Embryology and malformations of the male genital organs. Sexual development in boys - the norm and deviations Male and female development

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EMBRYOGENESIS

Internal and external genital organs are formed in men in the embryonic period, in puberty - their development and improvement continues, ending by 18-20 years.

Further, for 25-30 years, the normal functioning of the gonads is maintained, followed by a gradual fading of their function and reverse development.

The sex of a person is determined primarily by the set of sex chromosomes (chromosomal sex), on which the formation and construction of the sex glands depend, affecting the hormonal sex, which, in turn, determines the structure of the genital organs. The upbringing of the individual, the mental and civil sex, depends on the latter fact.

Intrauterine development of the genitals occurs in accordance with the genetic (chromosomal) sex of the embryo. The chromosome set determines directed sexual evolution and an adult individual. The human reproductive cell (male or female) contains 23 chromosomes (haploid set). Genetic, or chromosomal, sex is determined at the time of fertilization and depends on what chromosomal material the egg receives, which normally has 22 autosomes and a sex X chromosome, when it fuses with a sperm containing 22 autosomes and a sex X or Y chromosome ...

When the egg cell merges with the sperm carrying the X chromosome, the female genotype is formed - 46 (XX), the primary sex gland of the embryo will be laid according to the female type (ovaries). When an egg is fertilized with a sperm containing a sex Y chromosome, the embryo's primary sex gland will develop in a male pattern (testicles). The normal male genotype is therefore determined by a set of 44 autosomal and 2 sex chromosomes X and Y.

The structure of the gonads determines the gonadal sex.

The ovaries in the embryonic period are functionally inactive, and differentiation according to the female type is passive, without requiring control from the gonads. The testicle of the embryo very early becomes an active endocrine organ. Under the influence of androgens produced by the embryonic testicles, the development and formation of the internal and external genital organs of the male type occurs. The vas deferens, epididymis, seminal vesicles, prostate gland form and develop; the scrotum, penis, urethra are formed, the testicles gradually descend into the scrotum.

In the absence of androgens, violation of their production or insensitivity of peripheral receptors to them in the process of embryogenesis, the external genital organs can be formed according to the female type, or their various anomalies develop. From the moment of birth, sex is determined by the structure of the external genital organs, after which it is fixed by psycho-affective sexualization in the first 18-30 months of a child's life and reinforced throughout the rest of his life.

7. Scheme of sexual differentiation of the embryo: a - male embryo after 11 weeks; b - 6-week embryo; c - female embryo after 11 weeks; 1 - prostate gland; 2 - Cooper's glands; 3 - urethra; 4 - testicle; 5 - epididymis; 6 - seminal vesicles; 7 - canal aviaries; 8 - primary duct gland; 9 - wolffian body; 10 - Müllerian canal; 11 - merged Müllerian canal; 12 - vagina; 13 - ovary; 14 - gartner's strand; 15 - fallopian tube; 16-uterus.

During puberty, the testes actively produce testosterone, which contributes to the appearance of secondary sexual characteristics, and asserts the male hormonal sex. By this time, the civil sex is also formed, characterized by external signs of prla, clothing, manner, social behavior, orientation of sexual desire.

Development of the sex glands.

The genitals are closely related to the urinary organs and are formed from the primary kidney of the embryo - mesonephros. Due to the proliferation of the multilayer epithelial cover covering the mesonephros, an embryonic fold is formed - the epithelial primordium of the primary sex gland. It penetrates into the depths of the mesonephros, forming the primary sex cords, consisting of the primary sex cells - gonocytes. (precursors of sperm), connective tissue cells that will secrete sex hormones, as well as undifferentiated cells that play a trophic and support role.

From the 7th week, the tissue structures of the primary sex gland of the fetus begin to differentiate into male (testicles) or female (ovaries) sex glands. With the development of the testicle from the 8th week, the primary sex cords actively grow and turn into seminiferous tubules with the formation of gaps in them.

In the lumen of the seminiferous tubules there are sex cells - spermatogonia, which are formed from gonocytes and in the future will initiate spermatogenesis. Spermatogonia are located on sustentocytes that carry out a trophic function. Interstitial cells are formed from the connective tissue rudiments of mesonephros, which are capable of producing male sex hormones during a certain period of embryogenesis. The development of the primary sex gland into the testes is completed by the 60th day of intrauterine development of the fetus.

It has been established that androstandione, androsterone and other androgenic steroids are secreted by the fetal testis. The release of testosterone is more pronounced at 9-15 weeks. Already at the 10th week of development, the testosterone level in the testes of the human fetus is 4 times higher than in the ovaries. At 13-15 weeks of embryonic development, the testosterone content in the testicles exceeds that in the ovary by 1000 times. The further formation of internal and external genital organs depends on the production of testosterone.

Development of internal genital organs.

With the development of the pelvic kidney in higher vertebrates, the primary kidney loses its function as an excretory organ. At the end of the 2nd month of intrauterine development, the excretory canal of the primary kidney is split into 2 ducts: ductus mesonephricus (wolf's duct) and ductus paramesonepliricus (Müllerian duct - Fig. 7). From the ducti mesonephrici the vas deferens develop, from the ducti paramesonephrici the fallopian tube is formed. Male sex hormones secreted by the embryonic testicles contribute to the isolation and development of ducti mesonephrici. In addition, the testes secrete some other non-steroidal factors, under the influence of which regression and atrophy of the Müllerian canals occur. The upper part of the ducti mesonephrici (after the reverse development of the primary kidney) connects with the seminiferous tubules of the testicle and forms the vas deferens, the reticulum of the testicle, the canal of the epididymis.

8. Diagram of the differentiation of the external genital organs of the fetus (left - girl, right - boy), and - 2-3 months; b-c - 3-4 months; g-d - at the moment of birth; 1 - genital fold; 2 - anus; 3 - sex roller; 4 - genital gap; 5 - genital tubercle; 6-urethral fold; 7 - scrotal roller; 8 - urethral fissure; 9 - genital process; 10 - fold of the inner labia; 11 - roller of the outer labia; 12 - vulvar chips; 13 - the seam of the scrotum; 14 - scrotum; 15 - urethral suture; 16 - penis; 17 - small labia; 18 - the entrance to the vagina; 19 - opening of the urethra; 20 - large labia; 21 - clitoris.

The middle part of the ducti mesonephrici is converted into the vas deferens. The lower section of the ducti mesonephrici (adjacent to the urogenital sinus) expands ampouloidly, forms a protrusion, from which the seminal vesicle is formed. The lowest part of the ducti mesoneplirici, which opens into the urogenital sinus, becomes the ejaculatory duct. The pelvic part of the urogenital sinus is transformed into the prostate and membranous parts of the urethra and gives rise to the rudiments of the prostate gland, growing into the surrounding mesenchyme in the form of continuous cords. Muscular and connective tissue elements of the gland develop from the mesenchyme.

Gaps in the prostate gland appear after birth, by the time of puberty. Ductus paramesonepliricus disappears with the development of the male body, only their rudiments remain: the upper part is the process of the testicle and the lower part, from which the male uterus is formed - the blind appendage of the prostate urethra on the seminal tubercle.

Development of the external genital organs.

The external genitals are formed in both sexes from the genital tubercle and cloacal fissure. The common cloaca, even at the early stages of development of the embryo, is divided by a septum descending from above into 2 sections: the anterior (anus) and the anterior (urogenital fissure, into which the Wolffian and Müllerian ducts exit). From the genitourinary fissure, the bladder and urethra are formed, as well as the ureters and renal pelvis. At the neutral stage, the external genital organs are represented by the genital tubercle of the urogenital fissure and two pairs of folds covering it (Fig. 8).

The internal ones are called the genital folds, the external ones are called the genital ridges. From the 4th month of embryonic life, the differentiation of the external genital organs begins. In a male embryo, under the influence of androgens secreted by the testicle, the genital tubercle grows, and the head develops from it, and later the cavernous bodies of the penis.

The genital folds, surrounding the urogenital opening, extend to the lower part of the genital tubercle, form the urethral groove. The edges of the genital folds, growing together along the urethral groove, form the urethra, around which the cavernous body of the urethra is formed from the mesenchyme of the genital tubercle.

The genital ridges in men, connecting along their entire length, form the skin part of the scrotum. The testicles descend into it by the time the fetus is born. Chromosomal abnormalities (quantitative, structural, gene mutations), embryotoxic effects of endogenous and exogenous nature can lead to the development of anomalies of the internal and external genital organs. Testicular anomalies include abnormalities in position as well as quantitative and structural.

Testicular malformations testicular position anomalies (cryptorchidism)

In the process of embryogenesis, the testicles are laid together with the primary kidney, and by the end of the 3rd month they migrate to the iliac region. When displaced, the testicle protrudes into the abdominal cavity, pushing the peritoneum in front of it, which forms 2 folds. The cranial fold of the peritoneum covers the vessels and nerves that feed the testicle. The caudal fold forms the vaginal process of the peritoneum and covers the directional cord with its posterior leaf, which consists mainly of smooth muscle fibers. By the end of the 7th month, the testicle approaches the inner ring of the inguinal canal, where the guide cord penetrates before.

An active role in the movement of the testicle into the scrotum is played by the contractile ability of the directional cord, tension of the abdominal muscles, and increased intra-abdominal pressure. At the 8th month, the testicle passes the inguinal canal, while the lumen of the vaginal process of the peritoneum is widely communicated with the abdominal cavity. At the 9th month, the testicle descends into the scrotum. The guide cord is reduced, becoming a ligament connecting the caudal pole of the testicle with the scrotal floor. The processus vaginalis of the peritoneum is obliterated in the proximal region, and the abdominal cavity is delimited from the intershell sinus of the testicle.

The absence of one or both testicles in the scrotum is called cryptorchidism (from the Greek hryptos; - hidden and orxis - testicle). Cryptorchidism is determined in 10-20% of newborns, in 2-3% of one-year-old children, in 1% in puberty and only in 0.2-0.3% of adult men. This statistics is due to the fact that incomplete testicular prolapse in newborns in most cases is eliminated in the first weeks of extrauterine development. Up to 1 year, spontaneous descent of the testicle is noted in another 70% of children with cryptorchidism. In the future, the possibility of independent displacement of the testicles into the scrotum exists until puberty.

Etiology and pathogenesis.

The delayed migration of the testicle into the scrotum can be caused by endocrine disorders, mechanical causes, dysgenesis of the gonads, hereditary and genetic factors and a combination of these factors. In the occurrence of cryptorchidism, an important role is played by the endocrine factor. Hormonal dnscorrelations in pregnant women, impaired endocrine function of the testicles, thyroid gland, and pituitary gland of the embryo can cause a delay in the movement of the testicles into the scrotum. These reasons are important in bilateral kriltorchidism.

With unilateral testicular retention, mechanical factors play a certain role, among which the narrowness of the inguinal canal is revealed during the operation; lack of a tunnel into the scrotum; shortening of the spermatic cord, the vaginal process of the peritoneum, the vessels that feed the testicle; underdevelopment of the guiding ligament; peritoneal adhesions in the area of \u200b\u200bthe internal opening of the inguinal canal, etc. The listed changes can occur as a result of past diseases, injuries during pregnancy, but can also be secondary in nature against the background of hormonal disorders in the intrauterine period of fetal development.

Bilateral abdominal cryptorchidism is often combined with testicular dysgenesis. Histological studies in almost half of the observations establish primary hypoplasia of undescended testicles. Therefore, in some patients, despite early descent into the scrotum, the testicles remain defective. It is likely that an incorrectly formed testicle in the embryonic period predisposes to the development of cryptorchidism due to impaired endocrine function. In favor of testicular dysgenesis is evidenced by a large number of anomalies of the epididymis and the vas deferens, which are found in cryptorchidism.

In some cases, undescended testicles are hereditary and genetic in nature. Familial cryptorchidism is observed in men of several generations. Doctors who treat cryptorchidism should pay attention to studying the families of sick boys.

Classification.

Until now, there is no generally accepted classification of cryptorchidism. The classification of S.L. Gorelik, Yu.D. Mirles (1968) most corresponds to the correct interpretation of the terminology of this disease. We use our own classification of cryptorchidism and consider it convenient for use in practical work.
Cryptorchidism can be unilateral and bilateral. There are 4 types of cryptorchidism: caused by retention, ectopia, as well as false and acquired.

9. Variants of testicular descent (diagram). 1.4 - the usual way of descent of the testicle; 2 - retention of the testicle in the abdominal cavity; 3 - testicle retention in the inguinal canal; 5-8 - testicular ectopia, deviation from the usual path to the scrotum; 7 - penile ectopia; 8 - femoral ectopia.

Cryptorchidism due to retention (retention) of the testicle.

The retention can be abdominal, inguinal and combined. With abdominal retention, one or both testicles may be located in the lumbar or iliac region; with the inguinal - in the inguinal canal. With combined retention, the testicle is found on one side in the inguinal canal, and on the other side is in the abdominal cavity (Fig. 9).

Cryptorchidism due to ectopia (unusual location of a descended testicle).

Ectopia is perineal, pubic, femoral, penile, transverse, etc. Ectopia occurs due to the deviation of the testicle from the usual route to the scrotum. In this case, the testicle can be located on the pubis, perineum, inner thigh, at the base of the penis. With transverse ectopia, both testicles are located in one of the halves of the scrotum.

False cryptorchidism (the so-called migratory testicle).

The testicle can temporarily, under the influence of cold or physical exertion, migrate into the inguinal canal and even into the abdominal cavity. When the muscles are warmed and relaxed, it returns to the scrotum. With false cryptorchidism, the scrotum is always well developed, with pronounced folding and a noticeable median suture, the inguinal ring is somewhat expanded.

Acquired cryptorchidism.

Most often, after injury, the testicle can go into the abdominal cavity or inguinal canal. A migratory testicle is predisposed to this, in which the inguinal canal is wide enough. In other cases, migration of the testicle into the abdominal cavity contributes to its atrophy.

Diagnosis of cryptorchidism is based on the analysis of complaints and examination of the patient. The main symptoms are underdevelopment, scrotal asymmetry, absence of one or both testicles in the scrotum. Patients often complain of aching pain in the groin or abdomen. With cryptorchidism caused by inguinal retention or ectopia, pain appears at an early age due to frequent injury, infringement, testicular torsion. With abdominal testicular retention, pain, as a rule, joins only at puberty. It can worsen with physical exertion, stool retention, sexual arousal.

Many patients have a combination of cryptorchidism with an inguinal hernia. Therefore, patients should be examined lying down, calm and with abdominal tension. When straining into the inguinal canal, the hernial sac can descend along with the testicle, which becomes available for examination.

If it is not possible to probe the testicle in the inguinal canal, then the places of possible ectopia should be carefully examined and palpated. Only with the exclusion of unusual localization of the testicle can the presence of abdominal retention be suspected. In 5-10% of patients, especially with bilateral cryptorchidism, signs of endocrine insufficiency may be observed (eunuchoid physique, obesity, underdevelopment of the penis, female-type hair growth, gynecomastia).

However, these symptoms are more characteristic of anorchism. In some patients, there is a delay in sexual development. Abdominal bilateral testicular retention should be differentiated from anorchism, and unilateral - from monorchism, which is often quite difficult.

At present, magnetic resonance imaging, ultrasound scanning, and testicular scintigraphy after the administration of Tc compounds are successfully used for this. When scintigraphy using a gamma camera, it is possible to determine not only the localization and size of the testicle, but also its functional state. Valuable information can be provided by angiography: examination of the abdominal aorta to detect the testicular artery, as well as superselective probing of the internal testicular vein with performing venography of the undescended testis. In doubtful cases, an operative revision of the groin and retroperitoneal space is shown.

With various types of cryptorchidism, a testicle located in unusual conditions for it, a number of unfavorable factors act; fever, constant trauma, malnutrition, as well as hyperstimulation from the pituitary gland. These conditions lead to the development of atrophic processes in the testicle, to a violation of spermatogenesis and can cause its malignant transformation. In cryptorchidism, testicular entrapment or torsion may also occur.

Signs of these complications are the sudden appearance of pain in the impacted or ectopic testicle, swelling, and in rare cases, an increase in body temperature. If there is a suspicion of a circle or infringement, urgent surgery is needed to prevent necrotic changes in the testicle.

Treatment of cryptorchidism can be conservative, operative and combined. Conservative treatment should be aimed at improving the functional state of the testicle and correcting endocrine disorders that often accompany crnptorchidism. Therapy can be carried out in all cases as a preoperative preparation in patients with hormonal disorders, and also take place in the postoperative period.

Treatment begins at 4-5 years of age. Vitamin preparations are widely used. Tocopherol acetate (vitamin E) regulates histobiochemical processes in glandulocytes and epithelium of the testicular tubules by stimulating the hypothalamic-pituitary system. Retinol (vitamin A) activates the processes of cell regeneration in the testes, and also participates in the formation of the nuclear structures of the spermatogenic epithelium. Vitamins C, P, B, improve the redox processes in tissues, are of great importance for the normal functioning of the endocrine glands of the central and peripheral nervous system.

I.F. Yunda (1981) recommends starting the treatment of true cryptorchidism immediately after the birth of a child by administering tocopherol acetate intramuscularly to a nursing mother at 200-300 mg / day. At the age of over 1 month, the child is given tocopherol acetate in a mixture of 5-10 mg / day in 2-3 doses for 1 1/2-2 months. With a monthly break, the course of treatment is repeated 3-4 times a year: The nursing mother is prescribed multivitamins, Great importance should be attached to the nutrition of the child. Food should contain sufficient amounts of protein, fat and carbohydrates.

With reduced nutrition, you can treat with Nerobolil, which belongs to anabolic steroids, stimulates protein synthesis in the body, improves metabolic processes in the accessory gonads. In case of excessive nutrition, obesity, it is recommended to use thyroidin, which increases tissue respiration, improves metabolism in the body, activates the antitoxic function of the liver, renal excretory capacity, normalizes the functions of the thyroid and gonads.

These drugs are prescribed depending on the age, individual characteristics and condition of the patient. Thyroidin tablets are recommended at 0.005 g at the age of 5 years, 0.05 g - at the age of 15 1-2 times a day for 15-25 days. Nerobolil tablets are prescribed: from 3 mg 1 time per day at the age of 5 years, up to 5 mg 1-2 times a day at the age of 15 years (for 20-30 days).

The retracted testicle is characterized by a reduced ability to produce testosterone, which, with a bilateral, and often with a unilateral process, is accompanied by hypoandrogenemia. To stimulate the function of the testicular interreticular cells, therapy with chorionic gonadotropin or its analogue, containing predominantly LH, is performed. An increase in testosterone production by interstitial cells can promote prolapse of a retained testicle. Depending on age, intramuscularly 250, 500 or 1000 IU of chorionic gonadotropin (choriogonin) is injected 1 to 3 times a week, for a course of treatment 6-18 injections. M.G. Georgieva (1969) recommends injecting choriogonin at 500-700 IU once a day for 3 days into the inguinal canal from the side of the unstitched testicle, which, in addition to the usual, has a local depresore-lyzing effect.

With severe aidrogenic insufficiency, the combined use of Nerobolil (Nerobol) and Choriogonin in doses appropriate for age is possible. During puberty, with clear signs of hypogonadism, it is advisable to prescribe intramuscular testosterone injections of 10-20 mg every other day (for a course of 15-20 injections). After that, treatment with choriogonin 1000 IU intramuscularly 3 times a week is carried out (for a course of 12 injections).

The main method of treatment of cryptorchidism remains operative (orchnepexy). We believe that it is advisable to carry out orchipexy at the age of 5-6 years, by the time the child enters school. Earlier surgical treatment, obviously, does not make sense, since at this age the vascular system and spermatic cord have not yet formed.

There are many ways to bring the testicles down into the scrotum. But they all differ ultimately only in the methods of fixation.

The operation is performed under general anesthesia.

The incision is made in the groin area, as in a hernia repair. After opening the anterior wall of the inguinal canal, the testicle is found. The main method of bringing down the testicle into the scrotum is the mobilization of the spermatic cord (Fig. 10, a). In this case, it is imperative to separate the uncultivated vaginal process of the peritoneum from it (Fig. 10, b). In the presence of a hernia, the processus vaginalis turns into a hernial sac. In this case, it should be opened, then with the help of dissection, dissect in the transverse direction the peritoneum covering the spermatic cord, and, removing it from the spermatic cord, isolate, suture and bandage the neck of the hernial sac.

After that, you should use your finger to penetrate the inner ring of the inguinal canal, stupidly embroider it in the medial direction and separate the peritoneum from the spermatic cord. These manipulations, in most cases, contribute to the descent of the testicle into the scrotum. Recommendations to cross the testicular artery to lengthen the spermatic cord should be critically considered, as this can lead to testicular atrophy due to malnutrition. However, with a short vascular pedicle, autotransplantation of the testicle into the scrotum is possible using its lower epigastric artery for arterialization. Testicular transplantation to the iliac vessels is less favorable.

In the corresponding half of the scrotum, pushing the tissue apart, create a bed for the testicle. In adults, the testicle is often fixed in the scrotum with a thick silk ligature stitched through its mobilized shells, brought out through the bottom of the scrotum and attached by means of an elastic rubber traction to a special cuff worn on the upper third of the lower leg. The operation ends with plastic surgery of the inguinal canal according to the method of Martynov or Kimbarovsky.

10. Mobilization of the spermatic cord and testis as a single block together with the vaginal process of the peritoneum (a); mobilization of the spermatic cord by excretion of the vaginal process of the peritoneum and hernial sac (b).

In children, orchipexy can be performed in 2 stages using the Torek-Herzen method and modifications. After mobilization of the family cord, the testicle is passed into the corresponding half of the scrotum. Through an incision in the bottom of the scrotum and skin of the thigh, the testicle is brought and sutured to the wide fascia, the thigh. Then the edges of the scrotum and thigh skin incisions are sutured over the testicle. The leg is placed on the Beler splint.

Patients are discharged 10-12 days after the operation. The second stage of the operation is performed after 2-3 months. It consists in excision of the skin anastomosis and suturing of small wounds on the thigh and scrotum.

The operation for ectopia is quite simple due to the considerable length of the spermatic cord. Transverse testicular ectopia does not require treatment.

With bilateral retention, the issue is decided individually, taking into account the patient's complaints and the safety of one of the testicles. Preference should be given to separate descent of the testicles. In doing so, we start with a less complex surgical intervention.

The prognosis for cryptorchidism due to testicular retention improves after surgery. Infertility is cured in 80% of operated patients with unilateral cryptorchidism and in 30% with bilateral cryptorchidism.

CELL NUMBER ANOMALIES

The reason for the disruption of the normal course of embryogenesis of the gonads can be chromosomal abnormalities (structural or quantitative), disturbances in the course of differentiation of the gonads in the early stages of embryonic development due to severe infectious diseases, intoxication, alimentary dystrophy or hormonal changes in a pregnant woman. Purely quantitative testicular abnormalities are extremely rare, in most cases they are combined with their structural changes.

Polyorchism.

More than 2 testicles are rare abnormalities. Described 36 cases of polyorchism.

An accessory testicle may have its own epididymis and vas deferens. The testicle and epididymis are usually underdeveloped. Palpation is insufficient to confirm the presence of an additional testis, as testicular tumors, accessory epididymis, cysts, and other intrascrotal growths may be mistaken for an additional testicle. The duplicated testicles can be located in the abdominal cavity and undergo degenerative changes. Given the tendency of hypoplastic testicles to malignant degeneration, surgical removal of the accessory testicle with the reduction of the normal testis in the presence of cryptorchidism is indicated.

Synorchidism.

Intra-abdominal fusion of the testicles is extremely rare, which prevents them from descending into the scrotum. In this case, no hormonal disorders are detected, which distinguishes this pathological condition from anorchism and bilateral abdominal retention of the testicles. Diagnostics is based on ultrasound scanning and operative revision of the retroperitoneal space.

Monorchism (unilateral testicular agenesis) is a congenital anomaly characterized by the presence of one testicle.

This anomaly occurs as a result of a violation of the embryonic anlage of the primary kidney on one side, from which the gonad is formed, therefore, monorchism is often combined with congenital aplasia of the kidney, the absence of the epididymis and the vas deferens, there is an underdevelopment of the scrotum on the corresponding side. The presence of one normal testicle is not manifested by disorders of spermatogenesis and endocrine disorders. If the only testicle does not descend into the scrotum or is in a rudimentary state, then there are signs of hypogonadism.

Diagnosis should be made by angiography, testicular scintigraphy, or by revision of the retroperitoneal space and abdomen.

Treatment.

With hypoplasia of a single testicle, androgen replacement therapy is indicated, especially during puberty. Such therapy will promote the normal development of the genitals.

Anorchism (gonadal agenesis) is a congenital absence of testicles in an individual with a 46 XY karyotype.

Due to the fact that the testes do not secrete androgens in the embryonic period, the genitals develop according to the female type or have a rudimentary structure. Much less often, the external genital organs develop according to the male type. In this case, there is a eunuchoid physique, the absence of the epididymis, the vas deferens, the prostate gland; the scrotum is rudimentary.

The final diagnosis is made after excluding bilateral abdominal testicular retention. For this, radionuclide studies and testicular scintigraphy can be performed after the administration of Tc compounds. After intravenous administration of the drug on a gamma camera, the localization and nature of cryptorchidism are determined. With anorchism, there will be no local accumulation of the drug. You can test with choriogonin for the presence of testicular androgens in the blood. In doubtful cases, an operative revision of the abdominal cavity and retroperitoneal space is indicated.

Treatment.

With anorchism, sex hormone replacement therapy is carried out, depending on the structure of the external genital organs, and the morphotype of the patient.Androgen therapy includes the appointment of methyltestoeterone, andriol in tablets 3 times a day or testosterone propionate 50 mg (1 ml of 5% oil solution) intramuscularly daily. In the future, you can use drugs with prolonged action: sustanon-250, omnodren-250, testenat. All of them are injected intramuscularly in 1 ml 1 time in 2-3 weeks. Transplantation of mature testicles on a vascular pedicle is used, as well as free testicular transplantation of fetuses and newborns.

Feminizing therapy is performed during puberty. With a sharp underdevelopment of secondary sexual characteristics, a 0.1% oil solution of zeta-radiol dipropionate is prescribed, 1 ml intramuscularly 1 time in 7-10 days. Treatment lasts 3-4 months to stimulate secondary sexual characteristics, after which they switch to cyclic therapy. Estradiol dipropioate is prescribed in 1 ml of 0.1% oil solution 1 time in 3 days, 5-7 intramuscular injections. Together with the last injection, progesterone (1 ml of a 1% oil solution) is injected and then continue to be administered intramuscularly for 7 days in a row. Such courses of cyclic therapy are repeated 4-6 times.

ANOMALIES OF THE TESTIC STRUCTURE

Hermaphroditism (bisexuality) is manifested by the presence in one individual of signs of both sexes.

Distinguish between true and false hermaphroditism. With true hermaphroditism, elements of both testicular and ovarian tissue develop in the gonads. The sex gland can be mixed (ovotest), or, along with the ovary (usually on the left), there is a testicle on the other side. Violation of the differentiation of the gonads is due to chromosomal mosaics XX / XY; XX / XXY; XX / XXYY, etc., but also occur in the 46XX and 46XY karyotype.

Gonadal tissue develops unevenly.

On the side where ovarian tissue predominantly develops, derivatives of ducti paramesonephrici (uterus, tubes) remain. On the side where the testicle is formed, derivatives of the duett mesonephrici (vas deferens, epididymis) remain. The external genital organs have a dual structure with a predominance of male or female sexual characteristics. The morphotype of patients is determined by the prevalence of the hormonal activity of one of the gonads during puberty. The penis is developed, in the presence of hypospadias, an underdeveloped vagina is located under it. Cyclical discharge of blood from the vagina or urogenital sinus is often observed.

The mammary glands develop.

The mental sex of patients is determined more often by education, and not by the structure of the external genital organs. Depending on the structure of the internal and external genital organs, corrective surgical treatment is performed, as well as therapy with female or male hormones. False male hermaphroditism is observed in individuals with a 46XY karyotype, in whom, in the presence of testes, the external genital organs develop in a female or intersex pattern. The causes of false male hermaphroditism can be hormonal disorders during pregnancy, toxoplasmosis, and intoxication.

A number of genetically determined diseases also lead to this anomaly of the testicles, of which the most famous is the syndrome of feminizing testicles.

Feminizing testicular syndrome.

This anomaly develops in individuals with a male 46XY karyotype and a female phenotype. It is caused by insensitivity of peripheral tissues to androgens. The external genital organs are developed according to the female type. Patients have no uterus, fallopian tubes, the vagina is underdeveloped, ends blindly. The mammary glands are well developed. The testicles can be located in the thickness of the labia majora, in the inguinal canals, in the abdominal cavity.

The seminiferous tubules are underdeveloped, the interstitial tissue is hyperplastic. The testes produce normal amounts of androgens and an increased amount of estrogen. The disease is genetically determined, transmitted by a healthy woman, a carrier of a recessive gene, to half of her sons. Treatment by administration of exogenous androgens does not induce virilization. The testicles are retained as they are the source of estrogen. Feminizing hormonal therapy is performed (see Anorchism).

Klinefelter's syndrome (seminiferous tubule dysgenesis) was described in 1942. The disease is caused by the presence of at least one additional X chromosome in the complex of sex chromosomes. The main form of the 47XXY karyotype was established by P. Jacobs and 1. Strong in 1959. Other chromosomal variants of this syndrome - XXXY, XXXXY, XXYY, as well as mosaic forms of the XY / XXY type, etc. are observed. The frequency of the syndrome among newborn boys reaches 2.5 : 1000. The disease manifests itself during puberty in relatively normal boys. Adult men see a doctor for infertility.

The clinical picture is characterized by insufficient development of secondary sexual characteristics: high growth, eunuchoid physique, small testicles, a normally developed or reduced penis, scanty hair growth on the egg and female hair on the pubis.In 50% of patients, gynecomastia is detected. The clinical manifestation of androgen deficiency is due to impaired tissue reception of testosterone. Sometimes there is a varying degree of mental underdevelopment (more aggravated in patients with a large number of X chromosomes). When examining the ejaculate, azoospermia is detected. The presence of X-sex chromatin in the nuclei of the cells of the oral mucosa is established.

Testicular biopsy reveals aplasia of the spermatogenic epithelium with hyalinosis of the seminiferous tubules and hyperplasia of interstitial cells. The hormonal picture is characterized by low testosterone levels and high levels of FSH and LH in the blood plasma.

Treatment consists in the appointment of testosterone and other androgens, vitamin therapy. However, replacement therapy is not effective enough due to a violation of the reception of androgens by target cells of the accessory gonads, genitals and other tissues. Gynecomastia is subject to surgical treatment, since there is a risk of malignancy of the mammary glands.

During puberty and later, treatment is carried out with testenat, su-stanon-250 or omnodron-250, which are injected intramuscularly in 1 ml every 3-4 weeks. Treatment is aimed at developing secondary sexual characteristics, penile growth, and maintaining and enhancing libido. Spermatogenesis is not restored.

A chromatin-negative variant of Klinefelter's syndrome with a 47XYY karyotype has been described. Much less often, patients have a Y-chromosome polysomy with a set of XYYY or XYYYY. Individuals with such a set of chromosomes are distinguished by high growth, great physical strength, psychopathic behavior with aggressive traits and a mild degree of mental retardation. The frequency of this syndrome among newborn boys is 1: 1000. Men with the 47XYY karyotype are fertile. Children from them may have a normal karyotype or sometimes a heteroploid set of chromosomes.

Shereshevsky-Turner syndrome is a variant of pure glandular dysgenesis.

The disease was described in 1925 by N.A. Shereshevsky in women, in 1938 Turner proposed the main symptoms to characterize this syndrome: infantilism, pterygoid cervical fold, valgus deviation of the elbow and knee joints. In addition, Shereshevsky-Turner syndrome is manifested by short stature (wide shoulder girdle, narrow pelvis, shortening of the lower extremities with deformation of the fingers and toes). With this disease, sexual infantilism is expressed. The ovaries are underdeveloped, there is practically no follicular epithelium in them, the production of estrogen is at a very low level. This leads to underdevelopment of the uterus, vagina, amenorrhea, infertility, and the absence of secondary sexual characteristics.

It was found that more than half of women with this syndrome have monosomy of X chromosomes, karyotype 45X0. The occurrence of this anomaly is associated with a violation of spermatogenesis or ovogenesis in parents. Mosaic shapes (X0 / XX, X0 / XY) are observed. Less commonly, Turner phenotypic syndrome is determined in men with a 46XY karyotype. The etiology of the disease in this case is explained by the presence of a translocation of part of the X chromosomes to the Y chromosome. Sometimes X0 / XY mosaic is detected. Turner syndrome manifests itself in men of short stature and the indicated body changes, as well as anatomical and functional hypogonadism (malnutrition of the genitals, bilateral cryptorchidism, low testosterone production, hypoplastic testes).

Treatment is feminizing therapy for women and androgens for men. To stimulate the growth of patients and the development of the external genital organs, treatment with somatotropin, anabolic hormones and vitamin therapy can be carried out.

11. Variants of hypospadias. 1 - capitate; 2 - stem; 3 - scrotal; 4 - perineal.

Del Castillo's syndrome (terminal agenesis).

The ethnology of the disease has not been studied enough. The disease manifests itself in adult men with normally developed external genitalia and pronounced secondary sexual characteristics. The main complaint is infertility. The birds are of normal size or slightly reduced in size. Gynecomastia is not detected.

When examining the ejaculate, aspermia is determined, less often - azoospermia. A histological examination of the testicular biopsy material reveals the absence of spermatogenic epithelium in the tubules. Their basement membrane is lined with only sustentocytes. The interstitial tissue of the testicle does not suffer from this syndrome. The secretion of sex hormones is reduced. Gonadrotropin levels are elevated. In genetic studies, a normal karyotype 46XY is established in patients.

Del Castillo et al. (1947) considered terminal agenesis to be a congenital malformation. Subsequently, similar changes in the testicular tubules (terminal atrophy) were determined in patients after radiation exposure and in an animal experiment with the use of cystostatic drugs.

The prognosis for the restoration of spermatogenesis is poor.

Congenital hypoplasia of the testicles.

The etiology is not fully understood. It is based on the underdevelopment of the gonads in the embryonic period in the absence of cytogenetic abnormalities in patients with a 46XY male karyotype. Hypoplasia is more often diagnosed by chance, when patients refer to an infertile marriage. Characteristic for this entire group of patients is a decrease in the testes located in the scrotum, hypoplasia of the epididymis, penis, prostate gland, insufficient terminal hair growth, sometimes accompanied by disproportionate development of body parts, pseudogynecomastia. In the study of testicular biopsies, various degrees of hypoplasia of the spermatogenic epithelium in the tubules are detected, spermatozoa are rare or completely absent. In the interstitial tissue, degeneration and accumulation of glandulocytes are noted. The secretion of sex hormones is reduced with an increased or decreased level of gonadotropins.

Treatment consists in carrying out androgen therapy or prescribing gonadotropins, biogenic stimulants, vitamins A, E, etc.

ANOMALIES OF THE GENUS AND URINARY CANAL

Hypospadias is a congenital underdevelopment of the spongy urethra with replacement of the missing area with connective tissue and curvature of the penis towards the scrotum. It is one of the most common urethral abnormalities (in 1 in 150-100 newborns). Gylospadnya develops as a result of a delay or violation of the formation of the urethra at the 10-14th week of embryonic development. It can be caused by exogenous intoxication, intra-phobic infections, hyperestrogenism in the mother during the formation of the genitals and urethra in the fetus.

As a result, the external opening of the urethra opens above the natural one and can be located in the area of \u200b\u200bthe coronary sulcus, on the ventral surface of the penis, in the scrotum or perineum (Fig. 11). Depending on the localization of the external opening of the urethra, capitate, stem, scrotal and perineal hypospadias are distinguished ... In any form of hypospadias, a narrow strip of mucous membrane and a dense fibrious cord (chord) remain between the external opening and the head. With this form of anomaly, the urethra becomes shorter than the cavernous bodies. The presence of a shortened urethra and a short inelastic chord leads to curvature of the penis. The head of the penis is bent downward, wide, and the preputial sac looks like a hood.

The clinical picture.

The patient's complaints depend on their age and on the type of hypospadias. If children are mainly worried about urination disorder, then adults - difficulty or impossibility of sexual intercourse.

With capitate hypospadias, which accounts for almost 70% of all hypospadias, children and adults have almost no complaints. At the same time, the urethra opens at the site of the usual location of the frenum, which does not cause any particular disorders. Complaints occur only when there is stenosis of the external opening or when the head is tilted too much, when urine can get on the legs.

With stem hypospadias, the deformity of the penis is more pronounced. The outer opening is located on the back of the penis between the head and the root of the scrotum. During urination, the jet is directed downward, which makes it difficult to empty the bladder. The erection becomes painful, and the deformation of the penis disrupts intercourse.

In scrotal hypospadias, the penis is somewhat reduced and resembles the clitoris, and the external opening of the urethra is located in the area of \u200b\u200bthe split, labia-like scrotum. At the same time, patients urinate in a female pattern, urine is sprayed, which causes maceration of the inner surfaces of the thighs. Newborns with scrotal hypospadias are sometimes mistaken for girls or false hermaphrodites.

With pro-feldsnatal hypospadias, the urethral opening is located even more posteriorly, on the perineum. The penis also resembles the clitoris, and the split scrotum resembles the labia. Perineal hypospadias is often combined with cryptorchidism, which further complicates the sexual differentiation of patients.

Children begin to understand their inferiority early, become withdrawn, irritable, and retire. After the end of puberty, they complain about the impossibility of having intercourse.

Diagnosis of a typical hypospadias does not cause any particular difficulties. However, it is sometimes very difficult to differentiate scrotal and perineal hypospadias from female false hermaphroditism. It is necessary to pay attention to the foreskin, which in boys with hypospadias is located on the dorsal surface of the penis. With false hermaphroditism, it passes to the ventral surface of the clitoris and merges with the labia minora.

The vagina in these patients is well formed, but sometimes it leaves the urethral lumen like a diverticulum. It is also necessary to investigate the content of 17-KC in urine and identify male and female chromatin. From X-ray data, genitography (to detect the uterus and appendages), urethrography (to identify the urogenital sinus) and oxygenosuprarenography are used. Magnetic resonance imaging and ultrasound diagnostics have significant capabilities. In particularly difficult cases, laparoscopy or laparotomy is performed to identify the ovaries.

Treatment.

Capitate hypospadias and hypospadias of the distal truncal third of the urethra, if there is no significant curvature of the penis or stenosis, do not need surgical correction. In other cases, surgical treatment is the method of choice.

To date, many different surgical methods of treatment have been proposed, but the following recommendations are common to all: to perform the Operation already in the first years of life, i.e. even before the appearance of irreversible processes in the corpora cavernosa; the first stage of the operation - straightening the penis - is carried out at the age of 1-2 years; the second stage - the creation of the missing piece of the urethra - at the age of 6-13 years.

12. Variants of penile straightening surgery for hypospadias (1-5).

13. Scheme of plastics of a skin defect according to Smith - Blackfield modified by Savchenko (1-3 - stages of the operation).

15. Scheme of plastics of the urethra according to Cecil - Kappa (1-5 stages of the operation).

The first stage consists in careful excision of the notochord (scar tissue on the posterior surface), fibrous septum of the corpora cavernosa, mobilization of the penis from the scars in the scrotum, and excision of the frenum. At the same time, the external opening of the urethra is isolated and moved upward. For the normal development of the corpora cavernosa, the defect of the penis formed after straightening must be closed with a skin flap. Many different methods are used to close a skin defect (bridging flaps of the skin of the abdomen or thigh, moving the skin of the foreskin from the upper part of the glans penis to the lower one, the use of Fidat's stem, etc.). However, these methods are not widely used.

Most often, the so-called unified scheme is used to replace the defect, when the skin of the preputial sac and scrotum is used in the form of mobile triangular flaps on a wide feeding base [Savchenko NE, 1977] (Fig. 12). Unification makes it possible to mobilize and move the reserves of the skin of the foreskin and scrotum according to the Smith - Blackfield method modified by N.Ye. Savchenko (Fig. 13). As a rule, after surgery, there is a displacement of the urethra in the proximal direction and an increase in the degree of hypospadias. However, this does not affect the further course of the Operations. The penis is then fixed to the abdominal skin for 8-10 days. The operation ends with urine diversion through a urethral catheter.

The second stage of the operation is performed no earlier than 5 months after the first. About 50 different ways of forming the urethra have been proposed. However, the most promising are methods using nearby tissues! So, for example, according to Dupley, on the lower surface of the penis from the head and around the opening of the urethra cut out a skin flap and form the urethra (Fig. 14). Then the urethra is immersed by suturing along the midline with the remaining flaps on the sides. If there is not enough skin, then the newly created urethra can be immersed in opposite triangular flaps.

If the skin defect is total throughout the penis, then the urethra can be temporarily immersed in the scrotum. After engraftment on the scrotum, parallel incisions are made and flaps are cut out to cover the newly formed urethra along. Cecil - Kalpu (fig. 15). The operation technique modified by N.E. Savchenko allows to unify the plastic of the urethra for all types of hypospadias and is the method of choice. To avoid an erection, all patients after surgery are prescribed tranquilizers, valerian or bromides (camphor monobromide, sodium bromide).

16. Variants of epispadias. 1 - capitate form; 2 - epispadias of the penis; 3 - full epispadias.

Epispadias is a malformation of the urethra, which is characterized by underdevelopment or absence over a greater or lesser extent of its upper wall. It is less common than hypospadias, in about 1 in 50,000 newborns. Boys have epispadias of the head, epispadias of the penis, and total epispadias. The urethra in these cases is located on the dorsal surface of the penis between the split cavernous bodies.

In any form of epispadias, the penis is to some extent flattened and shortened due to its tightness to the anterior abdominal wall, and the foreskin remains only on its ventral surface. The cause of epispadias is abnormal development of the urogenital sinus, genital tubercle and urogenital membrane. As a result of the displacement of the urethral plate, it appears above the genital tubercle. During the formation of the urethra, the genital folds do not grow together, leaving its upper wall split.

The clinical picture.

Symptoms depend on the form of epispadias. Epispadias of the glans penis is characterized by splitting of the spongy body of the glans on the dorsal surface, where the external opening of the urethra is determined at the coronal sulcus. The head is flattened. With an erection, there is a slight, upward curvature of the penis. Urination is not impaired, only an abnormal direction of the urine stream is noted.

Epispadias of the penis is accompanied by flattening, shortening and upward curvature. The head and cavernous bodies are split, on the dorsal surface they are free from the foreskin, which is preserved on the ventral side of the penis. The external opening in the form of a funnel opens on the body of the penis or at the root of it (Fig. 16). From the external opening to the head stretches the urethral groove, lined with a strip of mucous membrane. The sphincter of the bladder is preserved, however, its weakness is often noted. Therefore, with tension of the abdominal press, urinary incontinence may occur. Significant splashing of urine makes it possible to urinate while sitting, pulling the penis towards the perineum. In adults, complaints about the difficulty or impossibility of having intercourse are added due to deformation and curvature of the penis, which intensify during an erection.

Total epispadias is characterized by the complete absence of the anterior wall of the urethra, splitting throughout the cavernous bodies and the sphincter of the urinary bladder. The penis is underdeveloped, bent upward and pulled up to the stomach. The external opening of the urethra in the form of a wide funnel is located at the base of the penis and is limited from above by the skin fold of the anterior abdominal wall. Due to the constant leakage of urine, there is maceration of the skin of the perineum and thighs. With total epispadias, there is a significant divergence of the bones of the pubic symphysis, in connection with which patients have a duck gait and a flat stomach.

The disease is combined with cryptorchidism, testicular hypoplasia, underdevelopment of the scrotum, prostate gland and malformations of the upper urinary tract. Total epispadias causes the greatest degree of urination disorders and completely deprives adult patients of sexual function.

Diagnosis of epispadias does not cause difficulties and is based on a simple examination of patients. The kidneys and upper urinary tract should be examined to rule out abnormalities and pyelonephritis.

Treatment.

Epispadias of the head most often does not need correction. In other cases, surgical treatment is indicated, which should be aimed at restoring the urethra, bladder neck, correcting deformities and curvature of the penis. The choice of the method of operation should be made depending on the form of epispadias and the individual characteristics of the patient. Surgical correction is performed at the age of 4-5 years. Before the operation, it is necessary to eliminate diaper rash and skin maceration.

Significant difficulties arise in the restoration of the sphincter of the bladder, followed by plastic surgery of the urethra. The most widespread are operations for plastics of the bladder neck and urethra according to Jung-Diss and plastics of the bladder neck according to Derzhavin.

The Jung-Diss operation consists in the excision of scar tissue and the formation of the posterior urethra and bladder neck due to the urinary triangle. The bladder is opened with an incision from the apex to the external sphincter: From the sides, cut out And 2 triangular sections of the mucous membrane are excised. The urethra is formed from the remaining median lane of the mucous membrane. The demucosed lateral flaps are mobilized and overlapped to form the bladder neck. The pubic bones are brought together with nylon filaments. The formed distal part of the urethra is immersed, suturing the cavernous bodies above it for the tunica albuginea and the skin of the penis (Fig. 17). For. urine diversion use epicystostomy.

a - the bladder was opened along the midline, triangular flaps of the mucous membrane were cut out and excised (dotted line); b - the mobilized walls of the bladder are overlapped; the urethra is formed from the median plate on the catheter.

18. Plastic surgery of the bladder neck according to Derzhavin with complete epispadias. a - the imposition of the first row of sutures narrowing the neck of the bladder; b - the imposition of the second row of sutures.

Derzhavin's operation consists in the formation of the sphincter of the bladder without dissecting the wall due to the longitudinal corrugation of the neck and its wall. The anterior wall of the bladder is exposed by a longitudinal transpubic dissection of the tissues. Then, on the catheter, each time a longitudinal strip of the bladder about 3 cm wide for 6 -7 cm is ingwagged with two rows of submerged sutures (Fig. 18). Having achieved a tight coverage of the catheter with stitched tissues, the paravesical space is drained, the wound is sewn up layer by layer. The catheter is left for 12-14 days to drain the bladder.

Plastic surgery of the urethra is used as an independent operation for epispadias of the glans or penis; it can also be the final stage in the treatment of total epispadias. Various methods of forming the urethra in epispadias differ from each other in the degree of mobilization of the mucous membrane when creating the urethral tube, as well as moving it to the ventral surface or leaving it on the dorsum of the penis.

19. Stages of plastics of the urethra according to Dupley in epispadias (a - e).

Operation Dupley (Fig. 19). An incision bordering the external opening of the urethra and continuing on the border of the mucous membrane and the skin of the integument, cut out a flap, the width of which should be at least 14-16 cm.The edges of the flap are separated from the corpora cavernosa by 3-4 mm and sutured on a catheter with thin synthetic threads along the stem. The second row of sutures brings the cavernous bodies closer together, the third row brings the skin together. A urethral catheter is used to drain urine or a cystostomy is applied. During this operation, there is a risk of urethral fistula formation along the line of coincidence of the sutures of the urethra and skin.

20. Plasty of the urethra according to Thirsh in epispadias (a - c - stages of the operation).

Thirsh's method lacks this drawback (fig. 20). With it, the lines of the inner and outer seams are in different projections. In addition, due to the mobilization of skin grafts, the formation of a large urethral tube is possible. If there is a lack of corny, the wound of the penis can be sutured to the anterior abdominal wall, followed by the use of the abdominal skin to close the skin defect (Fig. 21).

21. Closure of a wound defect during urethral plastic surgery for epispadias (a - b).

Jung's urethral plasty consists in moving the newly formed urethra to the ventral surface of the penis (Fig. 22). The incision is made on both sides of the urethral sulcus and the outer opening of the urethra is bordered, which is then mobilized to the bulbous section. The edges of the flap for the rest of the length are completely separated from one of the cavernous bodies and the spongy body of the head. On the other hand, the flap is mobilized only for suture capture.

After the formation of the urethral tube on the cutter, it is moved to the ventral surface and fixed there by stitching the cavernous and sponinous bodies above it. After that, the third row of veneer is sutured to the skin of the penis. Urine diversion is carried out using an instostomy.

22. Stages of urethral plasty according to Jung in epispadias (a - f).

In adults with urethral plastic surgery, we obtained the best results in those operated on by Jung's method. It should be noted that in all variants of operations, the most thoracic moment is the formation of the capitate part of the urethra.

The latent penis is considered a fairly rare malformation in which the penis does not have its own skin and is located under the skin of the scrotum, pubis, perineum or thigh. This anomaly must be differentiated from micropenis, from ectopia, or from congenital absence of the penis, in which splitting of the scrotum is often noted, and the external opening of the reduced urethra opens on the perineum or in the rectum.

Treatment should be prompt and consist in the release of the penis from the fiber and the formation of its own skin.

For the prevention of mental disorders of the child, as well as to create favorable conditions for the development of the cavernous bodies, surgical treatment is indicated at the age of 3 to 6 years.

Membranous penis.

With this anomaly, the skin of the scrotum moves away from the middle or even at the head of the penis. A fairly common anomaly, diagnosed, however, in adult men, as it makes it difficult to have sexual intercourse.

Treatment is prompt.

The penis is released by a transverse dissection of the membranous part of the scrotum. The incision after mobilization of the penis is sutured longitudinally. Sometimes it is necessary to resort to partial excision of the scrotum.

Phimosis.

A common malformation of the penis is phimosis - a narrowing of the foreskin, which prevents the release of the head from the prepucinal pawn.

With phimosis, a whitish sebaceous substance (smegma) accumulates inside the preputial sac, produced by the glands located on the head of the penis. Smegma can thicken, be encrusted with salts, and when an infection joins, it can decompose, causing inflammation of the glans and foreskin of the penis (balanoposthitis), which can further lead to the development of cancer. Severe phimosis can cause difficulty urinating in children, urinary retention and even cause the upper urinary tract to dilate (ureterohydronephrosis).

Treatment.

In children, it is often possible to free the head of the penis after expanding the opening of the foreskin and disconnecting the loose adhesions between the head and the inner leaf of the foreskin with a metal probe. In adults, as well as in severe phimosis in children, an operation is indicated - a circular excision of the foreskin with subsequent stitching of its inner and outer sheets, dissection of the foreskin, etc.

Paraphimosis.

One of the dangerous complications of phimosis is paraphimosis, when, due to any reason (sexual intercourse, masturbation, etc.), the narrowed foreskin moves behind the glans penis, its swelling develops, which leads to infringement of the head and disruption of its blood supply. In the absence of urgent care, necrosis of the strangulated glans may develop.

Treatment of paraphimosis consists in trying to set the glans penis abundantly lubricated with vaseline oil. If these attempts do not lead to success, then the restraining ring is dissected. The following shows a circular excision of the foreskin in a planned manner.

The short frenulum of the penis can accompany phimosis or meet on its own. The short frenulum prevents the glans from being released from the preputial sac, causing penile curvature during erection and pain during intercourse. In this case, the short bridle often tears, causing bleeding.

O. L. Tiktinsky, V.V. Mikhailichenko

Disorders of sexual development in boys are associated with the pathology of secretion or action of androgens. The clinical picture depends on the age at which the problem occurred.

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The formation of the male reproductive system goes on continuously until the end of adolescence. Doctors distinguish 3 stages of genital differentiation. Each of them has its own dominant influences and a certain physiological meaning.

Formation stages:

• intrauterine;
• pre-pubertal;
• pubertal.

Intrauterine period

The prenatal period begins with conception and ends with the birth of a child. At the time of fertilization of the egg, the chromosomal sex of the child is determined. The obtained genetic information remains unchanged and affects further ontogenesis. In humans, the XY set determines the male gender. Until 5-6 weeks, female and male embryos develop in the same way. Primary germ cells have the ability to differentiate according to one or another variant until the 7th week of pregnancy. Before this period, two internal ducts are laid: wolffian (mesonephral) and müllers (paramesonephral). Primary gonad up to 7 weeks old is indifferent (indistinguishable in boys and girls). It consists of the cortex and medulla.

After 6 weeks of development, sex differences appear in differentiation. Their occurrence is due to the influence of the SKY gene, which is located on the short arm of the Y chromosome. This gene encodes a specific "male membrane protein" H-Y antigen (testicular development factor). The antigen affects the cells of the primary indifferent gonad, makes it transform in the male pattern.

Testicular embryogenesis:

• the formation of genital cords from the cortex of the primary gonad;
• the emergence of Leydig and Sertoli cells;
• the formation of convoluted seminiferous tubules from genital cords;
• the formation of the tunica albuginea from the cortex.

Leydig cells begin to secrete testosterone, and Sertoli - anti-Müllerian factor.

At the 9th week of intrauterine development, the influence of the chromosomal and gonadal sex affects the reproductive ducts. The anti-Müllerian factor causes atrophy of the paramesonephral duct. Without this influence, the uterus, fallopian tubes, and the upper third of the vagina are formed from the duct. The regression factor leaves only rudiments in the male body.

Testosterone stimulates the development of wolf ducts. By the beginning of the 14th week, the fetus has formed the epididymis, seminal vesicles, vas deferens and vas deferens. Primary germ cells are transformed into spermatogonia.

At the prenatal stage, great influence belongs dihydrotestosterone... This hormone is formed from testosterone by the enzyme 5a-reductase. Dihydrotestosterone is involved in the formation of external organs (penis, scrotum).

In the prenatal period, the testicles descend into the scrotum. By birth, this process is completed in 97% of full-term boys and in 79% of premature babies.

• defects of the guiding ligament;
• dysgenesis of the gonads;
• hypogonadism in the prenatal period;
• immaturity of the femoral genital nerve;
• anatomical obstacles to the movement of the testicle;
• weakening of the muscle tone of the abdominal wall;
• violation of the synthesis and action of testosterone.

Pre-adolescence

The pre-pubertal period is characterized by relative functional rest. In the first months after birth, a child's blood levels can be high (due to the intake of maternal levels). Further, the concentration of FSH and LH, as well as testosterone falls to extremely low values. The pre-pubertal period is called "juvenile pause". It lasts until the end of prepubertal period.

Puberty

In the pubertal stage, testosterone synthesis is activated in the testicle. First, at 7-8 years old, the boy's blood androgen level rises due to the adrenal glands (adrenarche). Then, at the age of 9-10 years, inhibition decreases in the centers of the hypothalamus responsible for sexual development. This increases the levels of GnRH, LH and FSH. These hormones affect the testicle, increasing testosterone production.

Male sex steroids:

• enhance the growth of internal and external genital organs;
• affect the development of the accessory glands;
• form sexual characteristics (secondary, tertiary);
• enhance the linear growth of the body;
• increase the percentage of muscle tissue;
• affect the distribution of subcutaneous fat.

In puberty, the maturation of germ cells begins and the formation of mature sperm cells.

Normal onset of sexual development and determination of its delay

Boys' puberty starts with an increase. The average age at which this trait appears is 11 years.

Table 1 - Average values \u200b\u200bof testicular volume in different age periods (according to Jockenhovel F., 2004).

The rate of puberty is the rate at which signs of puberty appear.

Possible rates:

• medium (all signs are formed in 2-2.5 years);
• accelerated (formation occurs in less than 2 years);
• slow (formation takes 5 years or more).

The normal sequence of signs of puberty at puberty is:

1. an increase in testicles (10-11 years);
2. penile enlargement (10-11 years old);
3. development of the prostate, an increase in the size of the larynx (11-12 years);
4. significant enlargement of the testes and penis (12-14 years old);
5. female pubic hair growth (12-13 years old);
6. nodularity in the area of \u200b\u200bthe mammary glands, (13-14 years old);
7. onset of voice mutation (13-14 years old);
8. the appearance of hair in the armpits, on the face (14-15 years old);
9. pigmentation of the skin of the scrotum, the first ejaculation (14-15 years);
10. maturation of sperm (15-16 years);
11. male pubic hair (16-17 years old);
12. stopping the growth of bones of the skeleton (after 17 years).

Puberty is assessed by Tanner.

Table 2 - Estimation of the stage of sexual development according to Tanner.

Delayed puberty in boys

Delayed sexual development is determined if a boy, by the age of 14, has a testicle volume less than 4 ml, there is no growth of the penis in length and an increase in the scrotum. In this case, it is required to start an examination to identify the cause of the pathology.

The reasons

Delayed sexual development can be due to:

• constitutional features (family);
• violations of the hypothalamic-pituitary regulation ();
• primary testicular tissue failure ();
• severe somatic pathology.

Diagnostics

• collection of anamnesis;
• assessment of heredity;
• assessment of bone age by radiograph;
• general inspection;
• examination of the external genital organs, assessment of the volume of the testicles and the size of the scrotum;
• hormonal profile (LH, FSH, testosterone, prolactin, TSH);
• tomography of the brain, x-ray of the skull;
• cytogenetic study.

Treatment

Treatment depends on the cause of the delayed puberty.

Family forms of delayed puberty can be corrected with the help. To prevent stunting, adolescents with this form of the disease are prescribed anabolic steroids.

For secondary hypogonadism, gonadotropins and gonadorelin are used in the treatment. This therapy is the prevention of future infertility. The use of hormones in the hypothalamic-pituitary region stimulates the development of the testicles and.

With primary hypogonadism, from the age of 14, boys are prescribed testosterone replacement therapy.

Premature puberty in boys

The appearance of signs of puberty in boys under 9 years of age is considered premature. This condition can lead to social maladjustment. In addition, premature sexual development is one of the reasons for short stature.

The reasons

Premature sexual development is divided into:

• true (associated with the work of the hypothalamic-pituitary region);
• false (associated with the autonomous secretion of hormones by the adrenal glands or tumors).

True premature sexual development is complete (there are signs of masculinization and activation of spermatogenesis).

The reason for this condition may be:

• idiopathic;
• associated with diseases of the central nervous system;
• associated with the primary;
• arising against the background of prolonged hyperandrogenism (for example, with adrenal tumors).

False premature sexual development is usually not accompanied by activation of spermatogenesis (except in cases of familial testosterone toxicosis).

Reasons for false premature sexual development:

• congenital hyperplasia of the adrenal cortex;
• , testicles;
• cushing's syndrome;
• secreting tumors;
• hyperplasia of Leydig cells (familial testosterone toxicosis);
• androgen treatment;
• isolated premature adrenarche.

Diagnostics

Examination for signs of premature sexual development includes:

• collection of anamnesis;
• general inspection;
• examination of the genitals;
• analyzes of hormones (LH, FSH, testosterone, TSH,);
• samples with gonadoliberin;
• bone age research;
• x-ray of the skull, tomography of the brain, etc.

Treatment

For the treatment of true premature puberty, synthetic analogs of gonadoliberin are used. This drug suppresses the impulse secretion of LH and FSH. If the cause of the disease is a pathology of the central nervous system, then the patient is prescribed appropriate treatment (by a neurologist, neurosurgeon).

Treatment for false precocious puberty depends on the underlying causes. If the pathology is associated with an isolated adrenarche, only observation is carried out. If a hormonally active tumor is found, radical treatment (surgery, radiation therapy) is performed. In cases of congenital hyperplasia of the adrenal cortex, corticosteroid therapy is selected.

Endocrinologist I. G. Tsvetkova

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Female obesity (gynoid) is less common in men than abdominal obesity. However, the level of people with this type of excess weight is steadily increasing. Female obesity occurs mainly due to hormonal disruption. In this case, fatty tissues appear in the thighs, buttocks, and breasts. The male figure is rounded, there is a resemblance to the female, hence the name of the pathology. In the article, we will analyze in detail the causes of the disease, talk about the methods of its treatment, about the possible consequences. You will find out if this pathology can develop in adolescent boys, watch photos and videos on the topic.

Why does female obesity develop?

Normally, in men, the level of androgens and estrogen is maintained at a balanced level. Testosterone predominates, which is responsible for the formation of muscles, broad shoulders, narrow hips. Estrogen, although present in the body, has no noticeable effect on the formation of adipose tissue and body type.

A completely different picture emerges if, during hormonal disruption, the level of estrogen prevails over the amount of testosterone. In this case, the body reacts accordingly, potency decreases, fat is deposited on the hips and buttocks. Often fatty tissue also develops in the breast area. Therefore, the main reason for the development of obesity in men according to the female type is considered by doctors to be a violation of the endocrine system.

By the way, in adolescence, boys undergo a restructuring of the body. At the age of 10-15 years, hormonal disruption is not uncommon, therefore, adolescents may experience gynoid obesity. As a rule, the pathology disappears with age.

Other possible causes of pathology:

• violation of the diet;
• mental disorders;
• sedentary lifestyle;
• ecological situation.

Doctors notice that heredity also plays a role. If the parents have female obesity, then the child is more likely to develop such a pathology.

How is female obesity defined?

When a man goes to a doctor, a specialist finds out the details about the patient's lifestyle. It is necessary, without hesitation, to answer questions about the quality of food, daily regimen, and medication. Obesity can be calculated from the body mass index. The appearance of the figure also indicates a gynoid disease: the deposition of fat on the hips and buttocks makes the figure look like a pear.

Read also: Body mass index: table and calculations

Taking tests for testosterone and estrogen levels will help to make an accurate diagnosis. If the prevalence of the female sex hormone is revealed, the doctor will be able to say with confidence about the development of obesity in a man or adolescent by a female type.

At the final stage of the examination, you will need to do an MRI or ultrasound of the internal organs. This is necessary to determine the presence of visceral fat on the organs, its amount and the degree of organ dystrophy.

Having on hand the results of tests and examinations, the doctor will select the appropriate treatment.

How is gynoid obesity in men treated?

The principles of treatment for female obesity are the same as for abdominal obesity. Regardless of the reasons that caused the pathology, the fight against it begins with the transition to a diet. The following foods are gradually eliminated from a man's diet:

• fried and fatty foods;
• flour products;
• sweet and sugar;
• tea, coffee, juices.

Instead of excluded food products, cereals, fresh vegetables and fruits, and herbs are added. Meat is allowed, but no more than twice a week and preferably dietary, for example, rabbit meat. It is recommended to drink plain water instead of excluded drinks. The volume of fluid consumed daily should not be less than two liters.

Simultaneously with the transition to a therapeutic diet, the lifestyle is adjusted. The doctor recommends a man to devote more time to walking, running, cycling. Of the exercises, those that help burn excess fat in problem parts of the body are suitable. Daily squats give a good effect. At this stage, it is important for a man to know: first of all, visceral (internal) fat is burned. Therefore, if obesity does not go away from the buttocks and thighs immediately, do not despair.

Until now, one has to deal with the myth that initially the human embryo develops along the female path, and only then in future boys, under the influence of androgens, male genitals are formed from the female genital organs. This is not true.

Genetic sex determination occurs during fertilization. The Y chromosome is a genetically male determinant (the zygote contains 22 pairs of autosomes + XY sex chromosomes, i.e. 46XY). The karyotype of the genetically female zygote is 46XX.

Until about 6-7 weeks of intrauterine life of the embryo, its gonads develop identically in both men and women. This is the so-called indifferent stage development of the embryo, when the reproductive system of both sexes develops according to the same genetic program.
During the formation of the gonadal sex, the fetus develops a male ( wolves) and female ( müller) ducts. Initially, the development of these ducts begins unipotentially, that is, regardless of the future sex, and only one of them can develop into the reproductive tract associated with a specific genetic sex of the fetus. In particular, the Wolffian duct turns into the structures of the male reproductive tract, and the Müllerian - the female. The simultaneous presence of the Wolf and Müllerian ducts at this stage is a legacy from our distant hermaphrodite ancestors who lived hundreds of millions of years ago.

Primary germ cells are formed in the wall of the yolk sac and at the 5th week of embryogenesis begin to migrate into the gonadal ridges - rudiments indifferent gonads. IN indifferent period development of primary gonads in their stromal tissue contains two types of cells. One type of cell in the gonadal stage of sex differentiation develops into granulosa cells of the ovaries or Sertoli cells of the seminiferous tubules in the testes. The second type of cells, at the gonadal stage, differentiates into cells of the transparent membrane (theca pellucida) in the ovaries or into Leydig cells in the testes.

In the embryo male at the 6-7th week of embryonic life after the migration of primary germ cells into the primary gonads in the presence of a Y chromosome containing SRY gene, differentiation occurs sertoli cells... In the process of differentiation, Sertoli cells are located around the primary germ cells, as a result, the development of testicular tubules in the primary gonads begins. Differentiation of mesenchymal (stromal) cells of the gonads into interstitial leydig cells, which in the future will secrete the male sex hormone testosterone, starts from the 8-9th week and ends at the 10th week of fetal development.
In the female embryo, the differentiation of the primary gonads into the ovaries (determined fOXL2 genome) starts from the 9th week when the X chromosomes are activated. If the FOXL2 gene fails, the primary gonads will develop into ... testes!

Development internal male genital organs in the fetus it occurs under the influence of testosterone. The secretion of testosterone by Leydig cells in a male fetus begins at about 8-9 weeks of development, under the influence of placental chorionic gonadotropin, the secretion of which is stimulated by growth hormone. Under the influence testosterone wolffian ducts transforms in its development into the epididymis, vas deferens and seminal vesicles.
Sertoli cells of the testicles of the fetus secrete müllerian inhibiting factor (synonym - anti-mueller hormone) causing regression Müllerian ducts in a male fetus.

In a fetus developing by female type, granulosa cells and transparent membrane cells do not secrete anti-Müllerian hormone and testosterone. In the absence of anti-Müllerian hormone, the Müllerian ducts turn into internal female reproductive organs (fallopian tubes, uterus, upper vagina), and at the same time occurs regression wolffian ducts due to the lack of testosterone secretion in the fetus.

Differentiation of the external genital organs occurs from the urogenital sinus, genital tubercle, genital folds and genital ridges. The development of the external genitalia is dependent on sex hormones.
In a fetus developing by male type, under the influence testosterone the urogenital sinus gives rise to the prostate and bulbourethral glands.
5-alpha reductase catalyzes the conversion of testosterone to dihydrotestosterone. At about the 12th week of intrauterine development, the genital tubercle under the influence dihydrotestosterone differentiates into the penis, the genital folds form the distal urethra, and the genital ridges develop into the scrotum.
In a fetus developing by female type, in the absence of androgens at about the 14th week of intrauterine development, the urogenital sinus develops into the lower part of the vagina, the genital tubercle into the clitoris, and the genital folds and genital ridges differentiate into the labia minora and majora, respectively. Female sex hormones contribute to the differentiation of the extragonadal organs of the female reproductive system.

As we can see, a fetus with an XY karyotype cannot be considered a female fetus at any stage.