Malformations of the female genital organs. Anomalies of male genital organs
Lecture No. 17
Incorrect positions of the uterus.
Plan.
1. Development of the reproductive system.
2. Malformations of the genital organs.
3. Abnormal positions of the uterus, classification
4. Displacement of the uterus in the horizontal and vertical plane.
5. Prolapse and loss of genital organs.
DEVELOPMENT OF THE GENERAL SYSTEM.
Development of the ovaries.
The rudiments of the gonads appear in the early stages (first weeks) of intrauterine development, and they do not have predominant elements of the female or male sex. The formation of gonad primordia occurs through complex transformations of the epithelium of the abdominal cavity. This is a sexual cushion. Under the influence of genetic factors, either the ovary or the horsicicles are formed from the genital ridges. The process of ovarian formation occurs gradually; as they develop, they shift downward and descend into the small pelvis along with the uterine rudiment.
Uterus, tubes and vagina.
It develops from the Müllerian ducts, which form in the 4th week of intrauterine life. The Müllerian ducts are initially solid, then cavities form in them. As the embryo grows, the middle and lower sections of the Müllerian ducts merge. The uterus is formed from the fused middle ones, the vagina is formed from the fused lower ones, and the tubes are formed from the unfused upper ones.
External genitalia
They are formed from the urogenital sinus and the skin of the lower part of the body of the embryo.
Improper development of the female genital organs.
Developmental anomalies include:
1) a violation in the anatomical structure.
2) delayed development of properly formed genital organs.
Severe structural anomalies are usually accompanied by a violation of all or individual functions of the reproductive system. With some types (doubling), organ functions may remain normal.
Anomalies are characterized by structural deformity - they usually arise during intrauterine life, due to a disruption in the formation of the rudiments of the genital organs.
Delayed development of the genital organs can occur under the influence of unfavorable conditions affecting the body mainly in childhood and puberty.
The occurrence of malformations appears to depend on disturbances in nutritional conditions, gas exchange and other environmental conditions in which the fetus develops. Environmental conditions are determined by the state of the mother's body. Therefore, diseases especially of infectious etiology and intoxication can cause developmental anomalies.
Pathology associated with abnormalities
Anomalies of uterine development.
The complete absence of the uterus occurs only in non-viable fetuses.
Duplication of the uterus and vagina.
This type occurs due to a disruption in the process of connecting the middle and lower sections of the Müllerian ducts. These abnormalities can be observed throughout the entire uterus and vagina or only in some parts of these organs. The most pronounced form is the uterus, 2 cervixes and 2 ovaries. Between them is the bladder and rectum. It occurs very rarely, it is somewhat more common when both halves touch in the area of the cervix.
Bicornuate uterus.
There may be 2 cervixes or 1 cervix. The vagina may or may not have a septum. Bicornus may be slightly expressed and indicate a saddle-shaped uterus.
Symptomatology
May be asymptomatic. With sufficient development of both halves or one, menstruation and sexual function can remain normal. Pregnancy and normal labor are possible. If doubling is combined with underdevelopment of the ovaries and uterus, then corresponding symptoms are possible.
Underdevelopment of the genital organs.AND infantilism is a condition in which the development of the body is delayed, and in adulthood, anatomical and functional features are determined that are normally characteristic of childhood or adolescence.
A distinction is made between general infantilism, in which developmental delay affects all organs and systems of the body, and partial, when one of the systems lags behind in development, for example, cardiovascular, reproductive, bone, etc.
The following variants of underdevelopment of the reproductive system are noted: sexual infantilism in combination with general or partial underdevelopment of the woman’s body; A well physically developed woman with a correct physique and normal height has only underdevelopment of the reproductive system.
Underdevelopment of the reproductive system is caused by malnutrition (hypovitaminosis), chronic intoxication, chronic diseases, disorders of the functions of the endocrine glands, which were observed in childhood, or, most importantly, during puberty.
Underdevelopment of the uterus and other parts of the female reproductive system is mainly associated with delayed development of the ovaries and a decrease in their functional ability.
Clinical symptoms of underdevelopment of the reproductive system are as follows: underdevelopment of the labia majora and minora; trough-shaped crotch; narrow, short vagina with shallow arches and sharp folding of a conical shape; long cervix, its body is small, compacted; the oviducts are thin, tortuous, elongated, small dense ovaries.
It is customary to distinguish three degrees of uterine underdevelopment; embryonic uterus - length less than 3.5 cm; baby uterus - length from 3.5 to 5.5 cm; virgin uterus - length from 5.5 to 7 cm.
With infantilism of the genital organs, the most common disorders are menstrual function in the form of amenorrhea, hypomenstrual syndrome, menorrhagia, dysmenorrhea; sexual - decreased sexual feelings; childbearing infertility, miscarriage, ectopic pregnancy, weakness of labor, uterine bleeding during childbirth; secretory - hypersecretion of the glands of the body and cervix.
It is necessary to distinguish a hypoplastic uterus from an infantile one. The hypoplastic uterus is of regular shape, the body is longer than the cervix, but its size is small.
Treatment of underdevelopment of the genital organs is a very complex problem. Thus, it can be absolutely unsuccessful in the case of the embryonic uterus, while with less pronounced infantilism, persistent, long-term, complex treatment with the use of sex hormones, diathermy and other thermal procedures, mud therapy, vitamin therapy, therapeutic exercises, restoratives, and good nutrition can give positive results.
It is important to remember that with the onset of sexual activity, pregnancy cannot be interrupted under any circumstances, since the latter ensures the further development of the reproductive system. Abortion can lead to complete inhibition of ovarian function and the development of persistent amenorrhea.
In hygiene and physical development rooms for girls, due attention must be paid to identifying possible infantilism of the genital organs.
True hermaphroditism.
In general, it is genetically determined by the presence of a V chromosome.
Clinic. The gonads contain tissue from both the testicles and the ovary.
Karoitype: approximately 80% - 46XX, the remaining cases - 46XY.
The external genitalia may appear male, female, or a mixture of both. The internal genital organs are a combination of male and female glands. Based on the formation of the internal genital organs, there are 4 variants of true hermaphrodism:
A) on one side there is an ovary, on the other - a testicle;
B) on both sides - ovotestis;
C) on one side the ovary or testicle, on the other - ovotestis;
D) on one side there is an ovotestis, on the other - a cord.
Treatment includes surgical correction of the external genitalia.
The choice of sex depends on the predominance of male or female sex hormones.
Inversion of the uterus
It is observed very rarely. The serous membrane is located on the inside and the mucous membrane is located on the outside. With complete inversion, the body of the uterus is located in the vagina, and the cervix is higher. When incomplete, the membrane of the uterine fundus is pressed into the cavity. When eversion occurs, the fallopian tubes and cervix are pulled inward and funnels are formed. There is a circulatory disorder, swelling of the uterus. Eversion occurs when the period of labor is mismanaged, when a tumor with a short stalk is expelled from the uterus, when the placenta is squeezed out, or when the umbilical cord is pulled.
Symptomology- acute pain, shock and bleeding from blood vessels.
Treatment- reduction, or surgery. Reduction under anesthesia.
Etiology.
Various reasons lead to the inflection and tilt of the uterus, a violation of the tone of the uterus, causing relaxation of the ligaments.
1. Decreased tone with infantility (relaxation of the sacral
uterine ligaments).
2. Multiple births, especially complicated by surgery and infections. Prolonged keeping of a woman in labor in bed. Disorders of the muscles and fascia of the pelvic floor.
3. Inflammatory process, accompanied by the formation of adhesions.
4. Ovarian tumor, myomatous nodes growing on the anterior wall
uterus.
Conclusions.
Severe structural anomalies are usually accompanied by a violation of all or individual functions of the reproductive system. The occurrence of an anomaly depends on malnutrition, environmental conditions, ecology and other factors in which the fetus develops. Knowing the causes will help midwives prevent this pathology in time. Incorrect positions of the genital organs impair the function of the genital organs and can lead to infertility. Prevention of this pathology is the main task of health workers.
The student must know: anomalies in the development of the genital organs, incorrect positions of the genital organs, significance for women, the role of the midwife in the prevention of this pathology.
The student must understand: the mechanism of formation of this pathology, its significance for a woman’s reproductive function.
Questions for self-control.
1. During what period of the fetus’s intrauterine life does the formation of the genital organs occur?
2. Causes of developmental defects.
3. Types of developmental defects.
4. What is the typical position of the uterus?
5. Factors contributing to the physiological position of the uterus.
6. Changes in the inclination and inflections of the uterus, causes, clinic, diagnosis, principles of treatment.
7. Causes of uterine prolapse.
8. When does a hernia of the bladder and rectum form?
9. Clinic of genital prolapse and prolapse. Principles of treatment.
10. Prevention of incorrect positions.
Lecture No. 17
Topic: Developmental anomalies of the female genital organs.
Fallopian tube atresia- one-sided or two-sided, local or total. The result of congenital tubal obliteration.
Duplication of the fallopian tube- can be on one or both sides.
Fallopian tube lengthening- may be accompanied by kinks and twisting of pipes.
Shortening of the fallopian tube- the result of its hypoplasia. If the abdominal opening does not reach the ovary, it is unlikely that the egg will enter the tube.
Anomalies of vaginal development.
Vaginal agenesis– complete absence of the vagina due to its non-plugging. Rarely seen.
Vaginal aplasia- congenital absence of the vagina, develops in the 3-17th week of intrauterine development due to a violation of the central fusion of cells of the paramesonephric ducts. It can be complete or partial with a functioning normal or functioning rudimentary uterus. Leads to the development of hematometers, hematocolpos.
A) Complete vaginal aplasia– often combined with uterine aplasia or rudimentary uterus. In 43.6% of cases it is combined with anomalies of the urinary organs. With complete aplasia, the external opening of the urethra can be widened and displaced downward. There are 4 types of structure of the vaginal vestibule:
B) Partial vaginal aplasia– combined with a normally functioning uterus. In 19.3% of cases it is combined with malformations of the urinary organs. The upper, middle or lower third or 2 thirds can be applated.
Vaginal atresia (syn.: Müllerian duct aplasia)– the lower part of the vagina is replaced by fibrous tissue. Its upper sections, the cervix and body of the uterus, fallopian tubes, ovaries and external genitalia are formed correctly. During puberty, secondary sexual characteristics appear, but menstruation is absent, and hydrometrocolpos is possible. It can be combined with anal atresia (complete or fistulous) and agenesis of the urinary system. Population frequency – from 2: 10,000 to 4: 10,000. Type of inheritance – presumably autosomal dominant, limited by sex. There are several forms: hymenal; retrohymenal; vaginal; cervical.
Vaginal septum (syn.: vaginal division)– can be complete or partial, has underdeveloped epithelial and muscle layers.
Vaginal doubling (vagina duplex) – the septum between two organs is represented by all layers of the wall. Usually combined with uterine duplication.
Anomalies in the development of the external female genitalia
Agenesis of the clitoris– complete absence of the clitoris due to its non-clitoris. It is extremely rare.
Clitoral hypertrophy (syn.: clitoromegaly)– an increase in the size of the clitoris, observed with adrenogenital syndrome.
Hypoplasia of the labia majora– occurs as part of multiple anomaly syndromes.
Hypoplasia of the clitoris– is extremely rare.
Defects of the vulva and perineum- are combined, so they have a common embryogenesis. Divided into several groups:
A) Rectovestibular fistulas are common.
B) Rectovaginal fistulas are common.
C) Rectocloacal fistulas are common.
D) Partially masculinized perineum with fusion of the anus and/or vagina.
D) An anal opening located in front.
E) The perineum is grooved.
G) Perineal canal.
Intersex conditions
Hermaphroditism, or bisexuality, is a term for developmental disorders of the genital organs when their structure combines the characteristics of both male and female sexes. The word "hermaphrodite" comes from Greek mythology. This was the name of the son of the gods Hermes and Aphrodite, who was united in one body with the nymph Salmanida. Antique images of hermaphrodites have been preserved, representing a creature with a wide pelvis, mammary glands and a small penis.
True hermaphroditism (syn.: ambisexuality, bisexuality)– the presence in one organism of germ cells of both sexes and both reproductive apparatus. There are several forms:
A) True bilateral hermaphroditism– on each side there is an ovotestis (gonad having male and female reproductive cells) or a testicle and an ovary.
B) True unilateral hermaphroditism- on one side there is a normal gonad, on the other - ovotestis.
IN) Hermaphroditism true alternative(syn.: true lateral hermaphroditism)– on one side there is a testicle, on the other there is an ovary.
The ovaries and ovotestis are located in the abdominal cavity or inguinal canal, the testicle is located in the scrotum or inguinal canal. The more testicular tissue there is, the more likely the testicles are to descend into the scrotum. Histologically, the structure of the ovaries is normal, there is no spermatogenesis in the testes, and there are a large number of Leydig cells. Secondary sexual characteristics are often mixed. Etiology: 1) mosaicism 46, XX / 46, XY or 46, XX / 47, XXY; 2) translocation of a section of the Y chromosome to the X chromosome or autosome; 3) gene mutation. The type of inheritance is unknown.
False hermaphroditism (syn.: pseudohermaphroditism)– characterized by a discrepancy between the structure of the gonads and the structure of the external genitalia. There are:
A) False male hermaphroditism– patients have testicles, and the external genitalia are formed according to the female type or have one or another degree of feminization. According to phenotypic manifestations, 3 forms are distinguished:
1) feminizing – female body type,
2) virile, or masculinizing – male body type,
3) eunuchoid – eunuchoid body type.
Signs of male false hermaphroditism are present when gonadal dysgenesis syndrome And incomplete masculinization syndrome. Patients with these syndromes have functionally and morphologically defective internal genital organs, depending on the form of the disease, either male and female, or only male, and the external genitalia have characteristics of both sexes.
B) False female hermaphroditism– patients have ovaries, the external genitalia are developed according to the male type. Among patients with false female hermaphroditism, congenital adrenogenital syndrome is most often diagnosed.
– violations of the shape, size, localization, number, symmetry and proportions of the internal and external genital organs. The cause is unfavorable heredity, intoxication, infectious diseases, early and late gestosis, hormonal disorders, occupational hazards, stress, poor nutrition, poor environment, etc. The diagnosis is established on the basis of complaints, anamnesis, external examination, gynecological examination and instrumental results research. Therapeutic tactics are determined by the characteristics of the developmental defect.
General information
Anomalies of the female genital organs are disorders of the anatomical structure of the genital organs that arose during the period of intrauterine development. Usually accompanied by functional disorders. They account for 2-4% of the total number of birth defects. In more than 40% of cases they are combined with anomalies of the urinary system. Patients may also have lower gastrointestinal malformations, congenital heart defects, and musculoskeletal abnormalities.
The frequent combination of anomalies of the female genital organs with other congenital defects necessitates a thorough comprehensive examination of patients with this pathology. Congenital defects of the external genitalia are usually detected at birth. Anomalies of the internal genital organs can be detected during menarche, during a routine gynecological examination, when visiting a gynecologist with complaints about dysfunction of the reproductive system (for example, infertility) or during gestation. Treatment is carried out by specialists in the field of gynecology.
Classification of anomalies of female genital organs
Taking into account anatomical features, the following types of birth defects of the female reproductive system are distinguished:
- Absence of an organ: complete – agenesis, partial – aplasia.
- Violation of the lumen: complete fusion or underdevelopment - atresia, narrowing - stenosis.
- Change in size: decrease - hypoplasia, increase - hyperplasia.
An increase in the number of entire organs or their parts is called multiplication. Doubling is usually observed. Anomalies of the female genitalia, in which individual organs form an integral anatomical structure, are called fusion. If the organ is located in an unusual location, it is called ectopia. Depending on the severity, there are three types of anomalies of the female genital organs. The first is the lungs, which do not affect the functions of the genitals. The second is of moderate severity, having a certain effect on the functions of the reproductive system, but not excluding childbearing. The third is severe, accompanied by gross disorders and incurable infertility.
Causes of abnormalities of the female genital organs
This pathology occurs under the influence of internal and external teratogenic factors. Internal factors include genetic disorders and pathological conditions of the mother’s body. Such factors include all kinds of mutations and burdened heredity of unknown etiology. The patient's relatives may have developmental defects, infertile marriages, multiple miscarriages and high infant mortality.
The list of internal factors that cause abnormalities of the female genital organs also includes somatic diseases and endocrine disorders. Some experts in their studies mention the age of parents over 35 years. Among the external factors contributing to the development of abnormalities of the female genital organs are drug addiction, alcoholism, taking a number of medications, poor nutrition, bacterial and viral infections (especially in the first trimester of gestation), occupational hazards, household poisoning, unfavorable environmental conditions, ionizing radiation , being in a war zone, etc.
The immediate cause of abnormalities of the female genital organs is disorders of organogenesis. The most severe defects occur due to adverse effects in the early stages of gestation. The formation of paired Müllerian ducts occurs in the first month of gestation. At first they look like cords, but in the second month they transform into canals. Subsequently, the lower and middle parts of these canals merge, the uterine rudiment is formed from the middle part, and the vaginal rudiment is formed from the lower part. At 4-5 months, the body and cervix are differentiated.
The fallopian tubes, originating from the upper, unfused part of the Müllerian ducts, are formed at 8-10 weeks. The formation of the tubes is completed by week 16. The hymen comes from the lower part of the fused ducts. The external genitalia are formed from the skin and the urogenital sinus (anterior part of the cloaca). Their differentiation occurs at 17-18 weeks of gestation. The formation of the vagina begins at 8 weeks, its increased growth occurs at 19 weeks.
Variants of anomalies of female genital organs
Abnormalities of the external genitalia
Malformations of the clitoris can manifest themselves in the form of agenesis, hypoplasia and hypertrophy. The first two defects are extremely rare anomalies of the female genital organs. Clitoral hypertrophy is found in congenital adrenogenital syndrome (congenital adrenal hyperplasia). Severe hypertrophy is considered an indication for surgical correction.
Anomalies of the vulva, as a rule, are detected as part of multiple malformations, combined with congenital defects of the rectum and lower parts of the urinary system, which is due to the formation of these organs from the common cloaca. Anomalies of the female genital organs may be observed, such as hypoplasia of the labia majora or vaginal fusion, which may or may not be combined with fusion of the anus. Rectovestibular and rectovaginal fistulas are common. Surgical treatment – labiaplasty, vaginalplasty, excision of the fistula.
Anomalies of the hymen and vagina
Abnormalities of the ovaries and fallopian tubes
Quite common anomalies of the fallopian tubes are congenital obstruction and various variants of tube underdevelopment, usually combined with other signs of infantilism. Anomalies of the female genital organs that increase the risk of developing an ectopic pregnancy include asymmetrical fallopian tubes. Malformations such as aplasia, complete duplication of tubes, splitting of tubes, blind passages and additional holes in tubes are rarely detected.
Ovarian abnormalities usually occur due to chromosomal abnormalities, combined with birth defects or disruption of the functioning of other organs and systems. Ovarian dysgenesis is observed in Klinefelter syndrome. Agenesis of one or both gonads and complete duplication of the ovaries are among the extremely rare anomalies of the female genital organs. Possible ovarian hypoplasia, usually combined with underdevelopment of other parts of the reproductive system. Cases of ovarian ectopia and the formation of additional gonads adjacent to the main organ have been described.
The development of pregnancy in an abnormal fallopian tube is an indication for emergency tubectomy. With normally functioning ovaries and abnormal tubes, pregnancy is possible through in vitro fertilization of an egg taken during follicle puncture. In cases of ovarian anomaly, it is possible to use reproductive technologies with fertilization
Anomalies of the female genital organs include congenital disorders of the anatomical structure of the genitals in the form of incomplete organogenesis: deviations in size, shape, proportions, symmetry, topography; the presence of formations that are not characteristic of the female individual in the postnatal period. Postnatal delay in the development of correctly formed (in the antenatal period) genital organs is also erroneously classified as anomalies, for example in infantilism. These are purely anatomical disorders, as a rule, accompanied by a functional disorder.
Frequency. Developmental anomalies of the female genital organs account for 4% of all developmental defects (Tolmachevskaya, 1976). According to other authors (Sakuntala-Devi, 1976), among 44,756 patients who passed through an obstetrics and gynecology clinic over 11 years, 102 (0.23%) had anomalies of the uterus and vagina. Naturally, in highly qualified specialized institutions the number of corresponding patients is greater: from 3.9 to 12.4% in relation to all children admitted to the gynecological department. During mass examinations of women, anomalies in the development of the genital organs are detected in 0.9%.
Etiology. Currently, there are 3 groups of causes leading to intrauterine developmental defects: hereditary, exogenous, multifactorial. The occurrence of genital malformations refers to the so-called critical periods of intrauterine development. It is based on the absence of fusion of the caudal sections of the paramesonephric (Müllerian) ducts, deviations in the transformations of the urogenital sinus, as well as the pathological course of gonadal organogenesis (depending on the characteristics of the development of the primary kidney and on the timeliness of migration of gonocytes into the embryonic gonad anlage). The vicious differentiation of the genital organs is only partly (16%) due to genetic reasons, more often at the gene level than at the chromosomal level. Basically, the anomalies under consideration arise in connection with pathological conditions of the intrauterine environment, which, however, are realized through changes in the hereditary apparatus of embryonic cells or accelerate the manifestation of existing genotype defects.
It is significant that mothers of girls who suffer from abnormalities of the genital organs often indicate a pathological course of their pregnancy: early and late toxicosis (25%), malnutrition (18%), infections in the early stages - from 5% to 25%. Developmental anomalies can also be caused by other antenatal damaging factors: occupational hazards, pharmacological and household poisoning, extragenital diseases in the mother: in total, these factors account for up to 20% of the causes of genital abnormalities. Since the damaging factor acts not only (not strictly selectively) on the anlage of the genital organs, but also on other anlages at the same time, then with malformations of the genitals (unilateral agenesis of the kidney), intestines (anal atresia), bones (congenital scoliosis), as well as congenital heart defects and other abnormalities. This circumstance forces the gynecologist to subject girls to more thorough additional urological, surgical and orthopedic examinations.
Agenesis is the absence of an organ and even its rudiment. Aplasia is the absence of part of an organ, atresia is underdevelopment that occurs secondary to an inflammatory process suffered in utero. Most often it manifests itself as a complete absence of a channel or hole. The concept of “gynathresia” refers to various parts of the reproductive tract; this type of underdevelopment occurs in places of anatomical narrowing: the vulva, the opening of the hymen, the external and internal os of the uterus, the mouth of the fallopian tubes. Heterotopia is the presence of cells, groups of cells or tissues in another organ or in those areas of one organ where they are normally absent. Hyperplasia is a condition when an organ grows to excessive size due to an increase in the number and volume of cells, and hyperfunction or hyperplasia does not always develop. Hypoplasia is the underdevelopment and imperfect formation of an organ, the size of which is 2 sigma and deviates from the average. There are simple and dysplastic (with a violation of the structure of the organ) forms of hypoplasia. Animation is the multiplication (usually doubling) of parts or number of organs. Non-separation (fusion) - the absence of separation of organs or their parts that normally exist separately. Persistence is the preservation of rudimentary structures that should disappear in the postnatal period and be reduced. Stenosis is a narrowing of a canal or opening. Ectopia is the displacement of organs or their development in places where they should not be.
Anomalies of ovarian development. The duplication of this paired organ is practically not observed; It is extremely rare to find an additional, third, ovary, equipped with its own ligament. Relatively more often you can see partial or complete unlacing of a functionally defective ovary. If ovarian agenesis (anovaria) is extremely rare (in nonviable fetuses), then the absence of one ovary (monovaria) with a unicornuate uterus is not uncommon. Various gonadal dysgenesis, including the genetic variant (Turner syndrome), are characterized by ovarian hypoplasia. The gonadal parenchyma in these cases is represented by connective tissue rather than germinal elements. In the process of diagnosing and clarifying the degree of hypogonadism and hypogenitalism, one should not forget that genital inferiority can be considered as one of the symptoms of multiple malformations of both genetic and chromosomal origin (Lawrence-Moon, Roberts, Rüdiger syndromes). Anomalies of ovarian development and anomalies of the genital tract do not mutually determine each other and may not be combined, since the gonads and genital ducts are formed asynchronously and from heterogeneous embryonic rudiments. Abnormal ovaries are often located in unusual places, for example in the inguinal canal, which should be taken into account during diagnostic procedures and transsection in this group of patients. To correctly resolve a number of practical issues, it is very important to remember such a feature of abnormal ovaries as the presence in some of them of potential foci of blastomatous growth.
Anomalies in the development of the fallopian tubes. Duplication (on one or both sides) of the fallopian tubes has been described, but more often there is underdevelopment or absence of one fallopian tube, which is usually combined with an abnormal development of the uterus. Atresia of the fallopian tubes as an independent and single defect almost never occurs. All variants of the vicious development of the fallopian tubes, uterus and partly the vagina are due to one of three reasons: underdevelopment of the paramesonephric ducts, impaired recanalization and incomplete fusion of the latter.
Anomalies of uterine development. The most common. Uterus didelphys - duplication of the uterus and vagina when they are located separately. Both reproductive organs are separated by a transverse fold of the peritoneum. This anomaly occurs in the absence of fusion of correctly developed paramesonephric ducts; there is only one ovary on each side. Both uteruses function well, and with the onset of puberty, pregnancy can occur alternately. Treatment is usually not required. Uterus diplex et vagina duplex. These formations are formed in the same way, however, in a certain area they come into contact, and the silt is united by a fibrous-muscular layer. One of the uteruses is often inferior to the other in size and functionally, and on the less pronounced side, atresia of the hymen or internal uterine os may be observed. Uterus bicornis bicoltis. This anomaly is a less pronounced consequence of nonfusion of the paramesonephric tracts. As a result, there is a common vagina, and the rest of the passages are bifurcated. As a rule, the organs on one side are less pronounced than those on the opposite side. The doubling is even less pronounced in an anomaly called uterus bicornis unicoollis. In this case, the paramesonephric ducts merge only in the proximal parts of the middle sections. The bicornuate uterus is almost not manifested in the uterus introrsum arcuatus simplex variant. Uterus bicornis with vestigial horn; its vicious formation is due to a significant lag in the development of one of the paramesonephric ducts. If the rudimentary horn has a cavity, then it is practically important to know whether it communicates with the uterine cavity. A functioning rudimentary horn manifests itself clinically (polymenorrhea, infection, algomenorrhea); an ectopic pregnancy may subsequently occur in it. Histological examination of the removed rudimentary horn often reveals foci of endometriosis (congenital). The occurrence of a rare pathology - uterus unicornis - is possible with deep damage to one of the paramesonephric ducts. In this case, as a rule, one kidney and one ovary are missing. Functionally, the uterus may be complete. Uterus biparpitus solidus rudinentarius vagina solida. This anomaly is also known as “Rokitansky-Küster syndrome.” The vagina and uterus are represented by thin connective tissue cords. Sometimes the rudimentary cords have a lumen lined with single-layer columnar epithelium with an underlying thin layer of endometrial-like stroma without glandular elements.
Anomalies of vaginal development. Vaginal agenesis is the primary complete absence of the vagina. The pathology is based on the loss of the proximal parts of the paramesonephric ducts by the embryo. Since the lower third of the vagina is formed not from the indicated passages, but from the urogenital sinus, even with vaginal agenesis, a slight depression between the labia majora remains (does not exceed 2-3 cm). Vaginal aplasia is the primary absence of part of the vagina, caused by the cessation of sewerage of the developing vaginal tube, which normally ends at 18 weeks. intrauterine development. Secondary absence of the vagina - atresia - is nothing more than a complete or partial closure of the vagina associated with an inflammatory process related to the prenatal period. Sometimes the vagina has a septum that can extend from the fornix to the hymen. This anomaly is rarely combined with a bicornuate uterus. In addition to the longitudinal septum of the vagina, there is a transverse one.
Abnormalities of the hymen, vulva and external genitalia. There is a so-called perforated hymen, which is often complicated (due to poor outflow) by vulvovaginitis.
Atresia is detected with the onset of puberty. Menstrual blood gradually fills the vagina, which passively stretches, forming haematocolpos, the uterus - haematometra, fallopian tubes - haematosalpinx. Deformation of the vulva can be caused by hypo- and epispadias, in particular with hermaphroditism. In addition, deformation is inevitable if the anus opens in the vagina or vestibule (congenital anomaly). Congenital hyperplasia or hypertrophy of the external genitalia is rare. Acquired hypertrophy of the labia minora can occur at the beginning of puberty in response to the increasing level of sex hormones characteristic of this period of time. Masturbation can cause hypertrophy of the labia. A hereditary predisposition to their hypertrophy cannot be ruled out.
Diagnostics. Diagnosis of anomalies of the female genital organs is difficult due to the fact that a number of defects do not manifest themselves in any way, and many of them first make themselves felt only during puberty. These girls exhibit a female phenotype; external examination data, as well as the anthropometric profile, may not deviate from the norm. The detection of genital abnormalities has three peaks: birth, puberty, and the onset (or attempt) of sexual activity. Primary amenorrhea is the leading symptom of genital malformations, and in most cases it is false, due to the impossibility of outflow of menstrual blood due to atresia or aplasia of any area below the internal uterine os. Secondary amenorrhea is less typical; it may indicate pathological formation of the ovaries. Polymenorrhea is characteristic of an accessory horn or various variants of uterine duplication. Another common symptom is the appearance during puberty of monthly increasing abdominal pain, sometimes accompanied by loss of consciousness. Pathological manifestations do not necessarily recur with each menstrual cycle; There are intervals of 1-3 months when menstruation occurs physiologically. However, for quite a long time the girl may not experience any painful sensations. Gradually, the pain takes on the character of intestinal colic, accompanied by nausea and sometimes dysuria. The painful symptom is associated with a violation of the outflow of menstrual blood from the abnormal genital tract, as well as the fact that 70% of girls with genital abnormalities have endometriosis. In some cases, unjustified transection is performed. The observed hyperthermia (37-37.8) is caused by the absorption of pyrogenic substances from places where blood accumulates and (less often) suppuration during stagnation, for example in a rudimentary horn. The above-described painful manifestations can occur against the background of normal menstruation. This is possible with hemiaatresia, i.e., when there is a violation of the outflow from one half of the doubled genital tract, ending in a closed vagina. When palpating the abdomen, it is sometimes possible to identify a “tumor” in the lower abdomen; the boundaries of the tumor are clear (if the upper pole is located not high above the upper edge of the womb, they are unclear). Percussion can also determine dullness of sound in the hypogastrium area. The size of the hematometra is sometimes large, resembling the uterus in late pregnancy. The “tumor” is painless and removable. Peritoneal phenomena can occur with advanced infection, or when excess menstrual blood enters the abdominal cavity, which is observed when atresia is not recognized for a long time and the formation of not only hematocolpos, hematometra, but also hematosalpinx. If hematotra is present, urination and defecation disorders may occur. In every third girl with hematometra caused by gynathresia, a tumor in the abdomen is first discovered in a children's clinic during an examination by a local pediatrician. Examination of the external genitalia is of critical diagnostic importance for hymenal atresia. A translucent cyanotic “tumor” causes the hymen to bulge, and sometimes the entire perineum bulges. Tension in this area is noted. Probing of the vagina and uterus is recommended to identify certain types of anomalies (detection of septa, protrusions, additional passages). The manipulation is often performed under anesthesia, which requires special care when performing it. A bimanual vaginal examination, performed under anesthesia (provided that the shape and size of the opening of the hymen allows it to be performed), helps to detect a number of anomalies, expressed in the doubling of the uterus, the presence of a rudimentary horn; the same enlarged uterus (hematometra) is palpated. During a two-handed examination, attention is often drawn to the excessive expression of transverse folds on the anterior and posterior walls of the vagina, reminiscent of “cockscombs”. This finding suggests the presence of a bicornuate uterus. A two-manual rectal-abdominal wall examination has significant diagnostic value. In girls with no vagina, the uterus often cannot be palpated. In the presence of hematometra and hemotosalpinx, a large, round, elastic, painless “tumor” is detected rectally. At the same time, we must not forget that a dystopic kidney (or kidneys) can be palpated in the pelvis. Vaginoscopy can detect doubling of the cervix, remnants of Gartner's duct, vaginal septum, and the mouth of the fistula. A diagnostic puncture of a suspicious formation is permissible only as the first stage of surgical treatment that has already begun, otherwise such a puncture, performed as an independent and only manipulation, is fraught with infection, bleeding and other complications. Among the diagnostic measures, X-ray examination methods are mandatory. Hysterosalpinography is especially indicated if there is a suspicion of a bicornuate uterus, the presence of a septum in it, as well as with a rudimentary horn, if its lumen communicates with the uterine cavity. Gas pelviography is used for rudimentary horn and is especially useful when it must be differentiated from an ovarian cystoma. Bicontrast gynecography provides valuable information for all types of anomalies of the internal genital organs. Vaginography, urethrography, fistulography are performed for vaginal atresia, hermaphroditism, and congenital fistulas. Intravenous urography is mandatory in almost all cases of genital anomalies. Endoscopic methods (culdoscopy, laparoscopy, cystoscopy, sigmoidoscopy) have a certain significance in the diagnosis of anomalies, especially since with the modern version of their implementation it is possible to take material for a biopsy. However, in a number of cases, diagnostic transection becomes crucial for children, which, if necessary, can be turned into a therapeutic one: gonadectomy, ovarian resection, plastic surgery, excision of rudimentary formations, omentoovariopexy. An attempt to use the determination of hormone levels (LH, FSH, estradiol and other fractions, pregnanediol, testosterone) for diagnostic purposes did not lead to the desired results, since for the most part either normal or fluctuating production of pituitary and ovarian hormones was detected. Genetic methods have not yet taken a leading place in the diagnosis of abnormalities of the genital organs. It is important to indicate the functional inferiority of the reproductive system in mothers and sisters of girls with anomalies in the development of the female genital organs. Determination of sex chromatin and karyotyping in this group of girls does not reveal pathology in 96% of cases. Observed changes in dermatoglyphic parameters (increase in total comb count, number of curls and other characteristics) make us suspect disorders at the gene level.
Treatment. For some types of pathology (saddle uterus, unicornuate uterus), no treatment is required. It is enough just to know about the existing anomaly in order to properly manage pregnancy, childbirth, and perform intrauterine manipulations in the future. For other types of pathology, for example, with Rokitansky-Küster syndrome, treatment is ineffective and comes down to only creating an artificial vagina. In cases of complete atresia or agenesis of the vagina with normally formed internal genital organs, surgical treatment gives a satisfactory effect. An artificial vagina created using one of numerous methods not only provides these women with the opportunity to have sexual activity, but also acts as a channel leading to the release of the vaginal part of the cervix. Atresia of the lower third of the vagina requires a small amount of intervention (blunt access to the lower pole of the vaginal tube, dissection of the vaginal mucosa, evacuation of the contents of the hematocolpos and suturing of the reduced edges of the vaginal mucosa to the epidermis of the vulvar ring). The vestigial or accessory horn is removed during transection. In various cases of duplication of the genital tract with impaired outflow of menstrual blood on one side, maximum excision of the medial wall of the closed vagina is undertaken in order to create a common vagina. In general, the type of surgical intervention for anomalies of the genital structure is determined strictly individually. Often, to treat extensive anomalies of the genitals, two surgical teams are simultaneously formed: one to perform surgery from the perineum and vagina, the other for abdominal access. In case of anomalies of the internal genitalia, transection is a mandatory stage of diagnosis and treatment, since simultaneously with anomalies, tumors of the ovaries and other localizations are often detected. The most favorable results are obtained with atresia of the hymen. A cross-shaped incision (2*2cm) is made in its center and after releasing the main part of the accumulated blood, the edges of an artificially created hole in the hymen are formed using rare single catgut sutures. During the first days, uterine contractions are prescribed. In some girls, in order to prevent recurrence of atresia, the newly created hole has to be bougiened and tamponed. By the way, with early diagnosis of hymenal atresia during a similar operation or diagnostic puncture, usually preceding surgery, it is possible to obtain mucus rather than blood.
Forecast. In many cases, after surgical correction of the anomaly, the ability to conceive is not impaired. Often there are signs of a threatening miscarriage, but after the danger is eliminated, the pregnancy can end in spontaneous birth. During childbirth, labor abnormalities and a tendency to hypotonic bleeding are almost always observed. Caesarean sections are performed in 50%.
Bibliography
- 1. Gynecology of children and adolescents. Kobozeva N.V. Kuznetsova M.N. Gurkin Yu.A. Medicine, 1988
- 2. Gynecology of adolescents. Gurkin Yu. A. Guide for doctors.
- 3. Gynecology. Textbook, ed. Savelyeva G. M.
Ovarian developmental abnormalities
1. Agenesis (aplasia) of the ovary- absence of ovaries. Anovaria is the absence of two ovaries.
2. Ovarian hypoplasia- underdevelopment of one or both ovaries.
3. Ovarian hyperplasia- early maturation of glandular tissue and its functioning.
4. Accessory ovary- sometimes found near the main ovaries in the folds of the peritoneum. Differs in small size.
5. Ectopic ovary- displacement of the ovary from its usual place in the pelvic cavity. It can be located in the thickness of the labia, at the entrance to the inguinal canal, in the canal itself.
Abnormalities of the uterus
1. Uterine agenesis– complete absence of the uterus due to its non-laying, is rare.
2. Uterine aplasia– congenital absence of the uterus. The uterus usually looks like one or two rudimentary muscle ridges. The frequency ranges from 1:4000-5000 to 1:5000-20000 newborn girls. Often combined with vaginal aplasia. Possible combination with developmental anomalies of other organs: spinal column (18.3%), heart (4.6%), teeth (9.0%), gastrointestinal tract (4.6%), urinary organs (33.4%). There are 3 possible options for aplasia:
A) a rudimentary uterus is defined as a cylindrical formation located in the center of the small pelvis, on the right or left, measuring 2.5-3.0 x 2.0-1.5 cm;
B) the rudimentary uterus has the form of two muscular ridges located near the wall in the pelvic cavity, each measuring 2.5 x 1.5 x 2.5 cm;
C) muscle ridges (rudiments of the uterus are absent.
3. Hypoplasia of the uterus (syn.: uterine infantilism)– the uterus is reduced in size, has an excessive anterior bend and a conical neck. There are 3 degrees:
A) embryonic uterus (syn.: rudimentary uterus)– an underdeveloped uterus (up to 3 cm long), not divided into a cervix and body, sometimes without a cavity;
B) infantile uterus– uterus of reduced size (3-5.5 cm long) with a conical elongated neck and excessive anteflexion;
IN) teenage uterus– length 5.5-7 cm.
4. Duplication of the uterus– occurs during embryogenesis as a result of the isolated development of the paramesonephric ducts, while the uterus and vagina develop as a paired organ. There are several doubling options:
A) Double uterus (uterus didelphys)– the presence of two separate unicornuate uteruses, each of which is connected to the corresponding part of the bifurcated vagina, is due to the non-fusion of correctly developed paramesonephric (Müllerian) ducts along their entire length. Both reproductive organs are separated by a transverse fold of the peritoneum. There is one ovary and one fallopian tube on each side.
B) Duplication of the uterus (uterus duplex, synonym: bifurcation of the uterine body)– in a certain area of the uterus and vagina they touch or are united by a fibromuscular layer, usually the cervix and both vaginas are fused. There may be options: one of the vaginas may be closed, one of the uteruses may not have communication with the vagina. One of the uteruses is usually smaller in size and its functional activity is reduced. On the part of the reduced uterus, aplasia of part of the vagina or cervix may be observed.
IN) Uterus bicornis bicollis– the uterus is externally bicornuate with two cervixes, but the vagina is divided by a longitudinal septum.
5. Uterus bicornuate (uterus bicornus)– division of the uterine body into 2 parts in the presence of one cervix, without division of the vagina. Division into parts begins more or less high, but in the lower parts of the uterus they always merge. The division into 2 horns begins in the area of the uterine body in such a way that both horns diverge in opposite directions at a greater or lesser angle. With pronounced splitting into two parts, two one-horned uteruses are determined. Often it consists of two rudimentary and unfused horns that have no cavities. Formed at the 10-14th week of intrauterine development as a result of incomplete or very low fusion of the paramesonephric (Müllerian) ducts. According to the degree of severity, there are 3 forms:
A) Full form – the rarest option, the division of the uterus into 2 horns begins almost at the level of the uterosacral ligaments. Hysteroscopy shows that two separate hemicavities begin from the internal os, each of which has only one orifice of the fallopian tube.
B) Incomplete form – division into 2 horns is observed only in the upper third of the uterine body; As a rule, the size and shape of the uterine horns are not the same. Hysteroscopy reveals one cervical canal, but closer to the fundus of the uterus there are two hemicavities. In each half of the uterine body, only one orifice of the fallopian tube is noted.
IN) Saddle shape (syn.: saddle uterus, uterus arcuatus)– division of the body of the uterus into 2 horns only in the fundus with the formation of a small depression on the outer surface in the form of a saddle (the fundus of the uterus does not have the usual rounding, is pressed or concave inward). During hysteroscopy, both mouths of the fallopian tubes are visible, the bottom seems to protrude into the uterine cavity in the form of a ridge.
6. Uterus unicornuate (uterus unicornus)– the shape of the uterus with partial reduction of one half. The result of atrophy of one of the Müllerian ducts. A distinctive feature of the one-horned uterus is the absence of its bottom in the anatomical sense. In 31.7% of cases it is combined with developmental anomalies of the urinary organs. Occurs in 1-2% of cases among malformations of the uterus and vagina.
7. Divided uterus (syn.: bifid uterus, intrauterine septum)– observed in the presence of a septum in the uterine cavity, making it two-chambered. Frequency – 46% of cases of the total number of uterine malformations. The intrauterine septum can be thin, thick, on a wide base (in the form of a triangle). There are 2 forms:
A) Uterus septus– full form, completely divided uterus.
B) Uterus subseptus– incomplete form, partially divided uterus, septum length - 1-4 cm.