Congenital vices of the genitourinary system. Deviations in the development of the organs of the urinary system of anomaly of the development of the urogenital system

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GBOU VPO "OrenburgState Medical Academy "Ministry

Department of Histology, Cytology and Embryology

ESSAY

on the topic: « Anomalies of the development of the urinary system»

bydiscipline: Histology

Orenburg 2014.

Introduction

1. Anomalies for the development of kidneys

2. Anomalies of the urinary bubble

3. Methods for diagnosing the vices of the urinary system

Conclusion

Bibliography

Introduction

The urinary system is a combination of organs producing and identifying urine. The urethra is a tube, according to which the watering of the productive kidney zones enters the bladder, where it accumulates, and then dismissed through the channel, called the urethra. With congenital misuse (anomalies) of the urinary system, either products or urine removal are broken.

Anomalies of the urinary system They call violations in the development of the organs of the urogenital system, which do not allow the body to function normally.

Defects of the urinary system differ in gravity from insignificant to the life-threatening life. Most are serious, requiring surgical correction. Other defects do not cause violations of the function of the urinary system, but impede urination control.

1. Development Anomalieskidney

One-sided Aplasia (Agnesia) Kidney - the absence of one of the kidneys as a result of the construction of the body's development. Often, with this vice, there is no corresponding seeding duct. Aplasia usually does not show itself and is detected in a prophylactic examination. At the same time, in most cases there is no ureter, the mouth of the ureter and the corresponding part of the urinary triangle. However, in 15% of cases of Agenesia on the side of the damage, the lower third of the ureter is determined. Often A.P. Combined with other anomalies: monorchism, the atresia of the anal hole and the rectum, the defects of the development of fine and colon.

Aplasia is accompanied by an increase in the functional load on the opposite kidney. Bilateral Aplasia - extremely rare pathology, not compatible with life.

Doubling kidney - The body seems to be two parts. As a result, the size of double kidney is greater than normal. The upper and lower half is separated by a furrow, which can have a different degree of severity, while the upper part of the organ in size is always less than the lower. Two parts have separate blood supply. Doubling can be complete and not complete. The difference lies in the fact that with full doubling, the upper and lower half of the kidneys have their own cup-laughter. At the same time, the cup-making system at the bottom is formed normally, and in the upper underdevelopment. From each locher, the ureter departs - doubling the ureter (it can also be complete: both ureter in their own in the bladder; and incomplete: the upper ureter merges with the bottom). The treatment of pathology itself is often not required. However, double kidney is inclined to the development of various pathological processes (hydronephrosis, pyelonephritis, tuberculosis of the kidney, urolithiasis). In these cases, treatment is carried out, as well as treatment aimed at eliminating the root cause (violation of urodynamics, blood flow).

Extreme kidney - In essence, there are 3 organs. The added kidney has a separate blood supply and its own ureter, which can be included in the bladder, or flows into the ureter of the main kidney. The size of the additional P. is always less main and can fluctuate widely. Additional P. always is located below the main (lumbar, iliac or pelvic location). In the absence of complications, tactics are expectant. Indication to the removal of additional P. is: hydronephrosis, bubble-ureteral reflux, nephrolityiasis, pyelonephritis, tumor.

Kidney size anomalies

Hypoplasia kidney - decrease in size as a result of underdevelopment, but without disorders in the structure and functioning of the body. Pathology is one-sided. Treatment under the kidney hypoplasia is only necessary if there is complications (pyelonephritis, arterial hypertension).

Anomalies location (dystopia) kidneys

During the intrauterine development, the emerging kidney moves from the pelvic region to the lumbar. Violation of this process of movement leads to the formation distopia kidney. This vice may have one and bilateral nature. Allocate:

Lumbar dystopia - The kidney is located lower than usual and palpate in the hypochondrium. At the same time, the nephroptosis diagnosis is mistakenly established. A distinctive feature of nephropotosis from dystopia is the fact that when omitting the vessels, I direct down to the kidney from top to bottom, and during diSTopia go perpendicularly. Rarely distilled P. causes abdominal pain.

Iliac dystopia - The organ is located in the iliac yam, the vessels usually have multiple character and depart from the general iliac artery. The main sign of iliac dystopia is pain in the abdomen. It is also possible to appear signs of violation of urine outflow.

Plumber dystopia - The body is located deep in the pelvis, between the bladder and the rectum. Such a location disrupts the work of the rectum and the bladder and is accompanied by the corresponding symptomatics.

Thoracic dystopia - The organ is located in the chest cavity. This vice is rare and occurs mainly to the left. Often can manifest themselves the appearance of pain especially after eating. Usually diagnosed by chance.

Cross Distopia. - One of the kidneys in the process of its intrauterine development moves to the opposite direction. At the same time, on one side is determined by 2 organs. It is rare. It should also be said that the crossing dystopia is sometimes accompanied by a merger of two kidneys. When revealed on one side of two kidneys at once, nephroptosis should be excluded. Operational treatment is shown only in identifying secondary pathological processes (pyelonephritis, nephrolity, hydronephrosis, tumor).

Anomalies of Relationship - Breathing Kidney

Depending on the type of kidney fusion, several types of singles are distinguished:

Galeto-shaped kidney - Battle of the kidneys along the medial surface.

S-shaped - The battle of the upper pole of one kidney with the lower pole is different.

L-shaped (sticky) - Also occurs the battle of the upper pole of one kidney with the lower pole of another, but at the same time the first kidney unfolds, as a result, an organ resembling the letter L. is formed.

Horseshoe - The battle of the upper or lower poles. As a result, the organ resembles the horseshoe.

Anomalies of the number of kidneys

Aplasia(Agnesia)the ureter occurs very rarely and is 0.2% of kidney anomalies and urinary tract. Bilateral anomaly is usually combined with bilateral kidney anesthesia, less often with bilateral multistone kidney. This anomaly has no clinical value, since incompatible with life.

One-sided aplasia of the ureter is also an integral element of renal aplasia and the result of the absence of a ureteral sprout. Sometimes the ureter is detected in the form of a fine fibroids or an outflow that ends blindly.

The diagnosis of the aplaty of the ureter is based on the data of an excretory urography, which allows you to establish the absence of a function of one of the kidneys. Cystoscopy detects the hypoplasia or the complete absence of a half of the urohydrous triangle. The hole of the ureter can be located in an ordinary place, but be narrowed. With long observation, it is possible to detect the absence of its abbreviations. Sometimes the hole has a blind deepening look, which is determined when the catheter is administered, or blindly ends at any level. In these cases, cystography is quite informative. With a rudimentary branch of the ureter, it is recommended to carry out ultrasound examination, computed tomography.

The need for treatment occurs only at the blind end of the ureter, since this anomaly can cause an inflammatory process, sometimes with the fitting (emphasis), the formation of stone. In the case of scarring, a closed cavity resembling a cyst or a tumor of the abdominal cavity is formed.

Such complications are manifested by pain in the appropriate groin or styling area, disurium, an increase in body temperature for the intermittent type, phenomena of chronic intoxication. Watering contains a large number of leukocytes, protein, bacteria. In the presence of the stone, the macro or microhematuria is detected.

The treatment is to remove the ureter's cult.

Doublingureteral - One of the most numerous anomalies (1: 140). It is due to the simultaneous growth of two ureterals with two ureteral sprouts of nephrogenic blasmore or splitting of a single ureteral sprout. One of the ureters can develop normally, and the second is pathologically. If several bikes of the ureter are formed in the caudal duct of the primary kidney, it is possible not only a doubling, but also the tripling of morphologically full-fledged ureters. Two ureterals correspond to two renal loins, which are urine collectors for different kidney ends.

In such cases, the kidneys are rarely separate. Third, additional, kidney is formed.

Sometimes two ureterals and more depart from the pelvis of the non-double kidney or the proximal end of one of the ureters ends blindly. Both ureterals usually pass in one fascial case.

There is a complete (Ureter Duplex) and incomplete (Ureter Fissus) doubling of ureters. In case of incomplete doubling, both ureter departs from the renal loching down to the bladder and at different distances are merged into one. In this case, one hole is manifested in the bladder - a split ureter. Sometimes ureterals merge near the bladder, intravenously (intraminally) or even in the area of \u200b\u200bthe hole. One of the ureters falls into another at an acute angle.

As a rule, the length of both ureters from the lachangly ureteral segment to the place of merger is different and the sections of both ureters above it are located in different phases of the peristaltics. In case of incomplete doubling, the splitting is observed mainly in the upper third of the ureter, less often - in the middle, and in 1/3 of patients - in the lower.

The doubling of the ureter - both complete and incomplete, is more likely to be one-sided. Localized on both sides with the same frequency.

With full doubling, both ureter go separately to the bladder. They are closely adjacent to the walls, according to the Waireger-Meyer law they are crossed in proximal and distal departments and are opened by two holes on the corresponding half of the uroin triangle (one over the other or nearby), if there are no ectopia of one of them. In the bladder, the holes of the ureter of the upper pelvis are almost always contained below the holes of the ureter of the bottom lochank. Bubble-ureter reflux is more often observed with full doubling of ureters. This is due to the short intravenous department of the ureter, which opens proximal. Occasionally, the blind end of one of the twin ureters is observed. Anomaly is manifested by pain in the styling area and the symptoms of the inflammatory process.

The doubling of ureters is often combined with other defects of development: the absence of both or one ureteral holes, narrowing (ureter stricter, ureterocele, the ectopia of the hole of one of the ureters (more often), segmental or common dysplasia of the neuromuscular elements of ureters, aberrant vessels, spikes, fibroids, and t .P..

With a double urgench characteristic symptoms, it is not observed. For a long time, anomaly has an asymptomatic flow. Clinical manifestations occur when complications appear. Symptomatics is determined by the character and stage of complications or combined anomalies.

bladder kidney treatment

2. Urinary Bubble Development Anomalies

Anomalies blades. Urine duct, or urehus, the human embryo is the residue of Alantois and will fill with the transition of the fetus for placental blood flow at an early stage of intrauterine development. Despite the loss of functions (the supply of fetus with oxygen, protein substances, an excretory function), the reverse development of the atmosphere is not observed and the former urine duct is preserved. It participates in the formation of the umbilical cord, and over time turns into a median umbilical bunch. By the time the urine duct is bored and acquires the type of solid heavy, which is gradually atrophied.

In some cases, urinary duct remains open throughout (complete unction - bubble-poucher fistula) or in separate areas (blind ending of an unmatched external section - Undermined fistula; internal department - urinary bubble diverticulum, ureraus cyst).

In some cases, in particular, in premature babies, urinary duct obliteration occurs in the first year of life. But often there is a partial incorrect, which is not clinically manifested in adults.

Partial and complete unction of the blade is an anomaly, but they pay attention only when certain symptoms appear. Full unguardment of urinary ducts - bubble-poucher fistula - is observed extremely rarely. It is more often an unguarer of one of its sections: Uppernogogogo - Umbilical fistula; Medium - Cyst Urerahus; Bubble - diverticulous bladder.

If the urine duct is completely unfolded, the separation of urine from the navel is observed, more often - when urination. At the same time, most of the urine is distinguished through the urethra. This ratio depends on the diameter of the lumen of the unburited duct. Sometimes the separation of urine from the navel is stopped (the swelling of the mucous membrane of the blade channel, filling with its granulations or protein mass), and then resumes. Skin of the octopup macery through constant urine leakage. In some cases, the granulation tissue tuber is turned out (a plot of preserved umbilical cord).

The unguardment of the middle part of the urinary duct is also observed quite rarely. At the same time, since both ends of the duct are refused, between them remains the blind section of the channel. The epithelium, the lining channel, produces a mucus that accumulates in the channel, stretches its eschiba, gives them a rounded form. Gradually, a cyst is formed in the place of formation, which is palpable between the naughty and the bladder in the form of a rounded or oval low-altitude tumor of various sizes.

Agnesia of the bladderThat is, its innate absence, extremely rare anomaly. It is usually combined with other defects of development and incompatible with life.

Urinary bubble doubling Observed very rarely. With this anomaly, there is a partition between the right and left half of the ureage bubble. Each half opens a hole of the ureter. Each half of the bladder has a neck. Full doubling of the bladder is combined with doubling the urethra.

In case of incomplete doubling, the bladder has a shade and one urethra. Very rarely observed a complete bubble septum, which divides it in the sagittal or frontal direction.

The treatment consists in cutting the septum of the bladder.

Congenital muscle hypertrophy, pushing the urine (detour), it is very rare. Patency of the bubble-ureter segment is not violated. Anomaly leads to the defeat of the upper urinary tract and kidneys due to the compression of the invential parts of both ureters.

The only way of treatment is the removal of the bladder and replacing its intestinal loop.

Urinary bubble diverticulus - Basch-like protrusion of its wall. It can be solid and multiple. The cause of a congenital diverticulus is the wrong formation of the bladder wall. More often the diverticulus is formed near the holes of the ureters and in the side waters of the bladder, less often on its top or in the bottom area. The wall of the diverticula consists of the same layers as the wall of the bladder. It is often observed a diverticulum of large sizes, which exceeds the bladder by volume.

The anomalies of the development of the urethra include:

Doubling the urethra.

Congenital diverticulum of the urethra.

Hypospadia is cleaner of the rear wall of the urethra. Such anomaly of development is more common in boys.

Epispadia - the incoming of the dorsal (front) wall of the urethra. Epispadia is celebrated in 1 of 50,000 newborns, boys are five times more often than in girls.

Infraezicular obstruction is an obstacle to urine outflow at the level of the bladder neck or urethra. Infraezicular obstruction may be due to congenital contracture of the bladder neck, congenital valves of urethra, hypertrophy of the seed tuberca or congenital obliteration (in advance) of the urethra.

3. M.etododas of diagnostics of urinary system

For early diagnosis of congenital defects, ultrasound playing a huge role during pregnancy. It allows you to determine with great accuracy to the lowland, which is most often as follows to the amount of urine released in the absence of kidney or urine stagnation in urinary tract. If the vice appears before 28--32 weeks of pregnancy, it leads to underdevelopment of lungs and vices in the structure of the bodies of the fetus. Diagnosis of kidney cyst and urine stagnation in the urinary ways of the fetus to a large extent depends on the capabilities of the device. In the case of suspicion of malformations of urinary tract, labor should be carried out in clinics, where there is a neonatologist "Children's nephrologist and urologist.

In the pathological course of pregnancy, especially small alternates and congenital vices of the urinary system in relatives, it is possible to assume the malformations of the urinary system already at the fetus. The lack of diuresis (urinations) in the first two days of the newborn life, impairment of urinary and the change in the number of urine compared to the age norm should be indications of the study of the urinary system.

The flavors of the kidneys and the urinary tract are more likely in children with other defects of development than in the overall population. In some children, the first study after birth reveals in the abdominal cavity signs of hydronephrosis: a significant increase in the kidneys, bladder or huge ureters. The vices of the spine, spinal cord or the absence of sacral vertebrae may indicate the neurogenic bladder.

The diagnosis of insufficient kidney function makes it difficult to preserve conventional urinary in the period of newborn. In case of violation of urinary, efforts when urinating, an intermittent urine jet in boys can suspect the presence of an obstacle in the rear of the urethra, and the urinary accustomed to the urine in girls - the videos of the bladder. For some newborn hydronephrosis, associated with the presence of obstacles in the urinary tract, can be identified only after the second day when "the physiological separation of a small amount of urine" passes. After discovering on ultrasound expansion of urinary tract, cystography is carried out, which is based on the introduction of a contrast agent through the urethra in the bladder.

X-ray shift when the child urins. During the day before cystography, on the day of research and two days after research, the child must take drugs warning the development of infection in the catheterization and the introduction of contrast to the bladder (although aseptic measures are used). The combination of cystography and ultrasound allows to investigate urine selection from the bladder and urine, as well as to prove the presence of an obstacle in the rear of the urethra. In cases of infection of urinary tract, cystography should be carried out in the absence of changes to the ultrasound and only after the treatment of infection.

Scintigraphic study using isotopes helps in assessing the kidney function. Exposure with this study is significantly less.

Conclusion

Anomalies of the urogenital system are most frequent of all congenital malformations. Under congenital defects, resistant changes in tissues or organs that go beyond the limits of the variations of their structure are understood. The formation of such vices occurs as a result of a violation of the normal course of the intrauterine development of the embryo. The frequency of coarse congenital malformations, accompanied by a violation of functions, in a person population is 2 - 3%.

It is believed that the most frequent anomalies of the urinary system arise due to the influence of hereditary factors and various negative impacts on the fetus during the period of intrauterine development. Anomalies of the urinary system can develop in a child due to the transferred by the mother in the first months of pregnancy rubella, syphilis. It can provoke an emergence of anomalies alcoholism and drug addiction, use of hormonal contraceptives during pregnancy, as well as drugs without appointing a doctor.

Bibliography

1. Markosyan A.A. Issues of age physiology. - M.: Enlightenment, 1974

2. Sapin M.R. - Anatomy and human physiology with age characteristics of the children's body. - Publishing Center "Academy" 2005

3. Petrishina O.L. - Anatomy, physiology and hygiene children of younger school age. - M.: Enlightenment, 1979

4. N. V. Krylova, T. M. Sobolev "Moochephetical Agency", Anatomy in Schemes and Drawings, Publishing House of the Russian University of Friendship of Peoples, Moscow, 1994.

5. "Urology Guide" Edited by N. A. Lopatkin, Publisher "Medicine", Moscow, 1998. Free download "urinary system of man" in full

Internet sources

1. http://kotikit.ru/qanda/anomalii-mochevydelitelnoj-sistemy/

2. http://hvoroby.ru/systemy/47-anomalii -systemy.html.

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Fire development vices:

Violation of the development of the limbs can lead to the absence of limbs, fingers or part of the limb, to the appearance of additional limbs, fingers. Increased limb ( macromelia) or individual fingers ( mcShodtakia) It is often associated with circulatory impairment - the presence of arteriovenous fistula. No limb can be complete - etomeliamaybe the absence of the distal part of the limb - brushes, foot, shin, forearm - hemimelia. Lack of proximal part of the limb - shoulder, hips, as a result of which normally developed skin, forearm, brush or foot start from the body, called focomelia. Improving the limb function can be achieved only with prosthetics, which is used in children to ensure their growth and development.

Congenital dislocation of hips. Prevalence - 1 case per 1,000 newborns. It is expressed in violation of the position of the head of the femur, which shifts and is located outside the articular depression. Dislocation can be bilateral. It is determined not only to violate the position of the elements of the hip joint, but also the structural change of them: the head of the femoral bone is underdeveloped - its hypoplasia is noted, the articular of the iliac bone is thickened.

Modernly diagnosed dislocation allows you to carry out a complete correction. The newborn inspects immediately after birth; At the same time, the violation of passive movements in the joint is a leading, rotation - characteristic of dislocation of the thigh. If dislocation is not diagnosed in a timely manner, then with the development of the child there is a further displacement of the head of the femoral bone, and dislocation detect when the child begins to walk. The gait is sharply violated: the child goes over with his legs on the leg ("duck" gait), a shortening of the legs is marked. The appearance of the child when inspecting it standing in the profile is characteristic: pronounced lumbar lordosis, pelvic deformation, shortening limb. Radiography allows not only to clarify the diagnosis, but also determine the degree of hypoplasia of the joint surfaces and the position of the femoral bone.

Treatment: Dislocation provides for the elimination of the head offset - the headflow of the head and the immobilization of the limb with special orthopedic devices or a gypsum bandage.

Congenital closure of the foot It is found in 1 child from 1,500 newborns. The diagnosis is easily installed in the form and position of the foot.

Treatment: You should start as early as possible. It includes manual stopping of the foot and fixing it, massage and therapeutic physical culture. In the later dates, surgical treatment are used: crossing ligaments, tendon transplant or wedge-shaped foot bones with the installation of the foot in the correct position and fixation of the gypsum bandage.

Arthrogriposis - Multiple joint contractures due to the underdevelopability of muscles of limbs with symmetric localization. Tugidity, restriction of movements require conservative therapy (massage, healing physical culture, physiotherapeutic treatment).

Syndactilia - Development vice, expressed in the presence of battles between the fingers. The battle of the fingers can be skin or bone. The malformation is due to imbigenesis impaired: up to 2 months. The intrauterine life fingers are connected by membrane, and then divided. The separation of the fingers is carried out by the surgical method at the age of 2-3 years.

Polydactile - Increase the number of fingers. It is found both in his hands and on the legs, may be accompanied by a violation of the function of the brush or foot. Surgical treatment - removal of extra fingers.

McPrattyl - an increase in the volume of individual fingers. If the vice leads to a violation of the function of the brush or foot, amputation of the finger.

Ecroids - Reducing the number of fingers. Maybe the absence of one or more fingers on hand or leg. To restore the function of the brush and eliminate the cosmetic defect, resort to the transplant of the fingers from the foot on the brush using microsurgical techniques.

Anomaly(from Greek. anomalia -deviation, irregularity) is a structural and / or functional deviation due to disruption of embryonic development. The anomalies of the urinary apparatus are widespread and amount to about 40% of all congenital vices. According to autopsy, about 10% of people have various anomalies for the development of the urogenital system. To understand the reasons for their occurrence, it is necessary to highlight the basic principles of the formation of urinary and sexual systems. In its development, they are closely connected with each other, and their output ducts are opened in the total genital sinus (Sinus Urogenitalis).

Embrygenesis of the urogenital system

The urinary system develops not from one primary, but is represented by a number of morphological formations that consistently replacing each other.

1. Head kidneyor bounce (PRONEPHROS).In humans and higher vertebrates, she quickly disappears by replacing more important primary kidney.

2. Primary kidney (mesonephros)and its dashing (DUCTUS MESONEPHRICUS),which occurs before all the formations involved in the formation of urinary organs. On the 15th day, it appears in the mesoderm in the form of nephrotic shutter on the medial side of the body cavity, and at the 3rd week it reaches a cloac. Mesonphros.it consists of a number of transverse tubules located media from the upper mesonefral duct and one end of the flowing into it, while the other end of each tube ends blindly. Mesonphros.- Primary secretory body, with an output duct of which is the Meso-Nephiral Doc.

3. Paramesenefrica ductus ParameSonphricus.At the end of the 4th week along the outdoor side of each primary kidney, the longitudinal thickening of the peritoneum appears due to the development of the epithelial tape, which at the beginning of the 5th week turns into the duct. It opens a few kidney from the front end of the primary kidney into its cranial end.

4. Sex glandsthere are relatively later in the form of a cluster of an embryonic epithelium from the medial side. mesonphros.Seed egg tubes and eggs comprising ovarian follicles develop from embryonic epithelial cells. From the lower pole of the header down on the wall of the abdominal cavity stretches the connecting tapered (Gubernaculum Testis)- Egg conductor, which its lower end goes into the groin canal.

The final formation of the urinary organs is as follows. From the same nephrogogenic heavy, from which the primary

naya kidney, constant kidneys are formed (Metanephros),From the nephrogogenic shuttle, the actual parenchyma of constant kidney (urinary channels) is developing. Starting from the 3rd month, constant kidneys as functioning excretory organs are replaced by primary. With the increasing body of the kidney, as it were, move up and occupy their place in the lumbar region. Lohanka and ureter develop at the beginning of the 4th week from the diverticulus of the caudal end of the mesonephral flow. In the future, the ureter is separated from the me-Sonfral duct and flows into the part of the cloaca, from which the bottom of the bladder develops.

Cloaca- Overall cavity, where the urinary, genital paths and rear are originally open. It has a blind bag, closed outside with a cloacional membrane. In the future, inside the clock, a front partition occurs, which divides it into two parts: ventral (Sinus Urogenitalis)and dorsal (rectum).After the breakthrough of the cloacional membrane, both of these parts are opening out with two holes: sinus Urogenitalis- front, reservoir of the urogenital system, and rectum- Rear aisle (ANUS).

With urogenital sine connected urinary bag(ALLANTOIS),which in the lower vertebrate serves as a reservoir for the products of the renal selection, and in humans, part of it turns into the bladder. Allantois consists of three departments: nizhny- sINUS UROGENITALISfrom which the bladder triangle is formed; middle Extended Departmentwhich turns into the rest of the bladder and upper pressed departmentrepresenting the urinary move (Urachus),running from the bladder to the navel. In the lower vertebrates, it serves to disrupt the contents of Allantois, and in humans - by the time of birth, it starts and turns into fibrous litter (Lig. Umbilicale Medianum).

Ductus ParameSonphrici.give rise to development in women of uterine pipes, uterus and vagina. The uterine tubes are formed from the upper parts ductus ParameSonphrici,and the uterus and the vagina are from the spilled lower parts. In men ductus ParameSonphrici.reducing, and only science eggs remain from them (Appendix Testis)and prostatic uteros (Utriculus prostaticus).Thus, men reduction and transformation into rudimentary education are subjected to ductus ParameSonphrici,and in women - ductus Mesonphrici.

Around a sliding hole sinus Urogenitalisat the 8th week of intrauterine development, the primitive of the external genital organs is noticeable, initially the same in male and female germs. The front end of the outdoor, or sex, the sinus gap is lying in a sex tubercle, the edges of the sine are formed by urinary folds, gender tuberculos and sex folds are surrounded outside with long-mochelted tuberculos.

In men, these primitives are subjected to the following changes: sexual tuberculosis develops greatly, it is formed out of it penis.Along with its growth, the slot is increasing, located under the bottom surface penis.In the future, when the urinary folds grow together, this slit forms the urethra. Lifting-scrutinating tubercles grow strongly and turn into a scrotum, firing in the middle line.

In women, sex bumps turns into the clitoris. Ground sex folds form small sexual lips, but a complete connection

folds does not happen and sinus Urogenitalisit remains open, forming the spelling of the vagina (Vestibulum Vaginae).Long-scrutinating tubercles, which are then turning into large sex lips.

As a result of the close relationship of the development of the urinary and sexual system in 33% of cases of anomaly of the organs of the urinary system combined with the anomalies of the genital organs. The defects of the development of the urogenital system are often associated with the defects of the development of other organs and systems.

5.1. Kidney anomalies Classification

Anomalies of renal vessels

■ Number anomalies: solitary renal artery;

segmental renal arteries (double, multiple).

■ position anomalies: lumbar; iliac;

the pelvic dystopia of renal arteries.

■ Anomalies of the shape and structures of arterial stems: renal arterial aneurysms (single and bilateral); fibromascular stenosis of the renal arteries; Cheniform renal artery.

■ Congenital arteriovenous fistulas.

■ Congenital changes in kidney veins:

anomalies of the right renal vein (multiple veins, inventions of veins of eggs in the renal vein on the right);

anomalies of the left renal vein (ring-shaped left renal vein, Ret-rooortyl left renal vein, extracavative liner of the left renal vein).

Anomalies of the number of kidneys

■ Aplasia.

■ Doubling kidney (full and incomplete).

■ Additional, third kidney.

Anomalies of kidney magnitude

■ kidney hypoplasia.

Anomalies of the location and form of kidneys

■ Kidney Distopia:

one-sided (breast, lumbar, iliac, pompous); Cross.

■ Skilled kidney: one-sided (L-shaped kidney);

bilateral (horseshoe, galley-like, asymmetric - L- and S-shaped kidneys).

Anomalies of the structure of the kidneys

■ Kidney dysplasia.

■ Kidney multipoints.

■ Kidney Polycystic: Polycystic Adults; Polycystic children's age.

■ Solitary kidney cysts: simple; Dermoid.

■ Parapelvikal cyst.

■ Diverticulous cups or lochanks.

■ Chescho-medullary anomalies: spongy kidney;

megakalix, Paul Levygakalix.

Combined kidney anomalies

■ with bubble-ureter reflux.

■ with infraezical obstruction.

■ with bubble-ureteral reflux and infraezical obstruction.

■ with anomalies of other organs and systems.

Anomalies of renal vessels

Anomalies of quantity.These include the blood supply to the kidney by solitary and segmental arteries.

Solitary renal artery- This is a single arterial trunk, separating from the aorta, and then divided into relevant renal arteries. This pounding of the renal blood supply is a causual.

Normally, each kidney is bloodshed in one separate arterial barrel separating from the aorta. An increase in their amount should be attributed to the segmental scatching type of the building of the renal arteries. In the literature, including training, often one of two arteries, blood supplying kidney, especially if it is smaller diameter, called additive.However, in anatomy of an additional, or aubranding, the artery is considered to be the blood supply to a certain area of \u200b\u200bthe body in addition to the main artery. Both of these artery form a wide network of anastomoses in their common vascular pool. Two or more kidney arteries are bloodtained each one defined segment and do not form among themselves in the process of dividing the anastomoses.

Thus, in the presence of two or more arterial kidney vessels, each of them is for it the main, and not added. Ligation of any of them leads to necrosis of the corresponding portion of the renal parenchyma, and this should not be done when performing corrective operations during hydronephrosis due to the lower-insulated kidney vessels, if its resection is planned.

Fig. 5.1.Multispiral CT, three-dimensional reconstruction. Multiple segmental type of renal arterial structure

From these positions, anomalous one should consider the amount of renal arteries more than one, that is, the segmental type of blood supply to the organ. The presence of two arterial stems, regardless of their caliber - double (double) renal artery,and with their larger number - multiple Type of Building Arteries Kidney(Fig. 5.1). As a rule, this vice of development is accompanied by a similar structure of renal veins. Most often it is combined with the anomalies of the location and the number of kidneys (double, dystoped, horseshoe kidney), but may be observed in the normal structure of the organ.

Anomalies of the position of the renal vessels - Development vice, characterized by atypical dishevement of the renal artery from the aorta and determining the view of the kidney dystopia. Highlight lumbar(with a low emission of the renal artery from the aorta), iliac(when a total iliac artery from the overall) and taza(If you leave from the internal iliac artery) dystopia.

Anomalies of form and structure.Aneurysm of renal artery- Local expansion of the artery, due to the absence of muscle fibers in its wall. This anomaly, as a rule, is one-sided. Anneurysm of the renal artery can be manifested by arterial hypertension, thromboembolism with the development of kidney infarct, and when it is ruptured - massive internal bleeding. With aneurysm of the renal artery, surgical treatment is shown. Receive resection of the aneurysm, stitching the defect of the vascular wall

or renal artery plastic synthetic materials.

Fibromuscular stenosis- Anomaly of the renal arteries caused by excessive content in the vascular wall of fibrous and muscle tissue (Fig. 5.2).

This malforming is more common in women, often combined with nephroptosis and can be bilateral. The disease leads to a narrowing of the lumen of the renal artery, which is the cause of the development of arterial hypertension. Its feature at fibromous

Fig. 5.2.Multispiral CT. Fibromuscular stenosis of the right renal artery (arrow)

Fig. 5.3.Selective kidney arteriogram. Multiple arteriovenous fistulas (arrows)

large stenosis is high diastolic and low pulse pressure, as well as refractoriness for hypotensive therapy. The diagnosis is made on the basis of kidney angiography, multispiral computer angiography and radioisotope studies of the kidneys. Perform selective blood intake from renal vessels to determine the concentration of renin. Treatment operational. Balloon dilatation (expansion) of the stenosis of the renal artery and / or installation of the arterial stent is carried out. If it is impossible or ineffectiveness of angioplasty or stentirov, perform a reconstructive operation - renal artery prosthetics.

Congenital arteriovenous fistulas - Pulk development of renal vessels, in which there are pathological fatters between vessels of arterial and venous blood circulation systems. Arteriovenous fistulas, as a rule, are localized in arcuate and lolly kidney arteries. The disease often proceeds asymptomatic. Possible clinical manifestations of it can be hematuria, albuminuria and varicocele on the appropriate side. The main method of diagnosing arteriovenous fistula is renal arteriography (Fig. 5.3). Treatment is endovascular occlusion (embolization) of pathological stations with special embols.

Congenital change in renal veins. Anomalies of the right renal vein are observed extremely rarely. Among them are most often found increase in the number of venous trunks (doubling, tripling).The defects of the development of the left renal vein are presented anomalies for its quantity, shape and position.

Additional and multiple buds of the kidneys are found in 17-20% of cases. The clinical value is that those of them that go to the lower pole of the kidney accompanying the appropriate artery, crossed with the ureter, thereby causing a violation of the outflow of urine from the kidneys and the development of hydronephrosis.

For form and location anomalies ring-shaped(passes two trunks around the aorta), retrooactive(It takes behind the aorta and flows into the lower hollow vein at the level of II-IV lumbar vertebrae) extracaval-nyu(It does not fall into the lower hollow, but more often in the left overall iliac venu) renal veins. The diagnosis is based on the data of the vocalovography, selective renal episode. In cases of pronounced venous hypertension, it is resorted to an operation - imposing an anastomosis between the left testicular and general iliac veins.

In most cases, abnormal kidney vessels do not show themselves and are often a random find when examining patients, but information about them is extremely important when planning operational interventions. Clinically, the defects of the development of renal vessels are manifested in cases where they cause a disturbance of urine outflow from the kidneys. The diagnosis is established on the basis of Doppler ultrasound scanning, aorto and ve-nocavigraphy, multispiral CT and MRI.

Anomalies of the number of kidneys

Aplasia- Congenital absence of one or both kidneys and renal vessels. Bilateral kidney aplasia is incompatible with life. Aplasia of one kidney occurs relatively often - in 4-8% of patients with kidney anomalies. It arises due to underdevelopment of metanephrogenic fabric. At half past cases on the side of the aplasia, the kidney does not have the corresponding ureter, in other cases the distal end ends blindly (Fig. 5.4).

The aplasia of the kidney is combined with the anomalies of the genital organs in 70% of girls and in 20% of boys. For boys, the disease occurs 2 times more often.

Information on the presence of a single kidney in a patient is extremely important, since the development of diseases in it always requires special treatment tactics. The only kidney is functionally more adapted to the influence of various negative factors. In renal Aplasia, it is always observed with its compensatory (vicar) hypertrophy.

Fig. 5.4.Aplasia left kidney and ureter

An excretory urography of Jui allow you to detect the only, enlarged in the sizes of the kidney. A characteristic feature of the disease is the lack of kidney vessels on the aplasia side, so the diagnosis is reliably established on the basis of methods to prove the absence of not only kidneys, but also its vessels (renal arteriality, multispiral computer and magnetic resonance angiography). The cystoscopic pattern is characterized by the absence of an appropriate half of the intertaround fold and the mouth of the ureter. With a blindly end, the uterine of its mouth is hypotrophized, abbreviation and separation of urine. Such a variety of vice is confirmed by the catheterization of the ureter with the implementation of retrograde urset-rography.

Fig. 5.5.Sonogram. Doubling kidney

Doubling kidney- The most common anomaly for the number of kidneys is found in one case by 150 autopsy. In women, this malformation is observed 2 times more often.

As a rule, each of the half of the double kidney has its own blood supply. Characteristic for such anomaly is anatomy-functional asymmetry. Upper half is more often less developed. Symmetry of the organ or predominance in the development of the upper half is much less common.

Kidney doubling can be one-and bilateralas well as fulland incomplete(Fig. 41, 42, see col. Plot). Full doubling implies the presence of two cup-making systems, two ureterals opening with two guests in the bladder (Ureter duplex).In case of incomplete doubling, ureterals are ultimately merged into one and open by one mouth in the bladder (Ureter Fissus).

Often, the full doubling of the kidneys is accompanied by an anomaly for the development of the lower unit of one of the ureters: its intrainyl intapulusive ectopia

Fig. 5.6.Excretory Urograms:

but- incomplete doubling of urinary tract on the left (Ureter Fissus); B.- Full doubling of the urinary tract on the left (Ureter Duplex)(arrows)

(Opening in the urethra or vagina), the formation of the ureterocele or the inconsistency of bubble-ureteral coordinations with the development of reflux. A characteristic sign of ectopia is constant urine leakage with a stored normal urination. A doubled kidney, not affected by any disease, does not cause clinical manifestations and is found in patients with a random survey. However, it is more likely than normal, subject to various diseases, such as pyelonephritis, urolithiasis, hydronephrosis, nephroptosis, neoplasms.

The diagnosis does not represent complexity and is to carry out an ultrasound (Fig. 5.5), an excretory urography (Fig. 5.6), CT, MRI and endoscopic (cystoscopy, catheterization of ureters) research methods.

Operational treatment is performed only in the presence of violations of urodine-Miki associated with the anomalous move of ureters, as well as other doubted kidney diseases.

Extreme kidney- Extremely rare abnormal kidney number. The third kidney has its own blood supply system, fibrous and fat capsules and ureter. The latter flows into the ureter of the main kidney or opens with an independent mouth in the bladder, and in some cases it can be ectopied. The dimensions of the added kidney are significantly reduced.

The diagnosis is established on the basis of the same methods as with other kidney anomalies. Development in additional kidney such complications as chronic pyelonephritis, urolithiasis and others serves as an indication of nephrectomy.

Anomaly of the magnitude of the kidneys

Kidney hypoplasia (dwarf kidney)- congenital decrease in the organ in the amount with the normal morphological structure of the renal parenchyma without disturbing its function. This malformation, as a rule, combines-_ Xia with an increase in control

kidney. The hypoplasia is more likely to be one-sided, much less often is observed on both sides.

One-sidedrenal hypoplasia can not be clinically manifested, but in abnormal kidney pathological processes develop much more often. Bilateralthe hypoplasia is accompanied by symptoms of arterial hyper-tres and renal failure, the severity of which depends on the degree of congenital defect and complications that arise mainly due to the attachment of infection.

Fig. 5.7.Sonogram. Pelvic dystopia hypoplasmic kidney (arrow)

Fig. 5.8.Scintigram. Gayoplasia of the left kidney

The diagnosis is usually installed on the basis of ultrasound diagnostic data (Fig. 5.7), excretory urography, CT and radioisotope scanning (Fig. 5.8).

Special difficulties represent the differential diagnosis of hypoplasia from dysplasiaand crumbled as a result of nephrosclerosis kidney.In contrast to dysplasia, this anomaly is characterized by a normal structure of the renal vessels, a cup-laughter and ureter. NEFROM CLEAR is more often the outcome of chronic pyelonephritis or develops as a result of hypertension. Rubtsovaya rebirth of the kidney is accompanied by the characteristic deformation of its contour and cups.

Treatment of patients with hypoplasic kidney is carried out with the development of pathological processes in it.

Anomaly of the location and form of kidneys

Kidney Location Anomaly - distopia.- Finding the kidney in a non-typical anatomical area for it. This anomaly is found at one of 800-1000 newborns. Left kidney turns out to be dystoped more often than right.

The reason for the formation of this malformation is the violation of the movement of the kidney from the pelvis into the lumbar region during the period of intrauterine development. Distopia is due to the fixation of the kidney in the early stages of the embryonic development of an abnormally developed vascular apparatus or insufficient growth of the ureter in length.

Depending on the location level distinguish thoracic, lumbar, sacral-iliacand pelvic dystopia.(Fig. 5.9).

Kidney anomalies can be one-sidedand bilateral.Distopia Kidney without displacement of it on the opposite direction gomolateral.The dystoped kidney is located on its side, but above or below the normal position. Heterolateral-Naya (Cross) Distopia- A rare malformation, detected with a frequency of 1: 10,000 autopsy. It is characterized by a shift in the kidney on the opposite direction, as a result of which they are located on one side of the spine (Fig. 5.10). At cross-dystopia, both ureter open in the bladder, as well as at the normal arrangement of the kidneys. The urinary triangle is saved.

The dystoped kidney may cause constant or periodic pain in the appropriate half of the abdomen, the lumbar region, the sacrum.

Fig. 5.9.Types of kidney dystopia: 1 - thoracic; 2 - lumbar; 3 - cross-iliac cross; 4 - pelvis; 5 - Normally Located Left Kidney

Fig. 5.10.Heterolateral (Cross) Distopia of the right kidney

An abnormally located kidney is often able to try through the front abdominal wall.

This anomaly is in the first place among the causes of erroneously produced surgical interventions, as the kidney is often taken for the tumor, the appendicular infiltrate, the pathology of the female genital organs, etc. The pyelonephritis, hydronephrosis, urolithiasis, are often developing in dystopedania kidneys.

The greatest difficulties in the diagnosis causes the pelvic dystopia. Such a kidney location can manifest itself with pain at the bottom of the abdomen and simulate acute surgical pathology. Lumbar and iliac dystopias, not even complicated by any disease, can manifest themselves with pain in the relevant area. Pain with the most rare thoracic dystopia kidney is localized by the sternum.

The main methods of diagnosis of kidney position anomalies are ultrasound, x-ray, CT and renal angiography. The lower there is a dystoped kidney, the more ventrally arranged its gate and the Kepened Lohanka is rotated. With ultraster and excretory urography, the kidney is located in an atypical location and as a result of turning, it becomes flattened (Fig. 5.11).

In case of insufficient contrasturation of the dystoped kidney, according to the excretory urography, retrograde ureteropelography is performed (Fig. 5.12).

Fig. 5.11.Excretory Urogram. Pelvic dystopia left kidney (arrow)

Fig. 5.12.Retrograde ureteropelogram. Plug Distopia Right Kidney (arrow)

The lower the dystopia organ, the shorter there will be a ureter. On the en-giograms, the kidney vessels are located low and can move away from the abdominal aorta, the bifurcations of the aorta, common ileum and graft arteries (Fig. 5.13). Characteristic is the presence of multiple nutritional kidney vessels. The most distinctly given anomaly is detected on a multispiral CT from

rubbing (Fig. 39, see col. Plot). The incomplete twist of the kidney and the short ureter serve as important differential diagnostic signs, allowing to distinguish the dystopia of the kidney from nephropotosis. Dis-topted kidney, unlike early stages of nephropotosis, devoid of mobility.

Treatment of dystopic kidney is carried out only in the case of the pathological process in them.

Formal anomalies include various types face kidneybetween themselves. Fraggated kidneys are found in 16.5% of cases among all their anomalies.

The battle implies the connection of two kidneys into one body. Cro-

Fig. 5.13.Renal angiogram. Pelvic dystopia left kidney (arrow)

it is always carried out by anomalous multiple renal vessels. In such a kidney there are two cup-making systems and two ureter. Since the fighting occurs in the early stages of embryogenesis, the normal rotation of the kidneys does not occur, and both lochanks are on the front surface of the organ. An abnormal position or compression of the ureter Nizhneopolar vessels leads to its obstruction. In this regard, this anomaly is often complicated by hydronephrosis and pyelonephritis. It can also be combined with a bubble-ureter-loching reflux.

Depending on the interjection of the longitudinal kidney axes, horseshoe-shaped, galete-shaped, S- and L-shaped kidneys are distinguished (Fig. 45-48, see col. Plot).

Battle kidney can be symmetricand asymmetric.In the first case, the kidneys will grow up with the poles of the same name, as a rule, lower and extremely rarely upper (horseshoe kidney) or average departments (galleo-shaped kidney). In the second - the battle occurs with variepete poles (S-, L-shaped kidneys).

Horseshoe kidneyit is the most frequent battle anomaly. More than 90% of cases there is a blowspill of kidney by the bottom poles. More than the kidney consists of symmetrical, the same than the size of the kidneys and there is a dystoped. The dimensions of the splicing zone, the so-called ister, can be the most different. Its thickness, as a rule, fluctuates in the range of 1.5-3, width 2-3, length - 4-7 cm.

When the location of one kidney is located in a typical place, and the second, fucked with it at right angles, across the spine - the kidney is called L-shaped.

In cases where in a split kidney, lying on one side of the spine, the gate is directed in different directions, it is called S-shaped.

Galeto-shapedthe kidney is usually located below the promontory in the field of small pelvis. The volume of the parenchyma of each half of the galete-shaped kidney is poured, which explains the asymmetry of the organ. Ureterals, as a rule, fall into the bladder in a normal place and very rarely crossed together with each other.

Clinically fascinated kidneys can manifest themselves with pain in a para-sciliary area. Due to the peculiarities of the blood supply and innervation of horseshoe kidney and the pressure of its coastal on the aorta, a hollow vein and solar plexus, even in the absence of pathological changes, characteristic arises

Fig. 5.14.Excretory Urogram. L-shaped kidney (arrows)

Fig. 5.15.CT with contrast (front projection). Horseshoe kidney. Weak vascularization of the coastal due to the predominance in it fibrous tissue

Fig. 5.16.Multispiral CT (axial projection). Horseshoe kidney

symptoms. With such a kidney, a typical appearance or enhancement of pain in the navel area during the inflection of the body back (symptom of roving). There may be disorders from the digestive organs - pain in the opposite region, nausea, bloating, constipation.

Uzi, excretory urography (Fig. 5.14) and multispiral CT (Fig. 5.15, 5.16) are the main methods of diagnosing fragmented kidneys and identify their possible pathology (Fig. 5.17).

The treatment is carried out in the development of diseases of the abnormal kidney (urolithiasis, pyelonephritis, hydronephrosis). When the hydronephrosis of hiker kidney is detected, it is necessary to determine whether it is a consequence of the obstructive segment characteristic of this disease (stricture, intersection of a ureter with a nizhneopolar vascular beam) or formed due to the pressure on it of horseshoe kidney. In the first case it is necessary

put the plastic of the pyeluretheral segment, and in the second - resection (and not dissection) is a coastal or ureterocalyacanastomy (nevertert operation).

Anomalies of the structure of the kidneys

Kidney dysplasia is characterized by a decrease in its size with simultaneous violation of the development of blood vessels, parenchyma, a cup-making system and a decrease in renal function. This anomaly arises as a result.

Fig. 5.17.Multispiral CT (front projection). Hydronefrotic Sky Transformation of Hiking Kidney

insufficient induction of duct metanephros on the differentiation of methane-frogenic blastime after their merger. Extremely rarely such an anomaly is bilateral and accompanied by severe renal failure.

Clinical manifestations of kidney dysplasia arise as a result of the accession of chronic pyelonephritis and the development of arterial hypertension. Differential and diagnostic difficulties arise with the difference between dis-plasses from hypoplasia and wrinkled kidney. The radial methods are helped in diagnosis, first of all, multispirate CT with contrast (Fig. 5.18), static and dynamic nephrocintigraphy.

The most common disorders of the structure of the kidney parenchyma structure are cortical cystic lesions (multi-storey, in Likistosis and solitary kidney cyst). The abnormal data combines the mechanism of violation of their morphogenesis, which is a dissonance compounds of the primary metanephrogenic vanity channels with a metanephros protocol. They differ in the deadlines of the violation of such a fusion during the period of embryonic differentiation, which determines the severity of structural changes in the kidney parenchyma and the degree of its functional failure. The most pronounced parenchyma changes incompatible with its function are observed during the kidney multipoint.

Multial kidney- A rare anomaly characterized by multiple cysts of different shapes and the magnitude engaged in the entire parenchyma, with the lack of its normal tissue and underdevelopment of the ureter. Intercristoid spaces are represented by connecting and fibrous fabric. Multi-ticky kidneys is formed due to a violation of the methane refrigerant blast, and the lack of laying an excretory when preserving the secretory apparatus of constant kidney in the early stages of its embryogenesis. Watering, forming, accumulates in the tubules and, without having a way out, stretches them, turning into cysts. The contents of the cyst usually there is a transparent liquid, remotely remind

putting urine. By the time of birth, the function of such a kidney is absent.

As a rule, the kidney multi-storeyosis is a one-way process that is often combined with the definitions of the development of the countercourse kidney and the ureter. Bilateral multi-cystosis is incompatible with life.

Prior to the attachment of infection, one-sided multistallic kidney is not clinically manifested and may be a random find at a dispensary examination. The diagnosis is set using Sonography and X-rayRedionuclide research methods with a separate definition of the kidney function. Unlike

Fig. 5.18.Multispiral CT. Left kidney dysplasia (arrow)

Fig. 5.19.Sonogram. Polycystic kidney

from polykistosis, multi-storey is always a one-way process with the lack of a struck function.

Treatment operational, consisting in nephrectomy.

Polycystic kidney- Development vice, characterized by replacement of the renal parenchyma with multiple cysts of various sizes. This is a heavy bilateral process, chronic pyelonephritis, arterial hypertension and increasing chronic renal failure are often joined to it.

Polycystosis occurs quite often - 1 case per 400 autopsy. In a third of the patients, cysts are detected in the liver, however, they are few and do not disturb the function of the organ.

In pathogenetic and clinical terms, this anomaly is divided into kidney polycystosis in children and in adults. An autosomal-recessive single type of transmission of the disease is characterized for polycystic disease of children's age, for polycystosis of adults - autosomal dominant. This anomaly in children is hard for children, most of them do not live to adult

rasta. Polycystic in adults has a more favorable course, manifested in young or middle age, and for many years wears compensated in nature. The average life expectancy is 45-50 years.

Macroscopically kidneys are increased in size due to a variety of cyst of different diameters, the amount of functioning parenchyma is minimal (Fig. 44, see col. Plot). The growth of the cyst causes ischemia of unchanged renal tubules and the death of the renal tissue. This process contributes to the joining chronic pyelonephritis and nephrosclerosis.

Patients complain about pain in the abdomen and lumbar region, weakness, fatigue, thirst, dry mouth, headache, which is associated with chronic renal failure and an increase in blood pressure.

Fig. 5.20.Excretory Urogram. Polycystic kidney

Fig. 5.21.CT. Polycystic kidney

Palparato easily, significantly enlarged dense buggy kidneys. Other polycystic complications are macrohematuria, the suppuration and malignation of the cyst.

In blood tests, anemia is noted, an increase in creatinine and urea. The diagnosis is established on the basis of ultrasound and radiode-radionuclide research methods. Characteristic signs are enlarged

the kidney measures are completely represented by varying cysts, squeezing of lochanks and cups, whose necks are elongated, the medial deviation of the ureter is determined (Fig. 5.19- 5.21).

Conservative treatment of polycystosis is symptomatic and hypotensive therapy. Patients are under dispensary observation of the urologist and the nephrologist. Operational treatment is shown in the development of complications: the suppuration of cyst or malignation. Taking into account the bilateral process, it must wear a hardware character. In a planned order, percutaneous puncture of the cyst can be performed, as well as the excision of their laparoscopic or open access. With severe chronic renal failure, hemodialysis and kidney transplantation are shown.

Solitary cyst kidney.Development vice is most favorable and is characterized by the formation of one or more cyst, localized in the cortical coil layer. This anomaly is equally common in the face of both sexes and is observed mainly after 40 years.

Solitary cysts can be simpleand dermoid.A solitary simple cyst may not only be congenitalbut also purchased.An innate simple cyst is developing from germinal collective tubules, which have lost contact with blades. The pathogenesis of its formation includes a violation of the drainage activity of the tubules with the subsequent development of the retention process and ischemia of the renal tissue. The inner layer of cysts is represented by a single-layer flat epithelium. Its content is more often serous, in 5% of hemorrhagic cases. Hemorrhage in the cyst is one of the signs of its ma-lignization.

Simple cyst usually happens single (solitary),although found multiple, multi-chamber,including bilateral cysts.Their value ranges from 2 cm in diameter to gigantic formations of more than 1 liter. The most often cysts are localized in one of the kidney poles.

Dermoid kidney cysts are extremely rare. They may contain fat, hair, teeth and bones that are detected with radiographic examination.

Simple cysts of small sizes are asymptomatic and are a random find during the examination. Clinical manifestations begin as

Fig. 5.22.Sonogram. Cysta (1) Kidney (2)

increasing the size of the cyst, and they are bound primarily with its complications, such as squeezing the cup-making system, ureter, kidney vessels, suppuration, hemorrhage and malignancy. There may be a rupture of a large kidney cyst.

Large solitary kidney cysts are palpable in the form of an elastic, smooth, rolling painless education. A characteristic sonorous sign of cysts is the presence of hypo echogenic homogeneous,

with clear contours, a rounded liquid medium in the cortical kidney zone (Fig. 5.22).

On excretory urograms, a multispiral CT with contrasting and MRI of the kidney is increased in size due to a rounded thin-walled homogeneous liquid formation, to one degree or another deforming a cup-glass system and causing the deviation of the ureter (Fig. 5.23). The Lohanka is composed, the cups are pushed out, spread out, when obstruction, the cervical cervix occurs a hydrocalix. These studies also make it possible to identify the kidney vessels anomalies and the presence of other diseases

(Fig. 5.24).

On the selective renal arteriogram at the location of the cyst, the weakly contrast insavidation shadow of the rounded formation is determined (Fig. 5.25). Static nephrocintigraphy reveals a rounded defect accumulation of the radiopharmaceutical.

Fig. 5.23.CT. Solitary cyst of the lower pole of the right kidney

Fig. 5.24.Multispiral CT with contrast. Multiple segmental type of renal arteries (1), cyst (2) and tumor (3) kidneys

Fig. 5.25.Selective renal arteriogram. Solitary cyst of the lower pole of the left kidney (arrow)

The differential diagnosis is carried out with multi-storey, polykis, hydronephrosis and, especially, kidney neoplasms.

Escaling testimony are the size of cysts more than 3 cm and the presence of its complications. The simplest method is the percutaneous puncture of cysts under ultrasonic guidance with the aspiration of its contents, which is subject to cytological examination. If necessary, perform a cystograph. After evacuating the contents into the cavity of the cyst, sclerosing substances (ethyl alcohol) are introduced. The method gives a high percentage of relapses, since cyst shells are preserved capable of producing liquid.

Currently, the main method of treatment is the laparoscopic or retroperitoneoscopic excision of cysts. Open operational intervention - lumbotomy - is rarely used (Fig. 66, see Col. Plot). It is shown when the cyst achieves huge sizes, has a multifocal nature with atrophy of the kidney parenchyma, as well as in the presence of its malignation. In such cases, reducing kidney or nephrectomy.

Parapelvikal cyst- This is a cyst located in the field of renal sinus, kidney gates. The wall of the cyst closely goes to the kidney and lochank vessels, but not reported with it. The cause of its formation is the underdevelopment of the lymphatic renal sinus vessels during the newborn period.

Clinical manifestations of parapelvicious cysts are due to its location, that is, pressure on the pelvis and the vascular leg of the kidney. Patients have pain. Hematuria and arterial hypertension may be observed.

Diagnostics are the same as with kidney solitarian cysts. Differential diagnosis is carried out with the expansion of the jelly with hydronephrosis, for which ultrasound and radiological methods are used with the contrasting of urinary tract.

The need for treatment occurs with a significant increase in cysts in the amount and development of complications. Technical difficulties in its excision are associated with the proximity of lochanks and renal vessels.

Diverticulous cups or lochanksit is a rounded solitary fluid education lined with them. It resembles a simple kidney cyst and was previously incorrectly called a cup or loamal cyan. The principal difference of the diverticulus from the solitar cyst is the combination of its narrow cage with a curiosity system, which characterizes this formation as a true diverticulous

Fig. 5.26.Multispiral CT with contrast. Diverticulous cup of left kidney (arrow)

schokes or loancakes. The diagnosis is established on the basis of an excretory urography and multispiral CT with contrast (Fig. 5.26).

In some cases, retrograde ureteropy-elography or percutaneous culography can be performed. Based on these methods, the message of the diverticulus with a cup of kidney is clearly installed.

Operational treatment is shown at large sizes of the diverticule and arising in connection with these complications. It lies in rebellion of the kidney with excision of the diverticulus.

Cashechnically medullary anomalies.Spongy kidney- very rare defective vague, characterized by the cystic expansion of distal

parts of collecting tubes. The defeat is predominantly bilateral, diffuse, but the process may be limited to part of the kidney. The spongy kidney is more common in boys and has a favorable course, practically without disturbing the kidney function.

The disease can be asymptomatic for a long period, sometimes pain in the lumbar region occur. Clinical manifestations are observed only when attaching complications (infection, micro- and macrohematuria, nephroalcinosis, stone formation). The functional state of the kidneys remains normal for a long time.

Diagnosis of spongy kidney by radiological methods. Overview and excretory urograms are often discovered by nephocalcinosis - the characteristic accumulation of calcinates and / or fixed small concreters in the region of renal pyramids, which, like the cast, emphasize their contour. In a cerebral substance, respectively, the pyramids revealed a large number of small cysts. Some of them appear in the lumen of the cups, reminding grape bunch.

Differential diagnosis should be carried out, first of all, with the tuberculosis of the kidneys.

Patients with uncomplicated spongy kidney do not need treatment. Operational treatment is shown in the development of complications: stone formation, hematuria.

Megakalix (megakolikosis)- congenital unstructive expansion of the cup, which is the result of medullary dysplasia. Expansion of all groups of cups is called halfcalix (Megapolikalikosis).

With megakalce, the kidney dimensions are normal, the surface is smooth. Cortical layer of ordinary sizes and buildings, brainstava is underdeveloped and thinned. Pacifics are flattened, differently poorly. Extended

Fig. 5.27.Excretory Urogram. Megacolikosis from the left

cups can move directly into a loyalty, which, in contrast to the case of hydronephrosis, retains normal dimensions. The pie-leafy segment is usually formed, the ureter is not narrowed. With uncomplicated flow, the kidney function is not broken. The expansion of the cups is due not to the obstruction of their necks, as it happens in this area of \u200b\u200bstone or framework syndrome (squeezing the neck of the cup by a segmental arterial barrel), and is congenitally unstructive.

Ultrasound, radiological methods with contrasting urinary tract are used to diagnose. On excretory urograms, the expansion of all groups of cups with the absence of a laughter's existence is determined (Fig. 5.27).

Megapolikalikosis, in contrast to hydronephrosis, in uncomplicated cases does not require operational correction.

5.2. Anomalies of uroakers

Development defects ureteralsmake up 22% of all the anomalies of the urinary system. In some cases, they are combined with kidney anomalies. As a rule, the anomalies of ureters lead to violation of urodamics. The following classification of ureteral defects are adopted.

■ Agenesia (Aplasia);

■ doubling (full and incomplete);

■ Morning.

■ Retrokat;

■ retrolyacal;

■ Extopia of the mouth of the ureter.

Anomalies of the form of uroakers

■ Spiral (ring-shaped) ureter.

■ hypoplasia;

■ Neuromuscular dysplasia (Ahalasia, Megaureter, Megajaureter);

■ congenital narrowing (stenosis) of the ureter;

■ Valve of the ureter;

■ diverticulum of the ureter;

■ ureterocele;

■ Bubble-ureteral-laughter reflux. Anomalies of the number of ureachers

Amediesia (Aplasia)- congenital absence of a ureter due to the underdevelopment of the ureteral sprout. In some cases, the ureter can be determined in the form of a fibrous chute or blindly ending the outflow (Fig. 5.28). One-sidedthe anesthesia of the ureter is combined with the Agnesia of the kidney from the side of the same name or multi-storey. Bilateralit is extremely rare and incompatible with life.

Diagnostics is based on x-ray methods of research with contrasting and nephrombintigraphy, allowing to identify the absence of one kidney. Characteristic cystoscopic signs are underdevelopment or absence of half of the urohy triangle and the mouth of the ureter with the appropriate side. With the stored distal ureteral department, its hole is also underdeveloped, although it is located in a normal place. In this case, confirm the blind ending the ureter allows retrograde ureterography.

Operational treatment is performed in the development of the purulent-inflammatory process and the formation of stones in the blindly ending upholster. Produce operational removal of the affected organ.

Doubling- The most frequent videos of the ureterals. Girls have this anomaly 5 times more often than that of boys.

As a rule, with caudal migration, the ureter of the lower half of the kidneys is first connected to the bladder and, therefore, it takes a higher and lateral position than the ureter of its upper half. Ureterals in the pelvic departments are mutually crossed and fall into the bladder in such a way that the mouth of the upper is located below and medially, and the lower - above and lancer (the law of Waireger-Maee-ra) (Fig. 5.29).

Doubling the upper urinary tract can be one-or two-sided, full-mounted (ureter duplex)and incomplete (Ureter Fissus)(Fig. 41, 42, see col. Plot). In case of full doubling each

Fig. 5.28.Aplasia left kidney. Blindly edged with ureter

Fig. 5.29.The law of Waireger Meyer. Crossing the ureters and the location of their mouths in the bladder with full doubling of urinary tract

the ureter opens with a separate mouth in the bladder. The incomplete doubling of the upper urinary tract is characterized by the presence of two pelvis and ureters connected in the pelvic department and open in the bladder with one mouth.

The described features of the topography of ureters when doubling the upper urinary tract are predisposing to the emergence of complications. Thus, the ureter of the lower half of the kidney, having a higher and laterally located mouth, has a short submissible tunnel, which is the cause of the high frequency of Pu-duct-ureteral-loching reflux in this ureter. On the contrary, the mouth of the ureter of the upper half of the kidneys is often ectopopy and predisposed to the wall-zing, which is the cause of the development of hydrophureteronephrosis.

Doubling the ureter in the absence of a violation of urodamics is not clinically manifested. It is possible to suspect this anomaly by sleep-graphics, at which the kidney doubling is determined, and the ureters in the presence of expansion can be visible in their railing or pelvic departments. The final diagnosis is established on the basis of an excretory urography, multispiral CT with contrasting, MRI and cystoscopy. In the absence of a function of one of the half of the kidney, the diagnosis can be confirmed by antitegrand or retrograde ureteropelography.

The tripling of lochs and ureters is a causistic.

Treatment operational in the development of complications. With a narrowing or ectopia of the ureter, the ureterocystoyastomosis is performed, and with bubble-ureter, reflux - antirefluxic operations. With the loss of the function of the entire kidney, nephreterectomy is shown (Fig. 60, see the collar. Intection), and one of its half-heminectretectomy.

Anomalies of the position of uretera

Retrokatar ureter- a rarely found anomaly in which the ureter in the lumbar department leaves under the hollow vein and, ring-shaped ending it, returns to its former position when switching to the pelvic department (Fig. 43, see col. Plotch). The compression of the ureter of the lower hollow veloy leads to a violation of the passage of urine with the development of hydrometeronephrosis and its characteristic clinical picture. To suspect this anomaly allows an ultrasound and excretory urbory, which reveals expanding the strip kidney and ureter system to its middle third, loop-shaped bending and the normal structure of the ureter in the pelvic department. The diagnosis is confirmed using multispirate CT and MRI.

Treatment operational, consists in the intersection, as a rule, with resection of the modified areas of the ureter and the implementation of a yurterourmetroistomostomosis with the location of the organ into its normal position to the right of the vein hollow.

Retroilical ureter- Extremely rarely encountered malformation, in which the ureter is located behind the ileum vessels (Fig. 43, see the collar. Plot). This anomaly, as well as a returder ureter, leads to its obstruction with the development of hydrometeronephrosis. Surgical treatment consists in the intersection of the ureter, release it from the vessels and the implementation of the antsevasal ureterooreteroanastomosis.

Extopia of the mouth of the ureter- anomaly characterized by atypical intrainyl intapulsive arrangement of the mouth of one or both ureters. This malforming vice is more often found in girls and, as a rule, is combined with the doubling of the ureter and / or ureterocele. The cause of this anomaly is the delay or violation of the separation of the ureter sprout from the Wolf of the duct during the embryogenesis period.

TO intravenousthe types of ectopia of the mouth of the ureter include shifting it down and media into the neck of the bladder. Such a change in the location of the mouth, as a rule, proceeds asymptomatic. The mouth of the ureters when they ombudityectopias are opened in the urethra, parauretral, in the uterus, vagina, the seed-moving duct, the seed bubble, the rectum.

The clinical picture of the outfilling ectopia of the ureter's mouth is determined by its localization and depends on the floor. In girls, this malformation is manifested by urinary incontinence with a stored normal urination. For boys with intrauterine development of Wolf, the duct turns into seed-ending duct and seed bubbles, so the ectoped mouth of the ureter is always located proximal than the sphincter of urethra and urinary incontinence does not occur.

The diagnosis is based on the results of a comprehensive examination, which includes excretory urography, CT, vaginography, urethro- and cystoscopy, catheterization of ectopied mouth and retrograde urethro and ure-terrography.

Treatment with this anomaly surgical and consists in transplanting an ecto-saw ureter in the bladder (ureterocystoyastomy), and in the absence of kidney function - nephrimeterectomy or hemine-ereterectomy.

Anomalies of the form of uroakers

Spiral (ring-shaped) ureter- Extremely rare malformation, in which the ureter in the middle third has the shape of a spiral or rings. The process can wear one-and bilateralcharacter. This anomaly is a consequence of the inability of the ureter to rotate together with the kidney during its intrauterine movement from the pelvic to the lumbar region.

The twisting of the ureter leads to the development of obstructive-retention processes in the kidney, the development of hydronephrosis and chronic pyelonephritis. Install the diagnosis helps an excretory urography, multispiral CT, MRI, and, if necessary, retrograde or antitegrand percutaneous ureterography.

Surgical treatment. Perform resection of the ureter with ureterourie-roaanastomyoma or ureterocystoyastomy.

Anomalies of the structure of ureterals

Gapopiathe ureter is usually combined with the hypoplasia of the corresponding kidney or its half at doubling, as well as with multiple kidney. The intelligence of the ureter with this anomaly is dramatically narrowed or bored, the wall is thinned, the peristalsis is weakened, the mouth is reduced in size. Diagnosis is based on cystoscopy data, excretory urography and retrograde ureerography.

Neuromuscular dysplasiathe ureter was described as the "Mega-Urtyer" J. Goulk in 1923 as an innate disease, manifested by the expansion and elongation of the ureter (by analogy with the term "Megalon"). This is one of the frequent and severe defects of the development of ureters caused by underdevelopment or the complete absence of its muscular layer and innervation disruption. As a result, the ureter is not capable of active abbreviations and loses its function of moving urine from a locher to the bladder. Over time, this kind of dynamic obstruction leads to even greater expansion and elongation with the formation of crankshafts (Me-Gadolihojaureter). The worsening of the urine transport contributes to the normal tone of the drier of the bladder and the combination of this anomaly with other defects of the development (ectopia of the ureter's mouth, ureterocele, bubble-ureter-laughter reflux, neurogenic urinary bubble dysfunction). Frequent addition of infection against the background of Wrestz contributes to the development of chronic uretheritis, followed by the scarring of the ureter wall and even greater decrease in the function of the upper urinary tract. The characteristic histological signs of the megaureter are a significant underdevelopment of the neuromuscular structures of the ureter with a predominance of scar tissue.

Ahalasiathe ureter is the neuromuscane dysplasia of his pelvic department. The underdevelopment of the ureter under this anomaly is local in nature and does not affect its overlying departments, where they are not much changed or developed normally. From these positions, Ahalazia of the ureter should be considered not to be the stage of the development of the megaureter, but one of his species. As a rule, the expansion of the ureter in its pelvic department under ahalasia is preserved at the same level during the life. In some cases, the overlying ureteral departments can be involved

Fig. 5.30.Excretory Urogram. Ahalasia left ureter

the pathological process is secondary as a result of stagnation of urine in an extended cystoid, that is, dynamic obstruction.

The clinical picture of the neuro-nose dysplasia of the ureter depends on the degree of its severity. With one-sided Ahalasia or megarette, the general condition remains satisfactory for a long time. Symptoms are weakly pronounced or absent, which is one of the reasons for the late diagnosis of neuromuscular dysplasia already in adulthood. The first signs of the megaureter are due to the addition of chronic pyelonephritis. Pains appear in the appropriate lumbar region, an increase in temperature with chills, dizuriy. A severe clinical course is observed in a bilateral megarether. From an early age, symptoms of chronic renal failure

pOSSIBILITY: The child's backlog in physical development, reduced appetite, polyuria, thirst, weakness, fatigue.

The diagnosis of neuromuscular dysplasia is based on laboratory, radial, urodynamic and endoscopic research methods. Sono-graphic allows you to identify the expansion of a cup-lowering system and a ureter in its climbing and pre-examination departments, a decrease in the renal parenchyma layer. A characteristic feature of Ahalasia on excretory upright is a significant expansion of the pelvic ureter's pelvic department with unchanged overlying urinary tract departments (Fig. 5.30).

In the megarette, there is an increase in length and a significant expansion of the ureter throughout the sections of the knee-forming beggars. An-test pelleurography allows you to determine the diagnosis in the absence of a kidney function according to the excretory urography.

In the differential diagnosis, the megaureter should be distinguished from the thermionephrosis hydroreonphrosis, which arises as a result of the narrowing of the ureter.

Operational treatment of neuromuscular dysplasia of the ureter largely depends on the stage of the disease. Over 100 methods of operational correction are proposed. The degree of compensatory opportunities, especially characteristic of young children, depends on the severity of the anatomical and functional disorders, the diameter of the ureter and the activity of the pie-loneface process. Surgical treatment consists in resection of an extended ureter in length and a width with submucose implantation

its in the bladder on the political and lidbetter. More pronounced changes in the wall of the ureter with a significant impaired of its function are an indication of the intestinal plastic of the ureter (Fig. 54, 55, see Col. Plot).

Congenital narrowing (stenosis) of the ureter,as a rule, it is localized in its climax, less often - preposterous departments, resulting in hydronephrosis or hydrometeronephrosis. Due to the frequency, features of etiology, pathogenesis, clinical course and methods of operational correction, hydronephrotic transformation is isolated in a separate nosological form and is considered in chapter 6.

Valves of the ureter- These are local duplicators of the mucous membacity and submuble or less than all the layers of the wall of the ureter. This anomaly is extremely rare. The cause of its formation is congenital excess mucous membrane. Valves may have an oblique, longitudinal, transverse direction and are more often localized in the climbing or preposter diversion of the ureter. They may cause ureteral obstruction with the development of hydronephortic transformation, which is an indication for surgical treatment - resection of a narrowed ureter area with anastomaosis between the unchanged urinary tract departments.

The diverticulus of the ureter is a rare anomaly, which manifests itself in the form of a bag-shaped protrusion of its wall. Most often there are diverticulus of the right ureter with predominant localization in the pelvic department. Also described bilateral diverticulus of the ureter. The wall of the diverticulus consists of the same layers as the ureter himself. The diagnosis is established on the basis of an excretory urography, retrograde ureerography, spiral CT and MRI. Operational treatment is shown in the development of hydrometeronephrosis as a result of the obstruction of the ureter in the field of the diverticula. It lies in resection of the diverticule and the walls of the ureter with a yereterourmetterastomosis.

Ureterocele- a cystic expansion of the intramural department of the ureter with a mixture of it into the lumen of the bladder (Fig. 15, see col. Plot). It relates to frequent anomalies and is diagnosed in 1-2% of patients of all age groups subjected to cystoscopic examination.

Ureterocele can be one-and bilateral.The cause of its formation is the congenital neuromuscane underdevelopment of the submembratus layer of the intramural department of the ureter in combination with the narrowness of its mouth. Due to such a defective, the mucous membrane of the ureter of the ureter in the cavity of the bladder is gradually gradually occurs, with the formation of a rounded or pear-shaped cyst formation of various sizes. The outer wall is the mucous membrane of the bladder, and the inner - ureter mucosa. At the top of the ureterocela is the narrowed mouth of the ureter.

Distinguish two types of this anomaly of ureters - ortotopicand guitet-rotopic (ectopic)ureterocele. The first arises at the normal location of the mouth of the ureter. It is small sizes, well reduced and, as a rule, does not disturb the outflow of urine from the kidneys. Such a small-axipput of ureterocele is more often diagnosed in adults. The heterotopic ureterocele occurs at low ectopia of the mouth of the ureter towards the output from

bladder. In early age children in 80-90% of observations, the ectopic species of ureterocele is diagnosed, more often than the lower mouth, when doubling the ureter. One-sided form prevails, the disease is revealed from both sides.

Ureterocele causes violation of the passage of urine, which gradually leads to the development of hydrophureteronephrosis. Frequent complication of the ureterocele is the formation of stone in it.

Clinical symptoms depends on the size and localization of the ureterocea-le. The more ureterocele and pronounced the obstruction of the ureter, the sooner the symptoms of this anomaly appear more clearly. Pains appear in the appropriate lumbar region, when it is formed by stone and accession of infection in it - dysuria.At large sizes of the ureterocele, difficult urination may be observed due to the obstruction of the tin bubble cervix. In women, there is a fallout of ureterocele beyond the urethra.

The main place in the diagnosis is given to the radiation methods of research and cystoscopy. A characteristic feature for sonography is a rounded form of a hypo echogenic formation in the area of \u200b\u200bthe bladder neck, above which an extended ureter can be determined (Fig. 5.31, 5.32).

On excretory urograms, CT with contrast and MRI is visualized by the ureterocele and varying degrees of severity of hydrophureteronephrosis (Fig. 5.33).

Cistoscopy is the main method of diagnosing the ureterocele (Fig. 15, see col. Plot). With its help, it is safe to confirm the diagnosis of this anomaly, to establish the kind of ureterocele, its size, side of the damage. Ure-Terocele is defined as a rounded formation, located in the triangle of the bladder, on the top of which the mouth of the ureter opens, during the separation of urcheterocele, is reduced and decreases in size (falling).

Ortotopic ureterocene of small sizes without a disorder of urodynamics of treatment does not require. The type of operational intervention is determined by the size and localization of the ureterocele, as well as degrees

Fig. 5.31.Transabdominal sonogram. Ureterocele on the left (arrow)

Fig. 5.32.Transrectal Sonogram. Large sizes of ureterocele (1) with a significant expansion of the ureter (2)

Fig. 5.33.Excretory Urograms on the 7th (A) and 15th (b) minutes of research. Ureterocele (1) on the right with the expansion of the ureter (2) (hydrometeronephrosis)

hydronephortic transformation. Depending on this, a transuretral endoscopic resection of the ureterocele or its open resection with the implementation of the ureterocystoyastomosis is applied.

Bubble-ureteral-laughter Reflux (PMR)- The process of retrograde cast of urine from the bladder into the upper urinary tract. It is the most common pathology of the urinary system in children and is divided into primaryand secondary.Primary PMR occurs as a result of congenital insolvency (unfinished ripening) of bubble-ureteral coordinates. Secondary - is a complication of infrae-sliced \u200b\u200bobstruction, developing due to increased pressure in the bladder.

PMR may be activeand passive.In the first case, it occurs at the time of urination at the maximum increase in intravenous pressure, in the second - may be observed alone.

The characteristic clinical manifestation of the PMR is the occurrence of pain in the lumbar region when urine. When attaching an infection, symptoms of chronic pyelonephritis appear.

In the diagnosis of PMR, radiopuclide research methods occupy a leading role. Retrograde cystography alone and at urination (microcker cystography) reveals not only its presence, but also the degree of expression of anomaly (see ch. 4, Fig. 4.32).

Conservative treatment is possible in the initial stages of the disease, the surgical is to perform various antireflury operations

from which the easiest is the easiest of endoscopic submembrance in the area of \u200b\u200bthe formation of forming bioimplants (silicone, collagen, teflon paste, etc.) that prevent the reverse current of urine. Widespread applications found operations for the reconstruction of the ureter's mouth, which are currently being implemented including using robot-grade technology.

5.3. Anomalies of the bladder

The following vices of the bladder development are distinguished:

■ urinary duct anomalies (loan);

■ bladder anesthesia;

■ doubling the bladder;

■ congenital urinary bubble diverticulum;

■ Extrophy of the bladder;

■ Congenital belief cervical contracture.

Uhrahus.(Urachus)- Urinary duct, which connects the forming bladder through the umbilical umbilical umbilical water during the period of intrauterine development of the fetus. Typically, by the time of birth, he grabs. With defects, the urehus can completely or partially overgrow. Depending on this, the anomalies of the loan will distinguish.

Umbilical fistula- the incoming of a part of the loan, opening with a fistula in the navel area and not reported with the bladder. Permanent allocations from the fistula lead to skin irritation around it and the attachment of infection.

Bubble-pouche- Full incoming ureraus. In this case, there is a constant separation of urine from the fistula.

Cyst Urerahus- the unguardment of the middle part of the blade. Such anomaly proceeds asymptomatic and manifests itself only at large sizes or suppuration. In some cases, it can be adopted through the front abdominal wall.

The diagnosis of urachus anomalies is based on the use of ultrasound, radiographic (fistulography) and endoscopic (cystoscopy with the introduction of methylene blue methylene and detection in the urine) of the research methods. Operational treatment consists in excision of the loan.

Agnesia of the bladder- His congenital absence. An extremely rare anomaly, which is usually combined with defects, are not compatible with life.

Urinary bubble doubling- Also very rare anomaly of this organ. It is characterized by the presence of a partition that separates the urinary bubble cavity into two halves. Each of them opens the mouth of the appropriate ureter. This anomaly may be accompanied by doubling the urethra and the presence of two sheeps of the bladder. Sometimes the partition can be incomplete, and then there is a "two-chamber" bladder (Fig. 5.34).

Congenital urinary bubble diverticulum- Basch-like protrusion of the bladder wall outside. As a rule, it is located on the posterior wall of the bladder next to the mouth, somewhat higher and the lateral of it.

Fig. 5.34.Urinary bubble doubling: but- full; b.- incomplete

The wall of the congenital (true) diverticulus, unlike the acquired, has the same structure as the wall of the bladder. The acquired (false) diverticulus is developing due to the infrae-dispersion and increase pressure in the bladder. As a result of extracting the walls of the bladder, its thinning with the protrusion of the mucosa between the bunches of hypertrophied muscle fibers occurs. Permanent stagnation of urine in the diverticulus contributes to the formation of stones in it and the development of chronic inflammation.

The characteristic clinical symptoms of this anomaly are the difficulty of urinary and emptying the bladder in two stages (first empty the bladder, then the diverticulum).

The diagnosis is established on the basis of an ultrasound (Fig. 5.35), cystography (Fig. 5.36) and cystoscopy (Fig. 20, see col. Plot).

Treatment operational, consists in excision of the diverticulus and ears the formed bladder wall defect.

Extrophy of the bladder- severe malformation, consisting in the absence of the front wall of the bladder and the part of the front abdominal wall corresponding to it (Fig. 40, see Col. Plotch). This anomaly is more often observed in boys and occurs in 1 of 30-50 thousand newborns. Extrafia of the bladder is often combined with the defects of the development of the upper and lower urinary tract, deposit

Fig. 5.35.Transabdominal sonogram. Diverticulus (1) bladder (2)

Fig. 5.36.Downward cystogram. Urinary bubble diverticulus

the rectum, the boys, with the Epispadia, groin hernia, Crip-Torkhism, in girls - with anomalies of the development of the uterus and vagina.

Watering with such an anomaly is constantly poured outward, which further leads to maceration and the ulceration of the skin of the perineum, genitals and hips. When the child is satisfied (with a laugh, cry, crying), the bladder wall is pulled in the form of a bowl, and the selection of urine is enhanced. The mucous membrane is hyperemic, easy to bleed. In the lower corners of the defect are determined by the mouth of the ureters. Extrophy of the bladder, as a rule, is combined with the diastasses of the bones of the Lonatic joint, which is manifested by the "duck" by the gait. Permanent contact of the mucous membrane of the bladder and urethra with an external environment contributes to the development of chronic cystitis and pyelonephritis.

Surgical treatment is carried out in the first months of the child's life. There are three types of surgical interventions:

■ Reconstructive plastic operations aimed at the closure of the bladder defect and the abdominal wall with their own tissues;

■ Transplanting the bladder triangle together with the mouths in the sigmoid gut (currently being extremely rare);

■ Formation of an artificial orthotopic urinary reservoir from the site of the ileum.

Bladder cervical contracture- Development vice, characterized by excessive development of the connective tissue in this anatomical area. The clinical picture depends on the degree of severity of fibrous changes in the neck of the bladder and the associated violations of urination. The diagnosis of this anomaly is based on the results of the instrumental study (wrophlorometry in combination with cystomanometry), urethrography and urethrocystoscopy with the bubble cervical biopsy. The treatment is endoscopic, lies in the dissection or excision of scar tissues.

5.4. Anomalies of the urethra

The vices of the development of the urethra include:

■ hypospadia;

■ Epispeadia;

■ congenital valves, obliteration, strictures, diverticulus and urethra cysts;

■ hypertrophy of seed tuberca;

■ doubling the urethra;

■ urethro-recycled fistula;

■ Loss of the urethra mucosa.

Hypospadia- An innate absence of a plot of the forefront of the urethra with a substitution of the missing part with a dense connective tissue (chord) and the sparking of the penis back, towards the scrotum. This anomaly occurs with a frequency of 1: 250-300 newborns. In fact, hypospadia is combined with an abnormal structure of the penis. It, as a rule, anatomically underdeveloped, small, thin, is strongly curved in the dorsal direction. The bending is especially expressed when erection. The angle of curvature can be so large that sexual life becomes impossible. Usually the extreme flesh is split and covers the head in the form of a hood. May take place meatostenosis.

Highlight golovatu(most frequent), crown, stem, scrotaland cineryhypospadia. The first two forms are lighter and differ little from each other. They are characterized by the location of the outer opening of the urethra at the level of the head or the cornese groove and the insignificant sparking of the penis.

The trunk form is characterized by the location of the outer opening of the urethra in different parts of the penis. The more proximal it is ectopied, the more pronounced body curvature. At the expense of bending and meatostenosis, the emptying of the bladder is difficult, the jet is weak, directed the book.

The most severe is the scrotal and crotch forms of the hypo-sudden. They are characterized by a sharp underdevelopment and curvature of the penis and a pronounced urination disorder that is possible only in the sitting position. Newborn with scrotal hypospadhery is sometimes mistaken for girls or false hermaphrodites.

A separate form is the so-called "hypospadia without hypospadia", in which the outer opening of the urethra is in a normal place on the head of the penis, but it itself is significantly shortened. Between the shortened urethra and normal length, a tight connecting point (chord) is located, which makes the penis sharply curved in the dorsal direction.

The feminine hypospadia is extremely rare, at which the rear wall of the urethra and the front wall of the vagina is split. She may be accompanied by stress incontinence of urine.

The diagnosis of hypospadias is set with an objective study. In some cases, it is difficult to distinguish the sconational and crotch hypospadia from the female false hermaphroditism. In such cases, it is necessary to determine the genetic floor of the child. Radi methods allow you to identify the presence and type of structure of the internal genital organs.

Operational treatment is shown in all forms of this anomaly and is performed in the first years of the child's life. When dodged and crowned hypospadia

the operation is carried out with a significant curvature of the head of the penis and / or meatostenosis. For the correction of heavier forms of hypospadia, many different methods of operational treatment are proposed. All of them are aimed at achieving two main objectives: the creation of the missing part of the urethra with the formation of its outer opening in the normal anatomical position and straightening the penis by excision of connecting scars (chords). The forecast for a timely made plastic operation is favorable. A good cosmetic effect is achieved, normal urination, preservation of sexual and reproductive function.

Epispadia- Congenital splitting on the front surface of the entire or part of the urethra. Open Kepened This section of urethra, together with the cavernous bodies, create a characteristic groove passing along the dorsal part of the penis. This anomaly is much less common than hypospadia, and on average is detected in 1 of 50 thousand newborns. The ratio between boys and girls is 3: 1.

The boys distinguish three types of Eppispect: granny, stemand total. Epispeadia Head Headit is characterized by the fact that the front wall of the urethra is split to the Crown Groove. Sexual dick is slightly twisted and raised up. Urination and erection with this form of Eppispect are usually not violated.

Trunk formit is characterized by the fact that the front wall of the urethra is cleaved throughout the penis - to the field of skin transition to the pubic area. With this form of the epispade, the splitting of the pubic symphiz is observed, and sometimes the discrepancy between the abdominal muscles. The sexual member is shortened and bent in the direction of the front abdominal wall. A hole of the urethra has a funnel shape. When urination, the jet is directed upward, urine is sprayed, which leads to the wetting of clothes. Sex life is impossible, since the sexual member of small sizes and during the erection is strongly twisted.

Total (full) Epispadiain addition to splitting the front wall, the urethra is characterized by splitting the sphincter of the bladder. The urethra has a kind of funnel and is located immediately under Lona. This form is characterized by incontinence of urine due to underdevelopment of the bladder sphincter. The constant leakage of urine leads to skin irritation in the field of scrotum and crotch, dermatitis develops, violates the normal social adaptation of the child in the Society of peers. The underdevelopment of the penis and the scrotum is noted.

Epispadia of girls is less common than the boys. It is distinguished by three of its forms. Clicor Epispadia,it is characterized only by cleavage of the clitoris. The outer hole of the urethra is shifted up and opens above it. Urination is not violated.

For subsymphizar formthere is a splitting of urethra to the bladder neck and cleavage of the clitoris. The most severe is full epis-vulat which there are no front wall of the urethra and the bladder neck, and the outer hole of the urethra is located behind the pubic symphysis. There is a splitting of the LONA joint and the sphincter of the bladder, which is manifested by duck and urinary incontinence.

In the majority of patients with epispadia, the capacity of the bladder is reduced, the PMR is observed.

Operational treatment of Epispeadia is carried out in the first years of life. It lies in the reconstruction of the urethra and eliminate the curvature of the penis.

Congenital valves of urethra- The presence in its proximal department of pronounced folds of the mucosa protruding into the clearance of the urethra canal in the form of jumpers. This anomaly is more common in boys and is 1 to 50 thousand newborns. Urethra valves violate normal urination, make it difficult to emptying the bladder, lead to the appearance of residual urine, the development of hydrophureteronephrosis and chronic pyelonephritis. Treatment of valves of urethra endoscopic. The tour is performed.

Congenital obliteration of the urethrait is extremely rare, always combined with other anomalies, often not compatible with life.

Congenital stricture of urethra- A rare anomaly at which there is a rub-tissue narrowing of its lumen, leading to urination disorders.

Congenital diverticulus urethra- Also a rare malformation, which consists in the presence of bag-shaped protrusion of the rear wall of the urethra. More often is localized in the front of the urethra. Manifested by dysuria and separation of urine droplets after the end of the urination act. The diagnosis is established on the basis of urethrography and urethroscopy, mixture cysturometerography. The treatment is in the excision of the diverticulus.

Congenital cysts of urethradevelop as a result of obliteration output holes of the bulburetral glands. Mostly localized in the field of the bulb of the urethra. They are removed by the operational way.

Hypereed hypertrophy- Congenital hyperplasia of all elements of the seed tubercle. It causes urethra obstruction and the appearance of erection during urination. Diagnosed by urethroscopy and retrograde urethrogradi. Treatment is the tour of the hypertrophied part of the seed tubercle.

Doubling Urethra- Rare malformation. It happens complete and incomplete. Full doublingcombined with doubling penis. More often occur incomplete doubling of urethra.In most cases, an additional urethra ends blindly. Additional urethra always has a underdeveloped cavernous body.

Urethro-straightforward fistula- A rare malformation, which is almost always combined with the atresia of the rear pass. Arises as a result of underdevelopment of the urinary block partition.

Loss of the mucosa of the urethra- also rare anomaly. The fallen mucosa due to the microcirculation disorders has a blue shade, sometimes bleeding. Treatment operational.

5.5. Anomalies of men's genital organs

Anomalies of the testicles

Development defects are divided into anomalies of quantity, structures and positions. For anomalies of quantities include:

Anorhis- Congenital absence of both testicles. Combined with the underdevelopment of other genital organs. Differential diagnosis with bilateral abdominal cryptorchism is carried out on the basis of scintigraphy of eggs, CT, MRI and laparoscopy. Treatment is to appoint substantive hormone therapy.

MONORKHISM- Congenital absence of one egg, its appendage and seed-wearing duct. It should be distinguished from one-sided abdominal cryp-torchism using similar diagnostic methods, as well as in the anorrhism. With a cosmetic goal, the yachi prosthetics is possible.

Poliormalism- Extremely rare anomaly characterized by the presence of an additional egg. It is located next to the main, usually underdeveloped and, as a rule, does not have an appendage and a seed-winning duct. Due to the high risk of malignancy, it is advisable to remove it.

Synornchism- An innate battle of both eggs from the abdominal cavity. Treatment operational. It is separated and reduced to the scrotum.

To the anomalies of the structure belongs egg hypoplasia- His congenital underdevelopment. Diagnosed with objective inspection (sharply reduced testicles are palpable in the scrotum), using radiation and radionuclide research methods. In the treatment, especially with a bilateral process, use replacement hormone therapy.

To the anomalies of the state of the testicles include:

Cryptorchism- Development Pulk (from Greek. Kriptos - Hidden and Orchis - Egg), in which there is no uncomplicit in the scrotum of one or both testicles. Hour-

totes of cryptorchism in the ended newborn boys are 3%, and the premature increased 10 times. Cryptorchism in 25-30% of cases is combined with the anomalies of other organs.

The abnormal position of the egg leads to its anatomy-functional insufficiency up to atrophy. The cause of the most significant complication of cryptorchism is infertility - is the change in the temperature mode of the testicle. Sperm-naya function is significantly violated even with a slight increase in its temperature. In addition, the risk of ma-lignization of an uncomplicated egg, unlike normally located significantly increases.

Depending on the degree of indifference distinguish abdominaland pakhovoyforms of cryptorchism

Fig. 5.37.Forms of cryptorchism and ectopias of eggs:

1 - normally located testicle; 2 - Egg delay before entering the scrotum; 3 - gear ectopia; 4 - groove Cryp-Torchism; 5 - abdominal cryptorchism; 6 - femoral ectopia

Fig. 5.38.CT small pelvis. Abdominal cryptorchism (1). The left testicle is located in the abdominal cavity near the bladder (2)

(Fig. 5.37). This malformation may be one-sidedand bilateral, trueand false.False (pseudocrprintorchism) is noted under excessive mobility of the testicle when it is as a result of a reduction in the muscle raising the testicle (T. Cremaster),tightly pulls up to the outer inguinal ring or even immersed in the groin canal. In a relaxed state, it can be neat movements to reject in the scrotum, but it often returns back.

The diagnosis is established on the basis of data from physical research, sonography, CT (Fig. 5.38),

scintigraphy eggs and laparoscopy. Based on the same methods, cryptor-hism differentiates from the anorrhism, monorchism and egg ectopia.

Conservative treatment is shown at the location of the egg in the distal portion of the inkhan canal. Use hormonal therapy with chorionic go-noodotropin. Operational treatment is carried out in the first years of the child's life in the ineffectiveness of hormone therapy. It lies in the opening of the inguinal channel, mobilization of eggs, seed rope and reduce it into the scrotum with fixation in this position (orchipexia).

Ectopia Yachka- Congenital malformation, in which it is located in various anatomical areas, but not in the course of its embryonic route in the scrotum. This abnormality is different from cryptorchism. Depending on the localization of the eggs distinguish inguinal, femoral, perinealand crossectopia (see Fig. 5.37). Treatment operational - reduction of eggs to the appropriate half of the scrotum.

Forecast for the development of testicles in cryptorchism and ectopia is favorable if the operation is performed in the first years of the child's life.

Sexual abnormalities

Congenital phyms- Congenital narrowing of the opening of extreme flesh, not allowing to expose the head of the penis. Up to 3 years in boys in most cases, physiological phimosis is recorded. In the case of a pronounced narrowing of extreme flesh, it is resorted to its circular excision (Circumcision).

Hidden penis- Extremely rare anomaly, in which normally developed cavernous bodies are hidden by the surrounding tissues of the scrotum and the leather of the pubic area. The penis, as a rule, is reduced in size, cavernous bodies are determined only when palpation in the folds of the surrounding skin.

Ectopia penis- Extremely rare anomaly at which it has small sizes and is located behind the scrotum. Treatment Operational: Moving a penis in a normal position.

Updated penis (diphallia)- Also a rare malformation. Doubling can be fullwhen there are two penisters with two urethra channels, and incomplete- Two genital member with a urethral chute on the surface of each. Operational treatment is to remove one of the less developed sexual members.

Casuistry is agnesia of the peniswhich, as a rule, is combined with other anomalies that are not compatible with life.

Control questions

1. Give the classification of kidney anomalies.

2. What is the difference between the multi-kidney polycistosis?

3. What should be therapeutic tactic with a simple kidney cyst?

4. What are the types of ectopia of the mouth of the ureter?

5. What is the essence of the Waireger Lawer Act?

6. What is the clinical value of the Uneterocele?

7. What are the Irachus anomalies?

8. List the types of hypospadias.

9. Give the forms of cryptorchism and ectopia of eggs.

10. What peralia of the penis are you known? What is congenital phimosis?

Clinical task 1.

The patient, 50 years old, turned with complaints on periodically emerging stupid pains in the left flank of the abdomen and the lumbar region. In recent months, in the left hypochondrium, he independently adopted the elastic, rounded, easily biased education. In this case, increasing body temperature, urination disorders, urine color changes and other symptoms did not notice. He turned to the family doctor. The laboratory tests assigned to them turned out to be normal, after which a multispiral CT of the abdominal cavity was performed (Fig. 5.39).

What are you diagnosed? Are other research methods need to confirm it? What method of treatment to choose?

Clinical task 2.

The patient, 25 years old, turned with complaints of stupid pain in the lumbar region on the left, rapid painful urination. Such phenomena notes for several months. Palparent kidneys are not defined. Blood and urine tests are not changed. Uzi revealed rounded small sizes

Fig. 5.39.Multispical kt kidney with patient contrast 50 years

Fig. 5.40.An excretory urogram of the patient 25 years. Left ureter ends with a male-shaped expansion

hypo echogenic formation in the neck of the bladder neck. The patient has an excretory urogram (Fig. 5.40).

Give the interpretation of an excretory urogram. What is the diagnosis? What tactics should you choose?

Clinical task 3.

The parents of the boy at the age of 9 months turned to the urologist with a complaint about the lack of a child's left egg in the scrotum. From their words, the boy was born premature and the testicle was absent from the moment of birth. With objective inspection, it was revealed that the outer hole of the urethra is typically located, the folding of the scrotum is saved. The right testicle is determined in its usual place, the left is palpable in the center of the inguinal canal.

11. Anomalies of the urinary system

The anomalies of the urinary system are the most common defects of development. Some can lead to early death in childhood, others do not cause violations of the functions of the urinary system and are randomly found in ultrasound examination or tomography, as well as X-ray examination.

Part of the anomalies is very slowly progressing and clinically can manifest itself only in old age. Some developmental abnormalities can serve as a background for the development of another pathology (renal disease, chronic pyelonephritis, arterial hypertension).

The causes of the emergence of anomalies may be hereditary predisposition, the disease of the mother during pregnancy - rubella in the first months; Ionizing radiation, syphilis, alcoholism, consumption of hormonal drugs.

If one family member was found an anomaly, then you need to examine all family members.

Classification

The following groups of anomalies distinguish:

1) anomalies of the number of kidneys - bilateral Agenesia (lack of kidney), one-sided Agenesia (only kidney), kidney doubling;

2) kidney position anomalies - muscotral dystopia (lowered kidney is on its side); heterolateral cross dystopia (moving the kidneys on the opposite direction);

3) anomalies of kidney interconnections (fascinated kidneys), horseshoe kidney, galetooth, S-shaped, L-shaped;

4) anomalies of the values \u200b\u200band structure of kidneys - aplasia, hypoplasia, polycystic kidney;

5) Anomalies of the renal pelvis and ureters - cysts, diverticulus, splitting of the pellets, anomalies of the number, caliber, forms, the positions of ureters.

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Introduction

The greatest number of complications occurs with the mechanical obstacle of the urine outflow; Urine is stirred or returned (throws) to the upper departments of the urinary system. Fabrics in the field of mechanical obstacle swell and the tissues are damaged as a result. The most serious damage to the renal tissue, resulting in disruption of kidney functions.

Obstruction (mechanical obstacle) is a relatively rare pathology. Boys are sick more often. In girls, obstruction occurs more often in the place of branch of the urethra from the kidney or between the urethra and the wall of the bladder; Boys - between the bladder and the urethra. The obstruction in the upper urinary system sections is more common to the right.

Among complications of obstruction are the most common infectious processes and the formation of renal stones. In order to avoid their development, surgical intervention is shown. Operational interventions are more successful in infancy. In the absence of an operation for up to 2-3 years, the kidney defeat and the violation of their function is inevitable.

Another type of development anomaly is the absence or doubling of some urinary system organs, their improper location, the presence of unnecessary holes. In second place among the anomalies there is an extrefia (defect of the bladder, anterior abdominal wall, umbelical ligaments, pubic area, genitals or intestines) and an epispade (defect of the penis and urethra).

Most children who develop the blockage of the urinary system are born with the structural anomalies of the urinary tract; It is possible to excess the tissue in the urethra or the presence of pocket in the bladder, the inability of any part of the urethra to promote urine to the bladder. Damage to the urethra in injury to the small pelvis organs is possible.

The urinary tract is laid in the early stages of the formation of the fetus (the first 6 weeks after fertilization); The resulting defects may arise due to a number of reasons acting on the fetus, although the exact mechanism of their occurrence remains unclear. It has been established that the percentage of defects is higher in children born from mothers who abused drugs or alcohol.

The formation of renal stones in the blockage of the urinary system may be due to a number of reasons, however, formed, renal stones, in turn, increase the obstacle to the outflow of urine.

Quality anomalies

gOOD AGENESIA KIDK DISTOPIC

Anomalies of the urinary system are called violations in the development of the organs of the urogenital system, which do not allow the body to function normally.

It is believed that the most frequent anomalies of the urinary system arise due to the influence of hereditary factors and various negative impacts on the fetus during the period of intrauterine development. Anomalies of the urinary system can develop in a child due to the transferred by the mother in the first months of pregnancy rubella, syphilis. Alcoholism can provoke an emergence of anomaly, and the drug addiction, the use of hormonal contraceptives during pregnancy, as well as drugs without appointing a doctor.

The anomalies of the urinary system are divided into the following groups:

Ø Anomalies of the number of kidneys - bilateral Agenesia (absence of kidney), one-sided Agnesia (only kidney), doubling kidney;

Ø Kidney position anomalies - muscotral dystopia (lowered kidney is on its side); heterolateral cross dystopia (moving the kidneys on the opposite direction);

Ø Kidney interomalies (fucked kidneys), horseshoe kidney, galetooth, s-shaped, L-shaped;

Ø Anomalies of the magnitude and structure of kidneys - aplasia, hypoplasia, polycystic kidney;

Ø Anomalies of the renal pelvis and ureters - cysts, diverticulus, splitting of the pelvis, anomalies of the number, caliber, forms, the positions of the ureters.

Many of these anomalies are the cause of the development of renal disease, inflammatory process (pyelonephritis), arterial hypertension.

The influence of various forms of the anomalies of the urinary system on the child's body can be expressed in different ways. If some violations most often lead to the intrauterine death of the baby or his death in infancy, then a number of abnormalities have no significant impact on the work of the body, and often detects only by chance during routine medical examinations.

Sometimes an abnormal abnormal can cause serious functional disorders in adulthood or even old age.

It is believed that the risk of developing such violations in the first months of pregnancy is most high, when the main organs are laid, including the urinary system. The future mother is not necessary to make any medicine without appointing a doctor. In the event of colds and other diseases, under which there is a high temperature and intoxication, you should immediately contact the hospital.

When planning pregnancy, young parents are encouraged to undergo a medical examination, which will eliminate their various diseases leading to anomalies from the fetus. If the family has already been cases of anomalies, genetics is needed.

Extrophia Cloaca

Extrophy (coupling contamination of the hollow organ) Cloaca is a defect in the development of the lower front of the front abdominal wall. (Cloaca is a part of a germs, which ultimately develops the abdominal cavity bodies.) A child with an extrefia of a cloaca is born with multiple defects of internal organs. A portion of the colon is located on the outer surface of the body, on the other hand there are two halves of the bladder. The boys have a penal member short and flat, the clit cleaves the girls. Cases of such a coarse anomaly are found: 1 for 200,000 living newborns.

Despite the severity of the defect at Extrophy Cloaca, newborns are viable. The bladder can be restored surgically. The lower sector of the colon and the straight intestine are underdeveloped, so a small routing receiver is created by a surgically.

Extrophy of the bladder

Extrophy of the bladder is a congenital anomaly of the urinary system, characterized by turning the bladder outward from the abdominal wall. Such pathology is found in 1 of 25,000 children, for boys 2 times more often than girls.

In all cases, the Extrophy of the bladder is combined with an anomaly of external genital organs. The boys encounter Epispeadia, in 40% of boys with an eclaffin of the bladder are not lowered in the scrotum of the testicles, the sexual member is short and flat, the thickness of the usual, is attached to the outer abdominal wall at an irregular angle.

The girls cleaved the clitoris, sex lips (safety skin folds around the vaginal and urethral holes) can be widely separated, and the vaginal opening can be very small or absent. Most girls with this pathologies are capable of conceiving a child and natural breeding.

Extrophy of the bladder and boys, and girls are combined, as a rule, with an anomaly of the location of the rectum and anus - they are significantly shifted forward. The rectal of the rectum is a consequence of its location when it can easily slip out and is also easy to fire. Extrophy of the bladder can be combined with a low navel location and lack of cartilage connecting the pubic bones. The last circumstance on the gait, as a rule, does not affect.

The progress of surgical equipment allows in most cases to reliably correct this type of development defects.

Epispadia

Epispadia is a defect of the development, characterized by an anomaly of the alignment of the urethra. At the boys with Epispeadia, the hole of the urethra is located on the upper side of the penis, the root where the front abdominal wall begins. In girls, the hole of the urethra is normally located, but the urethra is widely disclosed. Epispeadia is often combined with an eclash of the bladder. As an isolated defect of Epispadia occurs in 1 of 95,000 newborns, the boys 4 times more often than girls.

Piilyectasia

Pioektasia is the expansion of the buds of the kidneys. The explanation of the origin of the term "pyelectasia" is very simple. Like most of the complex medical names, it is origin from the Greek roots: Pyelos - "trough", "Lohan", and Ektasis - "stretching", "stretching". With stretching is clear, but with what is called "Lohan", it is necessary to figure out.

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