics of disease. At long illness duration and development of haematometra and haematosalpinx later a woman can have problems with pregnancy. Destructive process in uterine tubes leads to their occlusion. Endometriosis of internal genitals develops frequently. ABNORMALITES OF OVARIAN DEVELOPMENT Hermaphroditism is a presence of signs of both sexes in one person. True hermaphroditism is presence of genital glands of both sexes in one person on condition of their simultaneous functioning. Such defects are almost not found in practice, because children, sexual glands of which contain simultaneously the tissue of ovary and testicle, are born with other different defects, and die during the first days of their life. False hermaphroditism (pseudoandrogeny) is a defect, at which the structure of external genitalia does not correspond to the character of sexual gland. Human sex is determined by chromosome set, according to which genital glands are developed. At false female hermaphroditism internal genitals and sexual glands are female (ovaries), and external sexual organs are developed like the male ones — clitoris is enlarged and looks like penis, major labia are hypertrophied and look like scrotum. Sometimes after the birth such children's sex is mistakenly determined and parents begin to bring a girl up as a boy. That's why in case of child birth with anomaly of genitals development it is necessary to carry out careful examination, including the genetic one. At false male hermaphroditism genital glands are male (testicles), and structure of sexual organs looks like the female ones. The congenital adrenogenital syndrome is the disease that develops by reason of adrenal glands cortical layer hyperfunction. It is followed by increasing in fetal organism of female sex sexual hormones (androgens) and causes the formation of female genitals according to the masculine type. It is very important to determine correctly the child's sex at birth. Clinic. In such girl the period of puberty begins at the age of 6-7 and it is followed by virilization signs (appearance of masculine secondary sexual signs) — hair growth, forming of skeleton and body building according to masculine type. Children are of a low height, lower extremities are short because of the early epiphisar cartilage closing. At postpubertal form, when the disease starts after the period of the puberty beginning, amenorrhea or oligomenorrhea are found in girls. Breasts, uterus and ovaries do not develop. Later the woman suffers from the primary infertility. Treatment is prescribed by an obstetrician-gynecologist together with endocrinologist. Medicines of glucocorticoid hormones (Prednizolone, Cortisone, Dexametasone) are prescribed to decrease androgens production by adrenal glands. Owing to this the gonadotropic pituitary function increases, ovarian stimulation and production of own estrogens begins. Ovarian absence — two or one — happens rarely, predominantly in fetuses having other severe development defects. Ovarian hypoplasy is the insufficient development of ovaries frequently combined with uterine underdevelopment. Clinically it is manifested as hypomenstrual syndrome. The gonads' dysgenesia (the Shereshevsky-Terner syndrome) is the disease, associated with chromosome abnormalities (one X-chromosome is absent) that causes ovarian tissue underdevelopment. The ovaries are represented by connective tissue, their function is absent. Diagnosis. These patients are of low hight (not higher 130-145 cm). Body weight after birth is low even at interm pregnancy. During examination a short neck with wing-like folds from ears to shoulders, wide shoulders and tubby thorax are typical. The external eyes' corners are drawn down, palate is high, that's why these patients have special timbre of voice. Psychic development is normal, sexual orientation is female, but in the puberty period secondary sexual signs develop not enough. During gynecological examination highly expressed signs of genital infantilism are found. External genitalia are underdeveloped, there is a severe vagina, uterine and ovarian hypoplasia. Genetic examination, that confirm the chromosome anomaly has a great importance for specification of diagnosis. Tests of functional diagnostics give a picture of the expressed lowering or practical absence of hormones, the basal temperature is permanently low, "fern" and "pupils" symptoms are absent. There is 50% of parabasal cells during colpocytological investigation. Treatment at prepuberty age is directed on the growth stimulation. After 15-17 years of age replacement therapy with hormones is prescribed: they are estrogens for 6-9 months, after this the cyclic therapy with Estrogens and Progesterone is indicated. Such treatment leads to development of secondary sexual signs, uterine cyclic bleedins initate. Polycystic ovarian disease (the Stein-Leventhal's syndrome). This is a genetically predisposed disease, pathogenesis of which is a violation of sexual hormones synthesis in ovaries in the result of insufficiency of enzyme systems. Excess amount of androgens is produced. Clinically this disease is characterized by excessive hairiness (hirsutism), by hypomenstrual syndrome or by amenorrhea and infertility. Well-developed secondary sexual signs and enlarged two-sided ovaries are found during the examination. During US-onografy the presence of a great deal of follicular cysts, that is a cause of ovarian enlargement is revealed. Excess androgen stimulation causes thickening of albuminous ovarian envelope, that's why ovulation does not come, and follicles, do not burst, transform into cysts. Treatment of disease can be conservative (hormonal therapy) or operative (wedgeshaped ovarian resection). You can get more detailed information about the Shereshevsky-Turner syndrome and the Stein-Leventhal's syndrome from the chapter "Menstrual function disorders". DELAYED PUBERTY Underdevelopment or absence of the secondary sexual signs at the age of 13-14 and lack of menses at the age of 15-16 should be considered as delay of sexual development (DSD). There are central and ovarian form of delayed puberty. It depends on the primary link of disease pathogenesis. At central genesis the ovarian insufficiency comes secondary in the result of insufficient gonadotropine stimulation. At primary lesion of gonads a secretion of gonadotropic hormones is raised. It is caused by the lack of inhibiting influence of sexual hormones on pituitary. Central form of delayed puberty is most frequently caused by such factors, as infectious-toxic diseases (rheumatism, viral influenza, chronic tonsillitis, tuberculosis), stress situations, excessive physical loading. These factors, acting in the child age, give rise to functional immaturity of hypothalamic structures that are responsible for sexual development, functional regulation of reproductive system is disturbed. The lesion level at central form can be different. To genetically predisposed forms delayed puberty at Lorenz-Munne-BardeBidle's syndrome is refered. Delay of sexual maturity develops in patients with hypopituitarism of organic origin. Delay of sexual development of ovarian genesis most frequently appears in patients with genetic defects. Hereditary factor is present in 2/3 of patients. Damage of ovaries happens still in pre-natal period, damage degree of fetal ovaries depends on the duration of pathogenic factor action such as taking of medicines especially hormonal ones by mother, infectious mother's diseases, etc. In childhood epidemic parotitis and measles most frequently cause ovarian insufficiency. Clinically a delay of sexual development is expressed by that or other degree of sexual (genital) infantilism. Genital infantilism is a such state, when in reproductive age women anatomic and associated with them functional peculiarities of genitalia, typical for child organism, are preserved. Diagnosis. External examination of women reveals low hight, frail body building, small breasts. Hairiness on pubis is weakly developed, major labia don't cover the minor ones. Vagina is narrow, vaults are not expressed. Uterus is small, 2/3 of it is the cervix, 1/3 is the body. Taking into consideration such anatomic peculiarity, the expressed uterine bend to front — sharp-angle anteflexion frequently occurs. Uterine cavity length is always shorter than the norm (6 cm and less). There are three degrees of uterine underdevelopment for cavity lenght. They are: I degree — 7- cm II degree — 5-3,5 cm III degree — less then 3,5 cm Uterine tubes are long and sinuous, ovaries are considerably smaller as compared with the Functional changes are closely connected with the structural ones. Menses in such women start lately at the age of 15-16. Primary amenorrhea can appear in the result of considerable underdevelopment. Amount of discharge is insignificant, menses duration is 1-2 days (hypomenstrualsyndrome). Sometimes menses comes not monthly, and is more rarely. Menses are followed by strong pain (algo-dysmenorrhea), that is connected with uterine structure. At expressed ovarian underdevelopment and considerable lowering of their function, sexual desire is absent in women. If hormonal background is moderately altered, sexual function is preserved. Women with hypoplastic uterus can't become pregnant (primary infertility) for a long time after marriage. If pregnancy comes, it can be ectopic (because of uterine tubes' structure), or it interrupts in early terms, because insufficient amount of hormones does not provide normal pregnancy development. Such interruptions of pregnancy in patients with genital infantilism can occur several times (regular abortions), but pregnancy and associated with it intensive hormones secretion always has positive influence on the patient's organism, for it contributes to uterine development. Treatment of such patients should be complex and includes restorative therapy, physiotherapy, prescribing small doses of hormones for ovarian function stimulation. Going in for sports, sanatorium-health-resort cure, gynecological massage are also recommended. The earlier the cure begins, the greater are the chances for success. At central genesis of disease Prephisone (25-50 AU i/m) is indicated during the first phase of menstrual cycle (at amenorrhea the first cycle day is considered the first day of cure) daily, 8-10 days. Then Choriogonine (2500-3000 AU i/m) is prescribed during the 12, 14, 15, 16, 18th cycle day. Clostylbept (Clomiphen) — 50 mg per day from 5th till 9th cycle day, then Microfolline — 0,05 nig 2 times per day till 12-14th cycle day are also indicated. Treatment with Clomiphen and estrogens takes 2-3 months, then synthetic Progestines in cyclic mode during 2 courses with 7-days intervals are taken. For better hormones' reception folic acid (0,06g per day) in first phase, in ovulation period and second phase — Thymidine — 25-50 mg per day and vitamin E 50-100 mg per day are prescribed. INCORRECT UTERINE POSITIONS Physiological uterine position is considered to its situation in the center of small pelvis on identical distance from symphysis, sacrum and lateral walls of pelvis. Uterine fundus is situated beneath the plane of inlet, external cervical os is on the level of ishial spines (linea interspinalis). This situation is provided by sustaining fixative and suspentive apparatus of uterus. Uterine and vaginal own tone, the tone of frontal abdominal wall, diaphragm and muscles of pelvic floor have a great importance. Uterine position is uterine relation to the leading pelvis axis. Uterus is able to displace as for its normal position. This displacement can be physiological (uterus goes back to its previous position) or pathological and fixed. For such conditions uterus is immovably fixed to pelvis walls or adjacent organs by adhesions or tumor. Anteposition — uterine displacement considering to leading axis to front. Retroposition — uterine displacement backwards. Lateroposition — (dextro- et sinistropositio) displacement of uterus to correspond side. Physiological retroposition of uterus happens at repletion of urinary bladder Anteposition appears at full rectum Pathological uterine displacement happens at tumors presence or pus accumulation Then uterus replaces to the healthy side. After operative interventions or after the carried inflammatory process with formation of adhesions, a connective cicatrix tissue drags uterus into this side. Uterine movability is limited or absent Inclination of uterus (versio uteri) is a relation of vertical uterine axis to horizontal plane. Inclination of uterus to front (anteversio), aside (lateroversio), and also backwards (retroversio) are distinguished. Causes of pathological uterine inclinations may be the tumors of genital organs (only uterine body is displaced, and cervix remains in its place) and insufficiency of uterine ligaments. Uterine flexion (flexio uteri) is the relation of uterine body to its cervix. Normally between uterine body and cervix there exists an obtuse angle (for about 120°), opened forward (anteflexio). If the angle is less than 120°, such anteflexion refers to the sharp one (it is found at genital infantilism). Uterine flexion to back (retroflexio), to the right (lateroversio dextra), or to the left (lateroversio sinistra) is pathological. Retroflexion is mobile and fixed. Fixation happens at accretion of uterus with parietal peritoneum. Combination of retroversion and retroflexion is called uterine retrodeviation. Retroflexion and uterine retrodeviation are followed by aching dull pain in lower abdomen, painful menses (algodysmenorrhea) and infertility. Uterine cervix erosions and endocervicites can develop in the result of blood supply violation and blood stagnation. Sometimes patients complain of frequent and painful urination. There can be a delay of evacuation and pain during it. These phenomena sometimes can be eliminated owing to uterine reposition. Aged women with retrodeviation can have uterine and vaginal prolapse often. UTERINE DESCENSE AND PROLAPSE Prolapse is a single pathological process based on a tight anatomic tie between uterus, vagina, ovaries, urinary bladder and rectum. Depending on the stage of this process, uterine descense and prolapse are distinquished. The vaginal walls descense—vaginal wall has lost its tone, they are descent and do not leave the borders of vagina's introitus. The vaginal walls prolapse — vaginal walls are beneath the introitus of vagina. Degrees of uterine displacement: I degree — vaginal part of uterine cervix is found lower, then sciatic spines (linea interspinalis), however it stays inside of pudendal cleft borders even during the exerting (uterine descense) II degree — external cervical os goes beyond the borders of pudendal cleft, it is found beneath vaginal introitus, and uterine body is above it (incomplete uterine prolapse) III degree — all the uterine and vaginal walls are found outside of pudendal cleft (complete prolapse) Etiology and pathogenesis. Multiple deliveries, vaginal and perineum ruptures during the previous delivery, constipations, weight lifting, hard work can cause weakening or violation of the supportive, fixative and suspentive apparatus of the uterine structure and uterine displacement. More frequently descense and prolapse of uterus develops in women after 50 years in connection with the beginning of age atrophy of sexual organs and ligament apparatus. Clinic. Uterine descense and prolapse is a long process. Woman complain of dragging pain in lower abdomen and in sacrum region, frequent urination, urine incontinence, that appears during the smallest physical loads — cough, quick motions. Later a tumorous formation — uterine cervix with external cervical os appears from vagina. Menorrhage is possible if woman menstruates. Sexual life is possible after uterine reposition. Woman can become pregnant. During the first months of pregnancy cases of its spontaneous interrupting are common. After the 12th week of gestation uterus stops to prolapse because of its largeness, after delivery the prolapse appears again. Together with the anterior vaginal wall urinary bladder wall discences and prolapses. Cystocele is formed. Descense and prolapse of posterior vaginal wall causes formation of rectum hernia (rectocele). At complete uterine prolapse, its body together with cervix is found beneath the introitus of vagina. Vagina is turned out by the mucous membrane. Elongation of the cervix develops frequently. Mucous membrane of vagina thins or thickens and dries out. Secondary trophic changes can develop — trophic (decubital) ulcers on cervix and vaginal walls, polyps near the external os are common. Histologically microcirculation impairment, hyper- and parakeratosis, inflammatory infiltration, sclerotic changes are found. Changes in urinary system can also appear. Patients complain of frequent urination and urine incontinence. At urine analysis bacteriuria is found. During chromocystoscopy trabecularity and cavities in mucous of urinary bladder, ureters dyslocation, cystitis, lowering of sphincters' tone are revealed. During excretory urography atony and dilation of ureters are present. At US examination nephroptosis, dilation of kidney are observed. Changes in this system are caused by violation of the blood circulation and position of urinary bladder, ureters and associated with this urine outflow. Diagnosis is not of a special difficulty because prolapse is found during inspection of external genitalia. It is important to determine whether it is a complete or incomplete prolapse. Doctor takes the prolapsed organs with index and forefingers on the level of vaginal introitus. If uterine body is palpated between them, then the prolapse is incomplete. If fingers close behind the uterine fundus — this is the complete uterine prolapse. Perineum inspection is necessary to find scars and to estimate the functional state of pelvic muscles. Treatment. Method of treatment for each patient is individually selected. It depends on the age, general patient's state, presence of menstrual and sexual functions. Conservative treatment is indicated for women at small descense of uterus, in reproductive age or for emaciated patients, the age or general state of which does not allow to use a surgical intervention. Medic ally-protective regimen with exclusion of physical loads is of great importance. Medical physical training, directed to the strengthening of abdominal press and pelvic floor muscles, rational feeding for constipation prevention should be recommended. Orthopedic method is in introduction of special devices into vagina for support of uterus in its place (rubber rings). Great attention is paid to hygiene of genitals during pessaries usage, taking them out regularly and sterilizing by boiling. Conservative treatment consist also of the treatment of trophic ulcers and vaginitis, that develop in such patients rather frequently. Doctor prescribes cure. Midwife or medical sister can fulfil it. Vaginal walls, that have prolapsed and uterine cervix are processed with antiseptic solutions (Potassium permanganate, Furacillin, Hydrogenium peroxide, Chlorhexidin bigluconate). After processing of the prolapsed tissues, they are dried up by a sterile gauze serviette. On decubital ulcer's surface ointment or liniment with antiseptics are applied, a surface is covered by sterile serviette, and the uterine is replaced into vagina. Then a tampon with aseptic remedy is inserted, tampon size depends on the vaginal size. After elimination of inflammatory process and vaginitis for acceleration of tissue regeneration on the region of decubital ulcer there can be applied an ointment with Solcoseril, Apilac, Methyluracil, Propoceum. Such procedures are hold daily during 1-2 weeks to complete ulcer epithelization. If ulcer is not healed up to that time, then biopsy for differential diagnostics with cervix cancer is made. Aged women which have used vaginal pessaries undergo careful supervision by a doctor of female dyspansery, a midwife, and a medical assistant. Their long usage can cause bedsores on the uterine cervix and vagina. Acute urine delay appears if tissues that have prolapsed squeeze. Urine should be let out by catheter, and patient should be hospitalized. During catheterization a catheter is directed not upwards to symphysis but on the contrary downwards, because urethra which is connected with anterior wall of vagina changes its usual location. Surgical treatment is the most radical method. The main aim of cure is restoring of pelvic floor muscles integrity, creation of uterine support, and also renewing of normal structure and function of uterine ligaments. Basic methods of surgical interventions are: the plastyc of frontal vaginal wall (anterior colporrhaphy), the plastyc of posterior vaginal wall and perineum (posterior colporrhaphy, colpoperineorrhaphy) — the plastyc of pelvic floor and perineum is made shortening of round uterine ligaments (is used in women of reproductive age); ventrofixation — uterus is fixed to the anterior abdominal wall (is combined with frontal and posterior plastics of vagina) amputation of the cervix by Shturmdorf is made at uterine cervix pathology In senile age at complete uterine prolapse in combination with concomitant pathology (uterine tumors etc), vaginal hysterectomy through vagina is made. This operation is combined with the plastic of posterior vaginal wall and levatoro-plastics. Obligatory condition for surgical intervention is the complete healing of decubital ulcers, absence of inflammatory process in vagina. Prevention of uterine and vaginal walls descense and prolapse is necessary in medical and social aspects. It is important for woman to do physical exercises, to go in for sport, to train abdominal press and pelvic floor muscles. During delivery it is necessary to diagnose interm and to restore perineal muscles in their rupture. Doctor and midwife of postnatal department have to take careful tendance for seams, to watch closely for the regimen and women's conduct in postnatal period, not to allow woman with perineum ruptures to get up and to sit down prematurely. Uterine inversion Uterine inversion is a state, in some causes of which uterine fundus is pressed inside. So serous membrane is inside, and mucous one is outside. In this case the ovaries get inside this formation, their blood supply violates. The stagnant phenomena and uterine edema develop. There are two forms of uterine inversion: puerperal (postnatal) and oncogenetic (caused by a tumor). The mechanism of puerperal inversion was described in obstetrics course. The oncogenetic uterine inversion is caused by the case of protruding myoma placed on a short pedicle. Clinically the oncogenetic uterine inversion is followed by extending strong pain low in the abdomen like delivery. A fibromatous node, that is situated in uterine cavity, more frequently near its fundus, descends into lower segment, is perceived by it as a foreign body, and uterus begins to push it out. Uterine cervix is dilated, node appears outside, but a pedicle stem does not allow it to be born. Node's and uterine blood supply is disturbed, node necrosis is developed. Hysterectomy is the treatment of oncogenetic uterine inversions. An attempt to remove the node through the uterine cervix is dangerous because of uterine perforation possibility, if nodes pedicle is short and wide. The uterine torsion The uterine torsion (torsio uteri) is turning of uterine body around vertical axis. It happens extraordinarily rarely. Uterine and ovarian tumors, adhesions process in small pelvis are the main causes of uterine torsion. TRAUMAS OF FEMALE GENITALS Damages of external genitals can be as contusion, haematoma, hypodermic effusions of blood, that ordinary are accompained by damage of skin. More often these damages appear as a result of trauma such as falls and blows. In the village hornblows of domestic animals are observed. These traumas can be followed by lacerated wounds which ordinary penetrating deeply into tissues, sometimes vagina and even rectum can be damaged. In case of heavy traumas of urethra, urinary bladder, and also pelvis bone can be damaged. In case of damage of vagina trauma can penetrate into abdominal cavity. Ruptures of lateral walls of vagina are very dangerous because vaginal branches of uterine arteries pass in this area. Clinically trauma is characterised by pain and haematoma of blue to purple colour in damaged place. In case of severe internal bleeding a picture of hemorrhagic shock develops. Bleeding can be followed by anaemia. In case of clitoris rupture bleeding can be especially massive. Sometimes inserting of foreign objects into genital organs can happen. Especially frequently it happens in girls before 10 years. Adult women can introduce catheters, sounds and other objects into uterus with aim of pregnancy interruption. In such cases frequently uterus perforation, bleeding can appear, that's why the woman applies for medical care. If there is no damage of genital organs, presence of foreign body causes inflammatory process. Purulent excretions from vagina, sometimes with blood admixtures appear. Foreign body in adults can be found due to speculum examination. For children one should use cautious rectal research and vaginoscopy. Diagnosis is based on examination. If there is suspicion on trauma of adjacent organs catheterization of urinary bladder, cystoscopy is made. US can be useful for diagnostics of foreign bodies in vagina. Treatment. Traumatized tissues are sutured. In case of haematoma it is incised, bleeding vessels are knitted and drained. If it is necessary hemotransfusion is performed. Uterine cervix, vaginal, uterine ruptures, associated with labor act, are described in obstetrics course. IV. Control questions and tasks 1. What is suspensive apparatus of the uterus? 2. What is fixative apparatus of the uterus? 3. What is supportive apparatus of the uterus? 4. Classification positional anomalies of female genitalia in woman. 5. Main causes of irregular positions of internal genitalia in woman. 6. What clinical symptoms occur at positional anomalies of female genitalia in woman? 7. Prevention of irregular positions of female genitalia. 8. Etiology, clinic, diagnosis, treatment of vulva and vagina injuries. 9. Etiology, clinic, diagnosis, treatment of uterine cervix and uterus injuries. V. List of recommended literature 1. Danforth’s Obstetric and gynaecology.-Seventh edition.-1994.-P.887-905 2. Gynecology.- Stephan Khmil, Zina Kuchma, Lesya Romanchuk.-2003.-P.81-101 3. Gynaecology illustrated. David McKay Hart, Jane Norman.-Fifth Edition.-2000.-P.233252