Congenital defects of the kidneys, urinary tract and genital organs. Developmental anomalies of the urinary tract Congenital anomaly of the urinary tract

Anomaly(from Greek anomaly - deviation, unevenness) - a structural and/or functional deviation caused by a violation of embryonic development. Anomalies of the genitourinary system are widespread and account for about 40% of all congenital defects. According to autopsy data, about 10% of people have various anomalies of the genitourinary system. To understand the reasons for their occurrence, it is necessary to highlight the basic principles of the formation of the urinary and reproductive systems. In their development, they are closely related to each other, and their excretory ducts open into the common urogenital sinus (sinus urogenitalis).

Embryogenesis of the genitourinary system

The urinary system does not develop from a single rudiment, but is represented by a number of morphological formations that successively replace each other.

1. head kidney, or preference (pronephros). In humans and higher vertebrates, it quickly disappears, replaced by the more important primary kidney.

2. Primary kidney (mesonephros) and its duct (ductus mesonephricus), which occurs before all formations involved in the formation of the genitourinary organs. On the 15th day, it appears in the mesoderm in the form of a nephrotic cord on the medial side of the body cavity, and on the 3rd week it reaches the cloaca. Mesonephros consists of a series of transverse tubules located medial to the upper part of the mesonephric duct and flowing into it at one end, while the other end of each tubule ends blindly. Mesonephros- the primary secretory organ, the excretory duct of which is the meso-nephric duct.

3. Paramesonephric duct (ductus paramesonephricus). At the end of the 4th week, a longitudinal thickening of the peritoneum appears along the outer side of each primary kidney due to the development of an epithelial cord here, which at the beginning of the 5th week turns into a duct. With its cranial end it opens into the body cavity somewhat anterior to the anterior end of the primary kidney.

4. Sex glands appear relatively later in the form of an accumulation of germinal epithelium on the medial side mesonephros. The seminiferous tubules of the testis and the egg-containing follicles of the ovary develop from germinal epithelial cells. A connective tissue cord stretches from the lower pole of the gonad down along the wall of the abdominal cavity (gubernaculum testis)- a conductor of the testicle, which with its lower end goes into the inguinal canal.

The final formation of the genitourinary organs occurs as follows. From the same nephrogenic cord from which the primary

permanent bud, permanent buds are formed (metanephros), The parenchyma of the permanent kidneys (urinary tubules) develops from the nephrogenic cord. Starting from the 3rd month, the permanent kidneys replace the primary ones as functioning excretory organs. As the body grows, the kidneys seem to move upward and take their place in the lumbar region. The pelvis and ureter develop at the beginning of the 4th week from a diverticulum at the caudal end of the mesonephric duct. Subsequently, the ureter separates from the mesonephric duct and flows into that part of the cloaca from which the bottom of the bladder develops.

Cloaca- a common cavity into which the urinary, reproductive tract and hindgut initially open. It looks like a blind sac, closed from the outside by a cloacal membrane. Subsequently, a frontal septum appears inside the cloaca, which divides it into two parts: ventral (sinus urogenitalis) And dorsal (rectum). After the cloacal membrane breaks through, both of these parts open outward with two holes: sinus urogenitalis- anterior, opening of the genitourinary system, and rectum- anus (anus).

Associated with the urogenital sinus urine bag(allantois), which in lower vertebrates serves as a reservoir for the products of kidney excretion, and in humans part of it turns into the bladder. The allantois consists of three sections: the lower- sinus urogenitalis, from which the triangle of the bladder is formed; middle extended section, which turns into the rest of the bladder, and upper narrowed section, representing the urinary tract (urachus), running from the bladder to the navel. In lower vertebrates, it serves to drain the contents of the allantois, and in humans, by the time of birth it is empty and turns into a fibrous cord (lig. umbilicale medianum).

Ductus paramesonephrici give rise to the development of the fallopian tubes, uterus and vagina in women. Fallopian tubes are formed from the upper parts ductus paramesonephrici, and the uterus and vagina are made of fused lower parts. In men ductus paramesonephrici are reduced, and only the appendage of the testicle remains (appendix testis) and prostatic uterus (utriculus prostaticus). Thus, in men, they undergo reduction and transformation into rudimentary formations. ductus paramesonephrici, and for women - ductus mesonephrici.

Around the slit-like hole sinus urogenitalis at the 8th week of intrauterine development, the rudiments of the external genitalia are noticeable, initially identical in male and female embryos. At the anterior end of the external, or genital, sinus fissure lies the genital tubercle, the edges of the sinus are formed by urogenital folds, the genital tubercle and genital folds are surrounded on the outside by labioscrotal tubercles.

In men, these rudiments undergo the following changes: the genital tubercle develops greatly in length, from which a penis Along with its growth, the gap located under the lower surface increases penis Later, when the urogenital folds grow together, this gap forms the urethra. The labial-scrotal tubercles grow rapidly and turn into the scrotum, fused along the midline.

In women, the genital tubercle turns into the clitoris. The expanding genital folds form the labia minora, but there is no complete union

no folds occur and sinus urogenitalis remains open, forming the vestibule of the vagina (vestibulum vaginae). The labial-scrotal tubercles, which then turn into the labia majora, do not fuse.

Due to the close connection between the development of the urinary and reproductive systems, in 33% of cases, abnormalities of the urinary system organs are combined with abnormalities of the genital organs. Malformations of the genitourinary system are often associated with malformations of other organs and systems.

5.1. KIDNEY ANOMALIES Classification

Renal vascular abnormalities

■ Quantity abnormalities: solitary renal artery;

segmental renal arteries (double, multiple).

■ Positional anomalies: lumbar; ileal;

pelvic dystopia of the renal arteries.

■ Anomalies of the shape and structure of the arterial trunks: aneurysms of the renal arteries (unilateral and bilateral); fibromuscular stenosis of the renal arteries; genicular renal artery.

■ Congenital arteriovenous fistulas.

■ Congenital changes in the renal veins:

anomalies of the right renal vein (multiple veins, the confluence of the testicular vein into the renal vein on the right);

anomalies of the left renal vein (annular left renal vein, retro-aortic left renal vein, extracaval entry of the left renal vein).

Kidney number abnormalities

■ Aplasia.

■ Kidney duplication (complete and incomplete).

■ Accessory, third kidney.

Abnormalities in kidney size

■ Renal hypoplasia.

Anomalies in the location and shape of the kidneys

■ Kidney dystopia:

unilateral (thoracic, lumbar, iliac, pelvic); cross.

■ Renal fusion: unilateral (L-shaped kidney);

bilateral (horseshoe-shaped, biscuit-shaped, asymmetrical - L- and S-shaped buds).

Abnormalities of kidney structure

■ Kidney dysplasia.

■ Multicystic kidney disease.

■ Polycystic kidney disease: adult polycystic disease; polycystic childhood.

■ Solitary renal cysts: simple; dermoid.

■ Parapelvic cyst.

■ Diverticulum of the calyx or pelvis.

■ Calyceal-medullary anomalies: spongy kidney;

megacalyx, polymegacalyx.

Combined kidney anomalies

■ With vesicoureteral reflux.

■ With bladder outlet obstruction.

■ With vesicoureteral reflux and bladder outlet obstruction.

■ With anomalies of other organs and systems.

Renal vascular abnormalities

Quantity anomalies. These include the blood supply to the kidney through the solitary and segmental arteries.

Solitary renal artery- This is a single arterial trunk that arises from the aorta and then divides into the corresponding renal arteries. This malformation of the blood supply to the kidneys is casuistry.

Normally, each kidney is supplied with blood by one separate arterial trunk, arising from the aorta. An increase in their number should be attributed to the segmental scattered type of structure of the renal arteries. In the literature, including educational literature, often one of the two arteries supplying the kidney, especially if it has a smaller diameter, is called additional. However, in anatomy, an accessory, or aberrant, artery is considered to be one that supplies a certain area of ​​the organ in addition to the main artery. Both of these arteries form a wide network of anastomoses among themselves in their common vascular basin. Two or more renal arteries each supply blood to one specific segment of the kidney and do not form anastomoses with each other during division.

Thus, if there are two or more arterial vessels of the kidney, each of them is the main one for it, and not additional. Ligation of any of them leads to necrosis of the corresponding area of ​​the renal parenchyma, and this should not be done when performing corrective operations for hydronephrosis caused by the inferior polar vessels of the kidney, unless its resection is planned.

Rice. 5.1. Multislice CT, three-dimensional reconstruction. Multiple segmental type of structure of the renal arteries

From these positions, the number of renal arteries greater than one should be considered abnormal, that is, the segmental type of blood supply to the organ. The presence of two arterial trunks, regardless of their caliber - double (double) renal artery, and if there are more of them - multiple type of structure of the renal arteries(Fig. 5.1). As a rule, this malformation is accompanied by a similar structure of the renal veins. Most often it is combined with anomalies in the location and number of kidneys (double, dystopic, horseshoe-shaped kidney), but can also be observed with a normal structure of the organ.

Abnormalities of the position of the renal vessels - a developmental defect characterized by an atypical origin of the renal artery from the aorta and determining the type of kidney dystopia. Highlight lumbar(with low origin of the renal artery from the aorta), ileum(when originating from the common iliac artery) and pelvic(when originating from the internal iliac artery) dystopia.

Anomalies of shape and structure. Renal artery aneurysm- local expansion of the artery due to the absence of muscle fibers in its wall. This anomaly is usually one-sided. A renal artery aneurysm can manifest itself as arterial hypertension, thromboembolism with the development of renal infarction, and if it ruptures, massive internal bleeding. For renal artery aneurysm, surgical treatment is indicated. The aneurysm is resected and the vascular wall defect is sutured

or plastic surgery of the renal artery with synthetic materials.

Fibromuscular stenosis- an anomaly of the renal arteries, caused by excess content of fibrous and muscle tissue in the vascular wall (Fig. 5.2).

This malformation is more common in women, is often combined with nephroptosis and can be bilateral. The disease leads to a narrowing of the lumen of the renal artery, which causes the development of arterial hypertension. Its peculiarity in fibromuscular

Rice. 5.2. Multislice CT. Fibromuscular stenosis of the right renal artery (arrow)

Rice. 5.3. Selective arteriogram of the kidney. Multiple arteriovenous fistulas (arrows)

Lary stenosis is characterized by high diastolic and low pulse pressure, as well as refractoriness to antihypertensive therapy. The diagnosis is established on the basis of renal angiography, multispiral computed angiography and radioisotope studies of the kidneys. Selective blood sampling is performed from the renal vessels to determine the concentration of renin. Treatment is surgical. Balloon dilatation (expansion) of the renal artery stenosis and/or installation of an arterial stent is performed. If angioplasty or stenting is impossible or ineffective, reconstructive surgery is performed - renal artery replacement.

Congenital arteriovenous fistulas - a malformation of the renal vessels, in which there are pathological anastomoses between the vessels of the arterial and venous circulatory systems. Arteriovenous fistulas are usually localized in the arcuate and lobular arteries of the kidney. The disease is often asymptomatic. Possible clinical manifestations may be hematuria, albuminuria and varicocele on the corresponding side. The main method for diagnosing arteriovenous fistulas is renal arteriography (Fig. 5.3). Treatment consists of endovascular occlusion (embolization) of pathological anastomosis with special emboli.

Congenital change of the renal veins. Anomalies of the right renal vein are extremely rare. Among them, the most common increasing the number of venous trunks (doubling, tripling). Malformations of the left renal vein are presented anomalies in its quantity, shape and position.

Accessory and multiple renal veins occur in 17-20% of cases. Their clinical significance is that those that go to the lower pole of the kidney, accompanying the corresponding artery, intersect with the ureter, thereby causing a violation of the outflow of urine from the kidney and the development of hydronephrosis.

Anomalies of shape and location include ring-shaped(passes in two trunks around the aorta), retroaortic(passes behind the aorta and flows into the inferior vena cava at the level of the II-IV lumbar vertebrae) extracaval(does not flow into the inferior vena cava, but more often into the left common iliac vein) renal veins. The diagnosis is based on data from venocavography, selective renal venography. In cases of severe venous hypertension, they resort to surgery - an anastomosis between the left testicular and common iliac veins.

In most cases, abnormal renal vessels do not manifest themselves in any way and are often an incidental finding during examination of patients, but information about them is extremely important when planning surgical interventions. Clinically, malformations of the renal vessels appear in cases where they cause a violation of the outflow of urine from the kidneys. The diagnosis is established on the basis of Doppler ultrasound scanning, aortic and venous cavagraphy, multislice CT and MRI.

Kidney number abnormalities

Aplasia- congenital absence of one or both kidneys and renal vessels. Bilateral renal aplasia is incompatible with life. Aplasia of one kidney is relatively common - in 4-8% of patients with kidney anomalies. It occurs due to underdevelopment of metanephrogenic tissue. In half of the cases, on the side of the kidney aplasia there is no corresponding ureter; in other cases, its distal end ends blindly (Fig. 5.4).

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

Information about the presence of a single kidney in a patient is extremely important, since the development of diseases in it always requires special treatment tactics. A single kidney is functionally more adapted to the influence of various negative factors. With renal aplasia, its compensatory (vicarious) hypertrophy is always observed.

Rice. 5.4. Aplasia of the left kidney and ureter

Excretory urography and ultrasound can detect a single, enlarged kidney. A characteristic sign of the disease is the absence of renal vessels on the side of aplasia, therefore the diagnosis is reliably established on the basis of methods that make it possible to prove the absence of not only the kidneys, but also its vessels (renal arteriography, multispiral computed tomography and magnetic resonance angiography). The cystoscopic picture is characterized by the absence of the corresponding half of the interureteric fold and the orifice of the ureter. When the ureter ends blindly, its mouth is hypotrophied, contraction and urine output are absent. This type of defect is confirmed by catheterization of the ureter with retrograde ureterography.

Rice. 5.5. Sonogram. Kidney duplication

Kidney duplication- the most common abnormality of the number of kidneys, occurring in one case in 150 autopsies. In women, this malformation is observed 2 times more often.

As a rule, each half of the doubled kidney has its own blood supply. A characteristic feature of this anomaly is anatomical and functional asymmetry. The upper half is often less developed. Symmetry of the organ or predominance in the development of the upper half is much less common.

Kidney duplication may be one- And bilateral, and complete And incomplete(Fig. 41, 42, see color insert). Complete duplication implies the presence of two pyelocaliceal systems, two ureters, opening with two orifices in the bladder (ureter duplex). In incomplete duplication, the ureters eventually fuse into one and open into one orifice in the bladder (ureter fissus).

Often, complete doubling of the kidney is accompanied by an anomaly in the development of the lower part of one of the ureters: its intravesical or extravesical ectopia

Rice. 5.6. Excretory urograms:

A- incomplete duplication of the urinary tract on the left (ureter fissus); b- complete duplication of the urinary tract on the left (ureter duplex)(arrows)

(opening into the urethra or vagina), the formation of ureterocele or incompetence of the vesicoureteral junction with the development of reflux. A characteristic sign of ectopia is the constant leakage of urine while maintaining normal urination. A double kidney, not affected by any disease, does not cause clinical manifestations and is detected in patients during a random examination. However, it is more often than normal susceptible to various diseases, such as pyelonephritis, urolithiasis, hydronephrosis, nephroptosis, and neoplasms.

Making a diagnosis is not difficult and involves ultrasound (Fig. 5.5), excretory urography (Fig. 5.6), CT, MRI and endoscopic (cystoscopy, ureteral catheterization) research methods.

Surgical treatment is performed only in the presence of urodynamic disorders associated with abnormal course of the ureters, as well as other diseases of the double kidney.

Accessory kidney- an extremely rare abnormality in the number of kidneys. The third kidney has its own blood supply, fibrous and fatty capsules and a 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 ectopic. The size of the accessory bud is significantly reduced.

The diagnosis is made using the same methods as for other kidney anomalies. The development of complications in the accessory kidney such as chronic pyelonephritis, urolithiasis and others is an indication for nephrectomy.

Abnormality in kidney size

Renal hypoplasia (dwarf kidney)- congenital reduction of an organ in size with a normal morphological structure of the renal parenchyma without disrupting its function. This malformation, as a rule, is combined with an increase in the contralateral

kidneys Hypoplasia is most often unilateral, much less often it is observed on both sides.

One-sided renal hypoplasia may not be clinically manifested, however, in an abnormal kidney, pathological processes develop much more often. Double-sided hypoplasia is accompanied by symptoms of arterial hypertension and renal failure, the severity of which depends on the degree of the congenital defect and complications arising mainly due to the addition of infection.

Rice. 5.7. Sonogram. Pelvic dystopia of a hypoplastic kidney (arrow)

Rice. 5.8. Scintigram. Hypoplasia of the left kidney

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

Particular difficulties are presented by the differential diagnosis of hypoplasia from dysplasia And kidney wrinkled as a result of nephrosclerosis. Unlike dysplasia, this anomaly is characterized by the normal structure of the renal vessels, pyelocaliceal system and ureter. Nephrosclerosis is often the outcome of chronic pyelonephritis or develops as a result of hypertension. Cicatricial degeneration of the kidney is accompanied by a characteristic deformation of its contour and calyces.

Treatment of patients with a hypoplastic kidney is carried out when pathological processes develop in it.

Anomaly of the location and shape of the kidneys

Anomaly of the location of the kidney - dystopia- location of the kidney in an anatomical area that is not typical for it. This anomaly occurs in one in 800-1000 newborns. The left kidney is dystopic more often than the right.

The reason for the formation of this malformation is a violation of the movement of the kidney from the pelvis to the lumbar region during intrauterine development. Dystopia is caused by fixation of the kidney in the early stages of embryonic development by abnormally developed vascular apparatus or insufficient growth of the ureter in length.

Depending on the level of location, there are thoracic, lumbar, sacroiliac And pelvic dystopia(Fig. 5.9).

Abnormalities in the location of the kidneys may be one-sided And bilateral. Dystopia of the kidney without its displacement to the opposite side is called homolateral. The dystopic kidney is located on its side, but above or below the normal position. Heterolateral (cross) dystopia- a rare developmental defect detected with a frequency of 1: 10,000 autopsies. It is characterized by a displacement of the kidney to the opposite side, as a result of which both of them are located on the same side of the spine (Fig. 5.10). With cross dystopia, both ureters open in the bladder, as with the normal location of the kidneys. The vesical triangle is preserved.

A dystopic kidney can cause constant or periodic pain in the corresponding half of the abdomen, lumbar region, or sacrum.

Rice. 5.9. Types of kidney dystopia: 1 - thoracic; 2 - lumbar; 3 - sacroiliac; 4 - pelvic; 5 - normally located left kidney

Rice. 5.10. Heterolateral (crossed) dystopia of the right kidney

An abnormally located kidney can often be palpated through the anterior abdominal wall.

This anomaly ranks first among the causes of erroneously performed surgical interventions, since the kidney is often mistaken for a tumor, appendiceal infiltration, pathology of the female genital organs, etc. In dystopic kidneys, pyelonephritis, hydronephrosis, and urolithiasis often develop.

The greatest difficulty in making a diagnosis is caused by pelvic dystopia. This location of the kidney can manifest as pain in the lower abdomen and simulate acute surgical pathology. Lumbar and iliac dystopia, even not complicated by any disease, can manifest as pain in the corresponding area. Pain in the most rare thoracic dystopia of the kidney is localized behind the sternum.

The main methods for diagnosing abnormalities of the kidneys are ultrasound, x-ray, CT and renal angiography. The lower the dystopic kidney is, the more ventrally its gate is located and the pelvis is rotated anteriorly. With ultrasound and excretory urography, the kidney is located in an atypical place and, as a result of rotation, appears flattened (Fig. 5.11).

If there is insufficient contrasting of the dystopic kidney, according to excretory urography, retrograde ureteropyelography is performed (Fig. 5.12).

Rice. 5.11. Excretory urogram. Pelvic dystopia of the left kidney (arrow)

Rice. 5.12. Retrograde ureteropyelogram. Pelvic dystopia of the right kidney (arrow)

The lower the dystopia of the organ, the shorter the ureter will be. On angiograms, the renal vessels are located low and can arise from the abdominal aorta, aortic bifurcation, common iliac and hypogastric arteries (Fig. 5.13). The presence of multiple vessels supplying the kidney is characteristic. This anomaly is most clearly detected on multislice CT with con-

by screening (Fig. 39, see color insert). Incomplete rotation of the kidney and a short ureter are important differential diagnostic signs that make it possible to distinguish kidney dystopia from nephroptosis. A dystopic kidney, unlike the early stages of nephroptosis, is deprived of mobility.

Treatment of dystopic kidneys is carried out only if a pathological process develops in them.

Shape anomalies include various types kidney fusion between themselves. Fused kidneys occur in 16.5% of cases among all their anomalies.

Fusion involves the union of two kidneys into one organ. Cro-

Rice. 5.13. Renal angiogram. Pelvic dystopia of the left kidney (arrow)

it is always supplied by abnormal multiple renal vessels. In such a kidney there are two pyelocaliceal systems and two ureters. Since fusion occurs in the early stages of embryogenesis, normal rotation of the kidneys does not occur, and both pelvises are located on the anterior surface of the organ. Abnormal position or compression of the ureter by the inferior polar vessels leads to its obstruction. In this regard, this anomaly is often complicated by hydronephrosis and pyelonephritis. It can also be combined with vesicoureteropelvic reflux.

Depending on the relative position of the longitudinal axes of the buds, horseshoe-shaped, biscuit-shaped, S- and L-shaped buds are distinguished (Fig. 45-48, see color insert).

Kidney fusion may be symmetrical And asymmetrical. In the first case, the buds are fused with poles of the same name, usually the lower ones and, very rarely, the upper (horseshoe-shaped bud) or middle sections (biscuit-shaped bud). In the second, fusion occurs with opposite poles (S-, L-shaped buds).

Horseshoe kidney is the most common fusion anomaly. In more than 90% of cases, fusion of the kidneys with the lower poles is observed. More often, such a kidney consists of symmetrical kidneys of the same size and is dystopic. The dimensions of the fusion zone, the so-called isthmus, can be very different. Its thickness, as a rule, ranges from 1.5-3, width 2-3, length - 4-7 cm.

When one kidney is located in a typical place, and the second, fused to it at a right angle, across the spine, the kidney is called L-shaped.

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

Galette-shaped the kidney is usually located below the promontorium in the pelvic area. The volume of the parenchyma of each half of the biscuit-shaped kidney is different, which explains the asymmetry of the organ. The ureters usually enter the bladder in the usual place and very rarely cross each other.

Clinically, fused kidneys may manifest as pain in the para-umbilical area. Due to the peculiarities of the blood supply and innervation of the horseshoe kidney and the pressure of its isthmus on the aorta, vena cava and solar plexus, even in the absence of pathological changes in it, characteristic

Rice. 5.14. Excretory urogram. L-shaped kidney (arrows)

Rice. 5.15. CT with contrast (frontal projection). Horseshoe kidney. Weak vascularization of the isthmus due to the predominance of fibrous tissue in it

Rice. 5.16. Multislice CT (axial projection). Horseshoe kidney

symptoms. With such a kidney, the appearance or intensification of pain in the navel area during bending of the body back is typical (Rovsing's symptom). Digestive disorders may occur - pain in the epigastric region, nausea, bloating, constipation.

Ultrasound, excretory urography (Fig. 5.14) and multislice CT (Fig. 5.15, 5.16) are the main methods for diagnosing fused kidneys and identifying their possible pathology (Fig. 5.17).

Treatment is carried out for the development of diseases of the abnormal kidney (urolithiasis, pyelonephritis, hydronephrosis). When hydronephrosis of the horseshoe kidney is detected, it should be determined whether it is a consequence of the obstruction of the pyelo-ureteral segment characteristic of this disease (stricture, intersection of the ureter with the lower polar vascular bundle) or formed due to pressure on it from the isthmus of the horseshoe kidney. In the first case it is necessary

perform plasty of the pyeloureteral segment, and in the second - resection (rather than dissection) of the isthmus or ureterocalicoanastomosis (Neiwert's operation).

Abnormalities of kidney structure

Kidney dysplasia is characterized by a decrease in its size with a simultaneous disruption of the development of blood vessels, parenchyma, pyelocaliceal system and a decrease in renal function. This anomaly occurs as a result

Rice. 5.17. Multislice CT (frontal projection). Hydronephrotic transformation of horseshoe kidney

insufficient induction of the metanephros duct to differentiate the metanephrogenic blastema after their fusion. It is extremely rare that such an anomaly is bilateral and is accompanied by severe renal failure.

Clinical manifestations of kidney dysplasia arise as a result of the addition of chronic pyelonephritis and the development of arterial hypertension. Differential diagnostic difficulties arise when distinguishing dysplasia from hypoplasia and a wrinkled kidney. Radiation methods help in making the diagnosis, primarily multislice CT with contrast (Fig. 5.18), static and dynamic nephroscintigraphy.

The most common malformations of the structure of the renal parenchyma are cortical cystic lesions (multycystic, polycystic and solitary renal cyst). These anomalies are united by the mechanism of disruption of their morphogenesis, which consists in the dissonance of the connection of the primary tubules of the metanephrogenic blastema with the metanephros duct. They differ in the timing of disruption of such fusion during the period of embryonic differentiation, which determines the severity of structural changes in the kidney parenchyma and the degree of its functional insufficiency. The most pronounced changes in the parenchyma, incompatible with its function, are observed in multicystic kidney disease.

Multicystic kidney- a rare anomaly characterized by multiple cysts of different shapes and sizes, occupying the entire parenchyma, with the absence of its normal tissue and underdevelopment of the ureter. Intercystic spaces are represented by connective and fibrous tissue. A multicystic kidney is formed as a result of a disruption in the connection of the metanephros duct with the metanephrogenic blastema and the absence of excretory anlage while maintaining the secretory apparatus of the permanent kidney in the early stages of its embryogenesis. Urine, when formed, accumulates in the tubules and, having no outlet, stretches them, turning them into cysts. The contents of cysts are usually clear liquid, vaguely resembling

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

As a rule, multicystic kidney disease is a unilateral process, often combined with malformations of the contralateral kidney and ureter. Bilateral multi-cystic disease is incompatible with life.

Before infection occurs, a unilateral multicystic kidney does not manifest itself clinically and may be an incidental finding during a clinical examination. The diagnosis is established using sonography and X-ray radionuclide research methods with separate determination of renal function. In contrast

Rice. 5.18. Multislice CT. Left kidney dysplasia (arrow)

Rice. 5.19. Sonogram. Polycystic kidney disease

from polycystic disease, multicystic disease is always a one-sided process with a lack of function of the affected organ.

Surgical treatment consists of nephrectomy.

Polycystic kidney disease- a developmental defect characterized by the replacement of the renal parenchyma by multiple cysts of various sizes. This is a severe bilateral process, often accompanied by chronic pyelonephritis, arterial hypertension and increasing chronic renal failure.

Polycystic disease is quite common - 1 case in 400 autopsies. In a third of patients, cysts are detected in the liver, but they are few in number and do not impair the function of the organ.

In pathogenetic and clinical terms, this anomaly is divided into polycystic kidney disease in children and adults. Childhood polycystic disease is characterized by an autosomal recessive single type of transmission of the disease, while adult polycystic disease is characterized by an autosomal dominant one. This anomaly in children is severe, most of them do not live to adulthood.

Rasta. Polycystic disease in adults has a more favorable course, manifesting itself in young or middle age, and has been compensated for many years. The average life expectancy is 45-50 years.

Macroscopically, the kidneys are enlarged in size due to many cysts of different diameters; the amount of functioning parenchyma is minimal (Fig. 44, see color insert). The growth of cysts causes ischemia of intact renal tubules and death of renal tissue. This process is facilitated by associated chronic pyelonephritis and nephrosclerosis.

Patients complain of abdominal and lumbar pain, weakness, fatigue, thirst, dry mouth, headache, which is associated with chronic renal failure and increased blood pressure.

Rice. 5.20. Excretory urogram. Polycystic kidney disease

Rice. 5.21. CT. Polycystic kidney disease

Significantly enlarged dense tuberous kidneys are easily identified by palpation. Other complications of polycystic disease are gross hematuria, suppuration and malignancy of cysts.

Blood tests show anemia, increased creatinine and urea levels. The diagnosis is established on the basis of ultrasound and X-ray radionuclide research methods. Characteristic signs are increased by a factor of

measures of the kidney, entirely represented by cysts of different sizes, compression of the pelvis and calyces, the necks of which are elongated, medial deviation of the ureter is determined (Fig. 5.19-5.21).

Conservative treatment of polycystic disease consists of symptomatic and antihypertensive therapy. Patients are under medical supervision by a urologist and nephrologist. Surgical treatment is indicated for the development of complications: suppuration of cysts or malignancy. Taking into account the two-way process, it should be organ-preserving in nature. Percutaneous puncture of cysts can be performed routinely, as well as their excision using laparoscopic or open access. In cases of severe chronic renal failure, hemodialysis and kidney transplantation are indicated.

Solitary renal cyst. The developmental defect has the most favorable course and is characterized by the formation of one or several cysts localized in the cortical layer of the kidney. This anomaly is equally common in people of both sexes and is observed mainly after 40 years.

Solitary cysts can be simple And dermoid. A solitary simple cyst can be not only congenital, but also acquired. A congenital simple cyst develops from germinal collecting ducts that have lost connection with the urinary tract. 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 the cyst is represented by single-layer squamous epithelium. Its contents are most often serous, in 5% of cases hemorrhagic. Hemorrhage into a cyst is one of the signs of its malignancy.

A simple cyst usually occurs single (solitary), although they meet multiple, multi-chamber, including bilateral cysts. Their size ranges from 2 cm in diameter to giant formations with a volume of more than 1 liter. Most often, cysts are localized in one of the poles of the kidney.

Kidney dermoid cysts are extremely rare. They may contain fat, hair, teeth and bones, which are revealed by X-ray examination.

Simple small cysts are asymptomatic and are an incidental finding during examination. Clinical manifestations begin as

Rice. 5.22. Sonogram. Cyst (1) kidneys (2)

an increase in the size of the cyst, and they are primarily associated with its complications, such as compression of the pyelocaliceal system, ureter, kidney vessels, suppuration, hemorrhage and malignancy. A large kidney cyst may rupture.

Large solitary renal cysts are palpated as an elastic, smooth, mobile, painless formation. A characteristic sonographic sign of a cyst is the presence of a hypoechoic homogeneous,

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

On excretory urograms, multispiral CT with contrast and MRI, the kidney is enlarged in size due to a round, thin-walled, homogeneous liquid formation, which to one degree or another deforms the pyelocaliceal system and causes deviation of the ureter (Fig. 5.23). The pelvis is compressed, the calyxes are pushed back, pushed apart, and with obstruction of the neck of the calyx, hydrocalyx occurs. These studies also make it possible to identify abnormalities of the kidney vessels and the presence of other kidney diseases.

(Fig. 5.24).

On a selective renal arteriogram, a low-contrast avascular shadow of a round formation is determined at the location of the cyst (Fig. 5.25). Static nephroscintigraphy reveals a round defect in radiotracer accumulation.

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

Rice. 5.24. Multislice CT with contrast. Multiple segmental type of renal arteries (1), cyst (2) and tumor (3) of the kidney

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

Differential diagnosis is carried out with multicystic disease, polycystic disease, hydronephrosis and, especially, kidney tumors.

Indications for surgical treatment are the size of the cyst more than 3 cm and the presence of its complications. The simplest method is percutaneous puncture of the cyst under ultrasound guidance with aspiration of its contents, which are subject to cytological examination. If necessary, cystography is performed. After evacuation of the contents, sclerosing substances (ethyl alcohol) are injected into the cyst cavity. The method gives a high percentage of relapses, since the membranes of the cyst, capable of producing fluid, are preserved.

Currently, the main treatment method is laparoscopic or retroperitoneoscopic excision of the cyst. Open surgery - lumbotomy - is rarely used (Fig. 66, see color insert). It is indicated when the cyst reaches enormous sizes, is multifocal in nature with atrophy of the renal parenchyma, and also in the presence of its malignancy. In such cases, kidney resection or nephrectomy is performed.

Parapelvic cyst is a cyst located in the region of the renal sinus, the hilum of the kidney. The wall of the cyst is closely adjacent to the vessels of the kidney and pelvis, but does not communicate with it. The reason for its formation is the underdevelopment of the lymphatic vessels of the renal sinus during the neonatal period.

The clinical manifestations of a parapelvic cyst are determined by its location, that is, pressure on the pelvis and vascular pedicle of the kidney. Patients experience pain. Hematuria and arterial hypertension may occur.

Diagnosis is the same as for solitary renal cysts. Differential diagnosis is carried out with the expansion of the pelvis during hydronephrosis, for which ultrasound and X-ray methods with contrasting of the urinary tract are used.

The need for treatment arises when the cyst increases significantly in size and complications develop. Technical difficulties during its excision are associated with the proximity of the pelvis and renal vessels.

Diverticulum of the calyx or pelvis It is a rounded single fluid formation communicating with them, lined with urothelium. It resembles a simple renal cyst and was previously incorrectly called a calyceal or pelvic cyst. The fundamental difference between a diverticulum and a solitary cyst is its connection by a narrow isthmus with the renal cavity system, which characterizes this formation as a true diverticulum of the kidney.

Rice. 5.26. Multislice CT with contrast. Left renal calyx diverticulum (arrow)

cervix or pelvis. The diagnosis is established on the basis of excretory urography and multislice CT with contrast (Fig. 5.26).

In some cases, retrograde ureteropyelography or percutaneous diverticulography may be performed. Based on these methods, the communication of the diverticulum with the pyelocaliceal system of the kidney is clearly established.

Surgical treatment is indicated for large diverticulum sizes and complications arising in connection with this. It consists of resection of the kidney with excision of the diverticulum.

Calyceal-medullary anomalies.Sponge bud- a very rare malformation characterized by cystic expansion of the distal

parts of the collecting ducts. The damage is predominantly bilateral, diffuse, but the process may be limited to part of the kidney. Spongy kidney is more common in boys and has a favorable course, practically without affecting kidney function.

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

A spongy kidney is diagnosed using x-ray methods. Observational and excretory urograms often reveal nephrocalcinosis - a characteristic accumulation of calcifications and/or fixed small stones in the area of ​​the renal pyramids, which, like a cast, emphasize their contour. In the medulla, corresponding to the pyramids, a large number of small cysts are detected. Some of them protrude into the lumen of the calyxes, resembling a bunch of grapes.

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

Patients with uncomplicated spongy kidney do not require treatment. Surgical treatment is indicated for the development of complications: stone formation, hematuria.

Megacalyx (megacalycosis)- congenital non-obstructive expansion of the calyx, resulting from medullary dysplasia. The expansion of all groups of calyxes is called polymegacalyx (megapolycalycosis).

With megacalyx, the size of the kidney is normal, its surface is smooth. The cortical layer is of normal size and structure, the medulla is underdeveloped and thinned. The papillae are flattened and poorly differentiated. Advanced

Rice. 5.27. Excretory urogram. Megapolycalicosis on the left

the calyces can pass directly into the pelvis, which, unlike in the case of hydronephrosis, remains normal in size. The pyeloureteral segment is formed normally, the ureter is not narrowed. In an uncomplicated course, kidney function is not impaired. The expansion of the calyces is not caused by obstruction of their necks, as happens when there is a stone in this area or Fraley's syndrome (compression of the neck of the calyx by a segmental arterial trunk), but is of a congenital non-obstructive nature.

For diagnosis, ultrasound and X-ray methods with contrast of the urinary tract are used. Excretory urograms show expansion of all groups of calyces with the absence of ectasia of the pelvis (Fig. 5.27).

Megapolycalicosis, unlike hydronephrosis, in uncomplicated cases does not require surgical correction.

5.2. URETER ANOMALIES

Developmental defects ureters account for 22% of all anomalies of the urinary system. In some cases they are combined with anomalies of kidney development. As a rule, abnormalities of the ureters lead to disturbances in urodynamics. The following classification of ureteral malformations is accepted.

■ agenesis (aplasia);

■ doubling (full and incomplete);

■ tripling.

■ retrocaval;

■ retroiliac;

■ ectopia of the ureteric orifice.

Abnormalities in the shape of the ureters

■ spiral (ring-shaped) ureter.

■ hypoplasia;

■ neuromuscular dysplasia (achalasia, megaureter, megadolihoureter);

■ congenital narrowing (stenosis) of the ureter;

■ ureteral valve;

■ ureteral diverticulum;

■ ureterocele;

■ vesicoureteropelvic reflux. Abnormalities in the number of ureters

Agenesis (aplasia)- congenital absence of the ureter, caused by underdevelopment of the ureteric germ. In some cases, the ureter can be determined in the form of a fibrous cord or a blindly ending process (Fig. 5.28). One-sided agenesis of the ureter is combined with agenesis of the kidney on the same side or multicystic disease. Double-sided It is extremely rare and incompatible with life.

Diagnosis is based on data from X-ray studies with contrast and nephroscintigraphy, which reveal the absence of one kidney. Characteristic cystoscopic signs are underdevelopment or absence of half of the vesical triangle and the orifice of the ureter on the corresponding side. When the distal part of the ureter is preserved, its opening is also underdeveloped, although it is located in the usual place. In this case, retrograde ureterography allows confirming the blind end of the ureter.

Surgical treatment is performed when a purulent-inflammatory process develops and stones form in the blind-ending ureter. The affected organ is surgically removed.

Doubling- the most common malformation of the ureters. This anomaly occurs 5 times more often in girls than in boys.

Typically, during caudal migration, the ureter of the lower half of the kidney is the first to connect with the bladder and, therefore, occupies a higher and lateral position than the ureter of its upper half. The ureters in the pelvic sections cross each other and flow into the bladder in such a way that the mouth of the upper one is located below and medially, and the lower one is located above and laterally (Weigert-Meyer law) (Fig. 5.29).

Duplication of the upper urinary tract may be one- or double-sided, full (ureter duplex) And incomplete (ureter fissus)(Fig. 41, 42, see color insert). In case of complete doubling each

Rice. 5.28. Aplasia of the left kidney. Blindly ending ureter

Rice. 5.29. Weigert-Meyer law. Crossing of the ureters and the location of their mouths in the bladder with complete duplication of the urinary tract

The ureter opens into a separate orifice in the bladder. Incomplete duplication of the upper urinary tract is characterized by the presence of two pelvis and ureters, connecting in the pelvic region and opening in the bladder with one mouth.

The described features of the topography of the ureters with duplication of the upper urinary tract predispose to complications. Thus, the ureter of the lower half of the kidney, which has a higher and laterally located mouth, has a short submucosal tunnel, which is the reason for the high frequency of vesicoureteropelvic reflux into this ureter. On the contrary, the orifice of the ureter of the upper half of the kidney is often ectopic and prone to stenosis, which is the cause of the development of hydroureteronephrosis.

Duplication of the ureter in the absence of urodynamic disturbances is not clinically manifested. This anomaly can be suspected by sonography, in which the doubling of the kidney is determined, and the ureters, if dilated, can be visible in their pelvic or pelvic regions. The final diagnosis is established on the basis of excretory urography, multislice CT with contrast, MRI and cystoscopy. In the absence of function of one half of the kidney, the diagnosis can be confirmed by antegrade or retrograde ureteropyelography.

Triplication of the pelvis and ureters is casuistry.

Treatment is surgical if complications develop. In case of narrowing or ectopia of the ureter, ureterocystoanastomosis is performed, and in case of vesicoureteral reflux, antireflux operations are performed. If the function of the entire kidney is lost, nephroureterectomy is indicated (Fig. 60, see color insert), and heminefroureterectomy is indicated for one of its halves.

Abnormalities of the position of the ureters

Retrocaval ureter- a rare anomaly in which the ureter in the lumbar region goes under the vena cava and, having gone around it in a ring, returns to its previous position when passing into the pelvic region (Fig. 43, see color insert). Compression of the ureter by the inferior vena cava leads to disruption of urine passage with the development of hydroureteronephrosis and its characteristic clinical picture. This anomaly can be suspected by ultrasound and excretory urography, which reveals an expansion of the cavity system of the kidney and ureter to its middle third, a loop-like bend and the normal structure of the ureter in the pelvic region. The diagnosis is confirmed using multislice CT and MRI.

Surgical treatment consists of intersection, as a rule, with resection of the altered sections of the ureter and performing ureteroureteroanastomosis with the placement of the organ in its normal position to the right of the vena cava.

Retroiliac ureter- an extremely rare malformation in which the ureter is located behind the iliac vessels (Fig. 43, see color insert). This anomaly, like the retrocaval ureter, leads to its obstruction with the development of hydroureteronephrosis. Surgical treatment consists of dividing the ureter, releasing it from under the vessels and performing antevasal ureteroureteroanastomosis.

Ectopic ureteral orifice- an anomaly characterized by an atypical intravesical or extravesical location of the orifices of one or both ureters. This malformation is more common in girls and, as a rule, is combined with duplication of the ureter and/or ureterocele. The cause of this anomaly is a delay or disruption of the separation of the ureteric germ from the Wolffian duct during embryogenesis.

TO intravesical Types of ectopia of the ureteric orifice include its displacement downward and medially into the neck of the bladder. This change in the location of the mouth is usually asymptomatic. The orifices of the ureters with their extravesical ectopia opens into the urethra, paraurethral, ​​uterus, vagina, vas deferens, seminal vesicle, rectum.

The clinical picture of extravesical ectopia of the ureteral orifice is determined by its location and depends on gender. In girls, this developmental defect manifests itself as urinary incontinence with preserved normal urination. In boys, during intrauterine development of the Wolffian duct turns into the ejaculatory duct and seminal vesicles, therefore the ectopic orifice of the ureter is always located proximal to the urethral sphincter and urinary incontinence does not occur.

Diagnosis is based on the results of a comprehensive examination, including excretory urography, CT, vaginography, urethro- and cystoscopy, catheterization of the ectopic orifice and retrograde urethro- and ureterography.

Treatment for this anomaly is surgical and consists of transplanting the ectopic ureter into the bladder (ureterocystoanastomosis), and in the absence of kidney function - nephroureterectomy or heminefroureterectomy.

Abnormalities in the shape of the ureters

Spiral (ring-shaped) ureter- an extremely rare malformation in which the ureter in the middle third has the shape of a spiral or ring. The process may wear one- And bilateral character. This anomaly is a consequence of the inability of the ureter to rotate with the kidney during its intrauterine movement from the pelvic to the lumbar region.

Torsion of the ureter leads to the development of obstructive-retention processes in the kidney, the development of hydronephrosis and chronic pyelonephritis. Excretory urography, multislice CT, MRI, and, if necessary, retrograde or antegrade percutaneous ureterography help to establish the diagnosis.

Treatment is surgical. Resection of the ureter is performed with ureteroureteroanastomosis or ureterocystoanastomosis.

Abnormalities of the ureter structure

Hypoplasia ureter is usually combined with hypoplasia of the corresponding kidney or half of it when doubling, as well as with a multicystic kidney. With this anomaly, the lumen of the ureter is sharply narrowed or obliterated, the wall is thinned, peristalsis is weakened, and the orifice is reduced in size. Diagnosis is based on data from cystoscopy, excretory urography and retrograde ureterography.

Neuromuscular dysplasia ureter was described under the name “mega-ureter” by J. Goulk in 1923 as a congenital disease manifested by dilation and elongation of the ureter (by analogy with the term “megacolon”). This is one of the most common and severe malformations of the ureters, caused by underdevelopment or complete absence of its muscle layer and impaired innervation. As a result, the ureter is not capable of active contractions and loses its function of moving urine from the pelvis to the bladder. Over time, this kind of dynamic obstruction leads to even greater expansion and lengthening with the formation of geniculate kinks (megadolichoureter). The deterioration of urine transport is facilitated by the normal tone of the detrusor of the bladder and the combination of this anomaly with other developmental defects (ectopic ureteric orifice, ureterocele, vesicoureteropelvic reflux, neurogenic bladder dysfunction). Frequent addition of infection against the background of urostasis contributes to the development of chronic ureteritis with subsequent scarring of the ureteral wall and an even greater decrease in the function of the upper urinary tract. Characteristic histological signs of megaureter are significant underdevelopment of the neuromuscular structures of the ureter with a predominance of scar tissue.

Achalasia ureter is neuromuscular dysplasia of its pelvic region. Underdevelopment of the ureter with this anomaly is local in nature and does not affect its overlying sections, where they are little changed or developed normally. From these positions, achalasia of the ureter should be considered not a stage of development of the megaureter, but one of its varieties. As a rule, the expansion of the ureter in its pelvic section with achalasia remains at the same level throughout life. In some cases, the overlying parts of the ureter may be involved

Rice. 5.30. Excretory urogram. Achalasia of the left ureter

into the pathological process secondary to stagnation of urine in the dilated cystoid, that is, dynamic obstruction.

The clinical picture of neuromuscular dysplasia of the ureter depends on the degree of its severity. With unilateral achalasia or megaureter, the general condition remains satisfactory for a long time. Symptoms are mild or absent, which is one of the reasons for the late diagnosis of neuromuscular dysplasia already in adulthood. The first signs of megaureter are caused by the addition of chronic pyelonephritis. Pain appears in the corresponding lumbar region, fever with chills, and dysuria. A severe clinical course is observed with bilateral megaureter. From an early age, symptoms of chronic renal failure are detected.

sufficiency: child’s retardation in physical development, decreased appetite, polyuria, thirst, weakness, fatigue.

Diagnosis of neuromuscular dysplasia is based on laboratory, radiation, urodynamic and endoscopic research methods. Sonography allows us to detect the expansion of the pyelocaliceal system and the ureter in its pelvic and prevesical sections, and a decrease in the layer of renal parenchyma. A characteristic sign of achalasia on excretory urograms is a significant expansion of the pelvic ureter with unchanged overlying parts of the urinary tract (Fig. 5.30).

With megaureter, there is an increase in length and a significant expansion of the ureter along its entire length with areas of knee-shaped bends. Antegrade pyeloureterography makes it possible to establish a diagnosis in the absence of renal function according to excretory urography.

In differential diagnosis, megaureter should be distinguished from hydroureteronephrosis, which occurs as a result of narrowing of the ureter.

Surgical treatment of ureteral neuromuscular dysplasia largely depends on the stage of the disease. Over 100 methods of surgical correction have been proposed. The degree of compensatory capabilities, especially characteristic of young children, depends on the severity of anatomical and functional disorders, the diameter of the ureter and the activity of the pyelonephritic process. Surgical treatment consists of resection of the enlarged ureter along the length and width with submucosal implantation

it into the bladder according to Politano-Leadbetter. More pronounced changes in the wall of the ureter with significant impairment of its function are an indication for intestinal ureteroplasty (Fig. 54, 55, see color insert).

Congenital narrowing (stenosis) of the ureter, as a rule, it is localized in its periplochanal, less often - prevesical sections, as a result of which hydronephrosis or hydroureteronephrosis develops. Due to the frequency, characteristics of etiology, pathogenesis, clinical course and methods of surgical correction, hydronephrotic transformation is identified as a separate nosological form and is discussed in Chapter 6.

Ureteral valves- these are local duplications of the mucous and submucosal or, less commonly, all layers of the ureteral wall. This anomaly is extremely rare. The reason for its formation is a congenital excess of the ureteral mucosa. The valves can have an oblique, longitudinal, transverse direction and are more often localized in the pelvic or prevesical sections of the ureter. They can cause obstruction of the ureter with the development of hydronephrotic transformation, which is an indication for surgical treatment - resection of the narrowed section of the ureter with anastomosis between the unchanged parts of the urinary tract.

A ureteral diverticulum is a rare anomaly that manifests itself as a sac-like protrusion of its wall. The most common diverticula are the right ureter, with a predominant localization in the pelvic region. Bilateral ureteral diverticula have also been described. The wall of the diverticulum consists of the same layers as the ureter itself. The diagnosis is established on the basis of excretory urography, retrograde ureterography, spiral CT and MRI. Surgical treatment is indicated for the development of hydroureteronephrosis as a result of obstruction of the ureter in the area of ​​the diverticulum. It consists of resection of the diverticulum and the ureteral wall with ureteroureteroanastomosis.

Ureterocele- cyst-like expansion of the intramural part of the ureter with its protrusion into the lumen of the bladder (Fig. 15, see color insert). It is a common anomaly and is diagnosed in 1-2% of patients of all age groups subjected to cystoscopic examination.

Ureterocele may be one- And bilateral. The reason for its formation is congenital neuromuscular underdevelopment of the submucosal layer of the intramural part of the ureter in combination with the narrowness of its mouth. As a result of this malformation, the mucous membrane of this section of the ureter gradually shifts (stretches) into the cavity of the bladder with the formation of a round or pear-shaped cyst of various sizes. Its outer wall is the mucous membrane of the bladder, and the inner wall is the mucous membrane of the ureter. At the apex of the ureterocele there is a narrowed orifice of the ureter.

There are two types of this ureteral anomaly - orthotopic And heterotopic (ectopic) ureterocele. The first occurs when the ureteral orifice is in a normal position. It is small in size, contracts well and, as a rule, does not interfere with the outflow of urine from the kidneys. This asymptomatic ureterocele is more often diagnosed in adults. Heterotopic ureterocele occurs when there is low ectopia of the ureteric orifice towards the outlet.

Bladder. In young children, in 80-90% of cases, an ectopic type of ureterocele is diagnosed, most often the lower orifice, with duplication of the ureter. The unilateral form predominates, less often the disease is detected on both sides.

Ureterocele causes disruption of urine passage, which gradually leads to the development of hydroureteronephrosis. A common complication of ureterocele is the formation of stones in it.

Clinical symptoms depend on the size and location of the ureterocele. The larger the ureterocele and the more severe the ureteral obstruction, the earlier and more pronounced the symptoms of this anomaly appear. Pain appears in the corresponding lumbar region, when a stone forms in it and an infection attaches - dysuria. With large ureteroceles, difficulty urinating may occur due to obstruction of the bladder neck. In women, ureterocele may prolapse beyond the urethra.

The main place in diagnosis is given to radiation research methods and cystoscopy. A characteristic sign on sonography is a rounded hypoechoic formation in the area of ​​the bladder neck, above which an enlarged ureter can be identified (Fig. 5.31, 5.32).

On excretory urograms, CT with contrast and MRI, ureterocele and hydroureteronephrosis of varying severity are visualized (Fig. 5.33).

Cystoscopy is the main method for diagnosing ureterocele (Fig. 15, see color insert). With its help, you can confidently confirm the diagnosis of this anomaly, establish the type of ureterocele, its size, and the side of the lesion. A ureterocele is defined as a round formation located in the triangle of the bladder, at the apex of which the orifice of the ureter opens; when urine is released, the ureterocele contracts and decreases in size (collapses).

Orthotopic ureterocele of small size without disturbance of urodynamics does not require treatment. The type of surgical intervention is determined taking into account the size and location of the ureterocele, as well as the degree

Rice. 5.31. Transabdominal sonogram. Left ureterocele (arrow)

Rice. 5.32. Transrectal sonogram. Large ureterocele (1) with significant dilation of the ureter (2)

Rice. 5.33. Excretory urograms at the 7th (a) and 15th (b) minutes of the study. Ureterocele (1) on the right with dilatation of the ureter (2) (hydroureteronephrosis)

hydronephrotic transformation. Depending on this, transurethral endoscopic resection of the ureterocele or its open resection with ureterocystoanastomosis are used.

Vesicoureteropelvic reflux (VUR)- the process of retrograde reflux 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 primary And secondary. Primary VUR occurs as a result of congenital incompetence (incomplete maturation) of the vesicoureteral junction. Secondary - is a complication of bladder outlet obstruction, developing due to increased pressure in the bladder.

PMR may be active And passive. In the first case, it occurs at the moment of urination with a maximum increase in intravesical pressure, in the second, it can be observed at rest.

A characteristic clinical manifestation of VUR is the occurrence of pain in the lumbar region when urinating. When an infection occurs, symptoms of chronic pyelonephritis appear.

In the diagnosis of PMR, the leading role is occupied by X-ray radionuclide research methods. Retrograde cystography at rest and during urination (void cystography) reveals not only its presence, but also the severity of the anomaly (see Chapter 4, Fig. 4.32).

Conservative treatment is possible in the initial stages of the disease; surgical treatment consists of performing various antireflux surgeries.

tions, of which the simplest is the endoscopic submucosal introduction into the area of ​​the mouth of formative bioimplants (silicone, collagen, Teflon paste, etc.) that prevent the reverse flow of urine. Surgeries to reconstruct the ureteral orifice have found widespread use, and are currently performed, among other things, using robot-assisted technology.

5.3. BLADDER ANOMALIES

The following malformations of the bladder are distinguished:

■ abnormalities of the urinary duct (urachus);

■ agenesis of the bladder;

■ bladder duplication;

■ congenital bladder diverticulum;

■ exstrophy of the bladder;

■ congenital contracture of the bladder neck.

Urachus(urachus)- the urinary duct, which connects the developing bladder through the umbilical cord with the amniotic fluid during the period of intrauterine development of the fetus. Usually by the time the baby is born it is overgrown. With developmental defects, the urachus may not heal completely or partially. Depending on this, anomalies of the urachus are distinguished.

Umbilical fistula- non-closure of part of the urachus, opening as a fistula in the navel and not communicating with the bladder. Constant discharge from the fistula leads to irritation of the skin around it and infection.

Vesico-umbilical fistula- complete nonfusion of the urachus. In this case, there is a constant release of urine from the fistula.

Urachal cyst- non-closure of the middle part of the urinary duct. This anomaly is asymptomatic and appears only when large in size or suppuration. In some cases, it can be felt through the anterior abdominal wall.

Diagnosis of urachal anomalies is based on the use of ultrasound, X-ray (fistulography) and endoscopic (cystoscopy with the introduction of methylene blue into the fistulous tract and detection of it in the urine) research methods. Surgical treatment consists of excision of the urachus.

Bladder agenesis- its congenital absence. An extremely rare anomaly, which is usually combined with developmental defects that are incompatible with life.

Bladder duplication- also a very rare anomaly of this organ. It is characterized by the presence of a septum that divides the bladder cavity into two halves. The mouth of the corresponding ureter opens into each of them. This anomaly may be accompanied by duplication of the urethra and the presence of two bladder necks. Sometimes the septum may be incomplete, and then a “two-chamber” bladder occurs (Fig. 5.34).

Congenital bladder diverticulum- saccular protrusion of the bladder wall outward. As a rule, it is located on the posterolateral wall of the bladder near the mouth, slightly above and lateral to it.

Rice. 5.34. Bladder duplication: A- complete; b- incomplete

The wall of a congenital (true) diverticulum, in contrast to an acquired one, has the same structure as the wall of the bladder. Acquired (false) diverticulum develops due to bladder outlet obstruction and increased pressure in the bladder. As a result of overstretching of the bladder wall, it becomes thinner with protrusion of the mucous membrane between the bundles of hypertrophied muscle fibers. Constant stagnation of urine in the diverticulum contributes to the formation of stones and the development of chronic inflammation.

Characteristic clinical symptoms of this anomaly are difficulty urinating and emptying the bladder in two stages (first the bladder is emptied, then the diverticulum).

The diagnosis is made on the basis of ultrasound (Fig. 5.35), cystography (Fig. 5.36) and cystoscopy (Fig. 20, see color insert).

Surgical treatment consists of excision of the diverticulum and suturing of the resulting defect in the bladder wall.

Bladder exstrophy- severe malformation, consisting in the absence of the anterior wall of the bladder and the corresponding part of the anterior abdominal wall (Fig. 40, see color insert). This anomaly is more often observed in boys and occurs in 1 out of 30-50 thousand newborns. Exstrophy of the bladder is often combined with malformations of the upper and lower urinary tracts, prolapse

Rice. 5.35. Transabdominal sonogram. Diverticulum (1) of the bladder (2)

Rice. 5.36. Descending cystogram. Bladder diverticula

in the rectum, in boys - with epispadias, inguinal hernia, cryptorchidism, in girls - with anomalies of the uterus and vagina.

With such an anomaly, urine constantly pours out, which subsequently leads to maceration and ulceration of the skin of the perineum, genitals and thighs. When a child strains (when laughing, screaming, crying), the wall of the bladder protrudes into a ball, and urine output increases. The mucous membrane is hyperemic and bleeds easily. In the lower corners of the defect, the orifices of the ureters are determined. Exstrophy of the bladder, as a rule, is combined with diastasis of the bones of the symphysis pubis, which is manifested by a “duck” gait. Constant contact of the mucous membrane of the bladder and urethra with the 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 surgery aimed at closing the defect of the bladder and abdominal wall with one’s own tissues;

■ transplantation of the bladder triangle along with the orifices into the sigmoid colon (currently performed extremely rarely);

■ formation of an artificial orthotopic urinary reservoir from a section of the ileum.

Bladder neck contracture- a developmental defect characterized by excessive development of connective tissue in a given anatomical area. The clinical picture depends on the severity of fibrotic changes in the bladder neck and associated urination disorders. Diagnosis of this anomaly is based on the results of instrumental examination (uroflowmetry in combination with cystomanometry), urethrography and urethrocystoscopy with biopsy of the bladder neck. Endoscopic treatment involves cutting or excision of scar tissue.

5.4. ANOMALIES OF THE URETHER CHANNEL

Malformations of the urethra include:

■ hypospadias;

■ epispadias;

■ congenital valves, obliterations, strictures, diverticula and urethral cysts;

■ hypertrophy of the seminal tubercle;

■ duplication of the urethra;

■ urethro-rectal fistulas;

■ prolapse of the urethral mucosa.

Hypospadias- congenital absence of a section of the anterior urethra with replacement of the missing part by a dense connective tissue cord (chord) and curvature of the penis back towards the scrotum. This anomaly occurs with a frequency of 1: 250-300 newborns. In fact, hypospadias is combined with an abnormal structure of the penis. It is, as a rule, anatomically underdeveloped, small, thin, and strongly curved in the dorsal direction. The bend is especially pronounced during an erection. The angle of curvature can be so great that sexual activity becomes impossible. Usually the foreskin is split and covers the glans in the form of a hood. Meatostenosis may occur.

Highlight capitate(most common), coronoid, stem, scrotal And perineal hypospadias. The first two forms are the easiest and differ little from each other. They are characterized by the location of the external opening of the urethra at the level of the head or coronary sulcus and a slight curvature of the penis.

The stem form is characterized by the location of the external opening of the urethra in different parts of the penis. The more proximally it is ectopic, the more pronounced the curvature of the organ. Due to the bending and meatostenosis, emptying the bladder is difficult, the stream is weak and directed downward.

The most severe are scrotal and perineal forms of hypospadias. They are characterized by severe underdevelopment and curvature of the penis and severe urination disturbance, which is only possible in a sitting position. Newborns with scrotal hypospadias are sometimes mistaken for girls or false hermaphrodites.

A separate form is the so-called “hypospadias without hypospadias,” in which the external opening of the urethra is in its usual place on the head of the penis, but it itself is significantly shortened. Between the shortened urethra and the normal-length penis there is a dense connective tissue cord (chord), which makes the penis sharply curved in the dorsal direction.

Female hypospadias, in which the posterior wall of the urethra and the anterior wall of the vagina are split, is extremely rare. It may be accompanied by stress urinary incontinence.

The diagnosis of hypospadias is established by objective examination. In some cases, it can be difficult to distinguish scrotal and perineal hypospadias from female false hermaphroditism. In such cases, it is necessary to determine the genetic sex of the child. Radiation methods make it possible to identify the presence and type of structure of the internal genital organs.

Surgical treatment is indicated for all forms of this anomaly and is performed in the first years of a child’s life. For capitate and coronal hypospadias

The operation is performed in case of significant curvature of the glans penis and/or meatostenosis. To correct more severe forms of hypospadias, many different surgical treatment methods have been proposed. All of them are aimed at achieving two main goals: creating the missing part of the urethra with the formation of its external opening in a normal anatomical position and straightening the penis by excision of connective tissue scars (chords). The prognosis for timely plastic surgery is favorable. A good cosmetic effect, normal urination, and preservation of sexual and reproductive function are achieved.

Epispadias- congenital clefting along the anterior surface of all or part of the urethra. This section of the urethra, open anteriorly, together with the cavernous bodies, creates a characteristic groove running along the dorsal part of the penis. This anomaly is much less common than hypospadias, and on average is detected in 1 out of 50 thousand newborns. The ratio between boys and girls is 3:1.

In boys, there are three types of epispadias: capitate, stem And total. Epispadias of the glans penis characterized by the fact that the anterior wall of the urethra is split to the coronary groove. The penis is slightly curved and raised upward. Urination and erection with this form of epispadias are usually not impaired.

Stem form characterized by the fact that the anterior wall of the urethra is split throughout the entire penis - to the area where the skin transitions to the pubic region. With this form of epispadias, there is a splitting of the pubic symphysis, and sometimes a separation of the abdominal muscles. The penis is shortened and curved towards the anterior abdominal wall. The opening of the urethra is shaped like a funnel. When urinating, the stream is directed upward, urine splashes, which leads to wet clothes. Sexual life is impossible, since the penis is small in size and is strongly curved during erection.

Total (complete) epispadias in addition to clefting of the anterior wall of the urethra, it is characterized by clefting of the bladder sphincter. The urethra has the shape of a funnel and is located immediately under the womb. This form is characterized by urinary incontinence due to underdevelopment of the bladder sphincter. Constant leakage of urine leads to skin irritation in the scrotum and perineum, dermatitis develops, and the child’s normal social adaptation in the society of his peers is disrupted. There is underdevelopment of the penis and scrotum.

Epispadias is less common in girls than in boys. There are three forms of it. Clitoral epispadias, characterized only by clefting of the clitoris. The external opening of the urethra is shifted upward and opens above it. Urination is not impaired.

At subsymphyseal form There is clefting of the urethra to the neck of the bladder and clefting of the clitoris. The most severe is complete epipadias, in which the anterior wall of the urethra and the neck of the bladder are absent, and the external opening of the urethra is located behind the pubic symphysis. There is a splitting of the symphysis pubis and sphincter of the bladder, which is manifested by a duck's gait and urinary incontinence.

In most patients with epispadias, bladder capacity is reduced, and VUR is observed.

Surgical treatment of epispadias is carried out in the first years of life. It consists of reconstructing the urethra and eliminating the curvature of the penis.

Congenital urethral valves- the presence in its proximal section of pronounced folds of the mucosa, protruding into the lumen of the urethra in the form of bridges. This anomaly is more common in boys and accounts for 1 in 50 thousand newborns. Urethral valves disrupt normal urination, make it difficult to empty the bladder, lead to the appearance of residual urine, the development of hydroureteronephrosis and chronic pyelonephritis. Endoscopic treatment of urethral valves. Their TOUR is being carried out.

Congenital obliteration of the urethra It is extremely rare and is always combined with other anomalies, often incompatible with life.

Congenital urethral stricture- a rare anomaly in which there is a cicatricial narrowing of its lumen, leading to urination disorders.

Congenital urethral diverticulum- also a rare malformation consisting in the presence of a sac-like protrusion of the posterior wall of the urethra. Most often localized in the anterior urethra. It manifests itself as dysuria and the release of drops of urine after the end of urination. The diagnosis is established on the basis of urethrography and urethroscopy, mictional cystoureterography. Treatment consists of excision of the diverticulum.

Congenital urethral cysts develop as a result of obliteration of the outlet openings of the bulbourethral glands. They are predominantly localized in the area of ​​the urethral bulb. They are removed surgically.

Hypertrophy of the spermatic tubercle- congenital hyperplasia of all elements of the seminal tubercle. Causes urethral obstruction and erection during urination. Diagnosed by urethroscopy and retrograde urethrography. Treatment consists of TUR of the hypertrophied part of the seminal tubercle.

Duplication of the urethra- a rare developmental defect. It can be complete or incomplete. Full doubling combined with doubling of the penis. More common incomplete duplication of the urethra. In most cases, the accessory urethra ends blindly. The accessory urethra always has an underdeveloped corpus cavernosum.

Urethro-rectal fistulas- a rare malformation that is almost always combined with anal atresia. Occurs as a result of underdevelopment of the urinary rectal septum.

Prolapse of the urethral mucosa- also a rare anomaly. The prolapsed mucosa due to impaired microcirculation has a bluish tint and sometimes bleeds. Treatment is surgical.

5.5. ANOMALIES OF THE MALE GENITAL ORGANS

Testicular abnormalities

Testicular malformations are divided into abnormalities of number, structure and position. Quantity anomalies include:

Anorchism- congenital absence of both testicles. Combined with underdevelopment of other genital organs. Differential diagnosis with bilateral abdominal cryptorchidism is carried out on the basis of testicular scintigraphy, CT, MRI and laparoscopy. Treatment consists of hormone replacement therapy.

Monorchism- congenital absence of one testicle, its epididymis and the vas deferens. It should be distinguished from unilateral abdominal creep-torchidism using similar diagnostic methods as for anorchism. For cosmetic purposes, testicular replacement is possible.

Polyorchidism- an extremely rare anomaly characterized by the presence of an accessory testicle. It is located next to the main one, is usually underdeveloped and, as a rule, does not have an appendage and a vas deferens. Due to the high risk of malignancy, it is advisable to remove it.

Synorchism- congenital fusion of both testicles that have not descended from the abdominal cavity. Treatment is surgical. They are separated and brought down into the scrotum.

Structural anomalies include testicular hypoplasia- his congenital underdevelopment. It is diagnosed by objective examination (sharply reduced testicles are palpated in the scrotum), using radiation and radionuclide research methods. In treatment, especially with a bilateral process, hormone replacement therapy is used.

Abnormalities of the position of the testicles include:

Cryptorchidism- a developmental defect (from the Greek kriptos - hidden and orchis - testicle), in which there is undescendancy of one or both testicles into the scrotum. Hour-

The incidence of cryptorchidism in full-term newborn boys is 3%, and in premature infants it increases 10 times. Cryptorchidism in 25-30% of cases is combined with anomalies of other organs.

Abnormal position of the testicle leads to its anatomical and functional failure, including atrophy. The cause of the most significant complication of cryptorchidism - infertility - is a change in the temperature regime of the testicle. Spermatogenic function is significantly impaired even with a slight increase in its temperature. In addition, the risk of malignancy in an undescended testicle, in contrast to a normally located testicle, increases significantly.

Depending on the degree of undescendedness, they distinguish abdominal And inguinal forms of cryptorchidism

Rice. 5.37. Forms of cryptorchidism and testicular ectopia:

1 - normally located testicle; 2 - retention of the testicle before entering the scrotum; 3 - inguinal ectopia; 4 - inguinal creep-torchidism; 5 - abdominal cryptorchidism; 6 - femoral ectopia

Rice. 5.38. CT scan of the pelvis. Abdominal cryptorchidism (1). The left testicle is located in the abdominal cavity next to the bladder (2)

(Fig. 5.37). This developmental defect may be one-sided And two-sided, true And false. False (pseudocryptorchidism) is noted when the testicle is excessively mobile, when it is a result of contraction of the levator testis muscle (ie cremaster), tightly pulled to the external inguinal ring or even immersed in the inguinal canal. In a relaxed state, it can be brought down into the scrotum with gentle movements, but it often returns back.

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

testicular scintigraphy and laparoscopy. Based on the same methods, cryptorchidism is differentiated from anorchism, monorchidism and ectopic testis.

Conservative treatment is indicated when the testicle is located in the distal part of the inguinal canal. Hormonal therapy with human chorionic gonadotropin is used. Surgical treatment is carried out in the first years of a child’s life if hormonal therapy is ineffective. It consists of opening the inguinal canal, mobilizing the testicle, spermatic cord and lowering it into the scrotum with fixation in this position (orchiopexy).

Ectopic testicle- a congenital malformation in which it is located in various anatomical areas, but not along its embryonic path to the scrotum. This anomaly differs from cryptorchidism in this way. Depending on the location of the testicle, there are inguinal, femoral, perineal And cross ectopia (see Fig. 5.37). Surgical treatment involves bringing the testicle down into the corresponding half of the scrotum.

The prognosis for testicular development with cryptorchidism and ectopia is favorable if the operation is performed in the first years of the child’s life.

Penile abnormalities

Congenital phimosis- congenital narrowing of the opening of the foreskin, which does not allow exposure of the head of the penis. Up to 3 years of age, physiological phimosis is registered in most cases in boys. In case of pronounced narrowing of the foreskin, they resort to its circular excision (circumcision).

Hidden penis- an extremely rare anomaly in which the normally developed corpora cavernosa are hidden by the surrounding tissues of the scrotum and skin of the pubic area. The penis is usually reduced in size; the corpora cavernosa are determined only by palpation in the folds of the surrounding skin.

Ectopia of the penis- an extremely rare anomaly in which it is small in size and located behind the scrotum. Surgical treatment: the penis is moved to its normal position.

Double penis (diphallia)- also a rare developmental defect. Doubling can be full, when there are two penises with two urethra, and incomplete- two penises with a urethral groove on the surface of each. Surgical treatment involves removing one of the less developed penises.

Casuistry is agenesis of the penis, which, as a rule, is combined with other anomalies incompatible with life.

Control questions

1. Give a classification of kidney anomalies.

2. What is the difference between multicystic kidney disease and polycystic kidney disease?

3. What should be the treatment tactics for a simple kidney cyst?

4. What types of ectopia of the ureteral orifice exist?

5. What is the essence of the Weigert-Meyer law?

6. What is the clinical significance of ureterocele?

7. What anomalies of the urachus are found?

8. List the types of hypospadias.

9. Give the forms of cryptorchidism and ectopia of the testicle.

10. What abnormalities of the penis do you know? What is congenital phimosis?

Clinical task 1

A 50-year-old patient complained of recurring dull pain in the left flank of the abdomen and lumbar region. Over the past months, I began to independently palpate an elastic, round, easily displaced formation in the left hypochondrium. At the same time, he did not notice any increase in body temperature, urination problems, changes in the color of urine or other symptoms. I contacted my family doctor. His ordered laboratory tests were normal, after which a multislice CT scan of the abdomen was performed (Fig. 5.39).

What diagnosis will you make? Are other research methods needed to confirm it? Which treatment method should I choose?

Clinical task 2

A 25-year-old patient complained of dull pain in the left lumbar region and frequent painful urination. Such phenomena have been observed for several months. The kidneys are not detected by palpation. Blood and urine tests are unchanged. Ultrasound revealed a round shape of small size

Rice. 5.39. Multislice CT scan of the kidneys with contrast in a 50-year-old patient

Rice. 5.40. Excretory urogram of a 25-year-old patient. The left ureter ends in a club-shaped extension

hypoechoic formation in the bladder neck. The patient underwent an excretory urogram (Fig. 5.40).

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

Clinical task 3

The parents of a 9-month-old boy consulted a urologist with a complaint that the child had no left testicle in the scrotum. According to them, the boy was born premature and the testicle was missing from the moment of birth. An objective examination revealed that the external opening of the urethra is located in a typical manner, the folding of the scrotum is preserved. The right testicle is identified in its usual place, the left one is palpated in the center of the inguinal canal.

5510 0

Polycystic kidney disease of infantile type(polycystic kidney disease type I Potter syndrome, autosomal recessive polycystic kidney disease) is a bilateral symmetrical enlargement of the kidneys due to the replacement of parenchyma by secondarily dilated collecting ducts without proliferation of connective tissue.

The disease occurs with a frequency of 2:110,000 newborns and is inherited in an autosomal recessive manner. It is based on a primary defect of the collecting ducts. The renal pelvis, calyces and papillae remain intact, since there is no defect in the ureteric bud. Combined anomalies are rare.

Diagnostics: Ultrasound examination reveals bilateral enlargement of the kidneys against the background of oligohydramnios and the absence of echotenosis of the bladder. Typical renal hyperechogenicity is accompanied by increased sound conductivity due to multiple microscopic cystic structures in the renal parenchyma.

Forecast unfavorable. Death of newborns occurs from renal failure.

Obstetric tactics: termination of pregnancy at any stage.

Adult-type polycystic kidney disease(autosomal dominant disease, hepatorenal polycystic disease of adult type, type III Potter syndrome) is characterized by the replacement of the renal parenchyma with numerous cysts of different sizes, which are formed due to the expansion of the collecting ducts and other tubular segments of the nephron. Cysts coexist with intact renal tissue. The kidneys are affected on both sides and are enlarged, but a unilateral process may be the first manifestation of the disease. Involvement of the liver in the process is observed less frequently and is expressed in periportal fibrosis, which is focal in nature.

Etiology unknown. The type of inheritance causes a 50% risk of developing the disease. Its genetic focus is located on the 16th pair of chromosomes.

Frequency: one in 1000 people carries the mutant gene; penetration of the gene occurs in 100% of cases, but its expressiveness can vary from severe forms leading to death in the neonatal period to asymptomatic forms detected at autopsy.

Clinically, polycystic adult type is combined with cystic changes in the liver, pancreas, spleen, lungs, ovaries and epididymis.

Prenatal diagnosis can be carried out by chorionic villus sampling and examination of the biopsy using a special genetic probe to detect the mutant gene locus. Single observations of ultrasound diagnostics of this pathology are described.

Forecast: polycystic kidney disease is a chronic disease; its first symptoms can appear at any age. The average age of onset of clinical manifestations (lumbar pain, enlarged kidneys, renal failure and uremia) is 35 years. Hypertension is recorded in 50-70% of patients.

Obstetric tactics: parents at risk should be informed about the possibility of diagnosing pathology in the first trimester of pregnancy and terminating pregnancy in the early stages; Once the fetus reaches viability, standard obstetric tactics are generally accepted.

Multicystic kidney disease(multycystic kidney disease, cystic kidney disease type II Potter syndrome, dysplastic kidney disease) is a congenital renal anomaly manifested by cystic degeneration of the renal parenchyma due to primary dilatation of the renal tubules. The process can be bilateral, unilateral or segmental.

The disease occurs mainly sporadically and can be a secondary manifestation as part of other syndromes:

• autosomal recessive (Meckel syndrome, Dandy-Walker syndrome, Roberts syndrome, Smith-Lemli-Opitz syndrome, etc.);
• autosomal dominant (Apert syndrome);
• chromosomal disorders.

A bilateral process occurs in one in 10,000 newborns. A unilateral process is observed 2 times more often in male newborns.

The pathogenetic mechanisms of the disease are unknown. It is believed that this complex anomaly may be the result of two types of damage: failure of the metanephric blastema, responsible for the formation of nephrons, and early obstructive uropathy.

Unilateral lesions can be combined with neural tube defects, diaphragmatic hernia, cleft palate, and gastrointestinal abnormalities.

Prenatal diagnosis is based on the detection of cystic kidneys by ultrasound, insufficient visualization of the bladder, even after a test with furosemide.

Forecast: a bilateral process, in most cases, leads to death.

Obstetric tactics: in case of a bilateral process diagnosed in the early stages of pregnancy, termination of pregnancy is recommended; a unilateral process with a normal karyotype and without associated anomalies does not affect obstetric tactics. Delivery is recommended to take place in a perinatal center.

Hydronephrosis is the result of obstruction of the urinary tract at the junction of the renal pelvis and ureter.

Its frequency has not been established, since the anomaly is a sporadic phenomenon. After birth, it is diagnosed 5 times more often in males.

In children with obstruction of the ureteropelvic junction, combined anomalies of the urinary tract are detected in 27% of cases, including vesicoureteral reflux, bilateral duplication of the ureters, bilateral obstructive megaureter, non-functioning contralateral kidney, agenesis of the contralateral kidney. In 19%, anomalies of other organs and systems are noted.

Obstruction of the ureteropelvic junction is diagnosed using echography, based on the detection of dilatation of the renal pelvis.

With a unilateral process, the prognosis is favorable. Obstetric tactics depend on the time of detection of the pathological process and the degree of renal dysfunction.

Selected lectures on obstetrics and gynecology

Ed. A.N. Strizhakova, A.I. Davydova, L.D. Belotserkovtseva

Malformations of the ureters account for 22-25% of all anomalies of the urinary system and 4.2-5% of lesions of the urinary organs. Some of these anomalies are discovered by chance. Others can cause severe impairment of kidney function, promoting urinary stasis and fixation of infection in the kidneys.

Numerous anomalies in the number, shape, location and structure of the ureter lead to disruption of the outflow of urine from the kidney. Urodynamics are disturbed not only in the presence of anatomically pronounced obstacles, but in almost all malformations of the upper urinary tract, even with unnoticeable obstacles to the outflow of urine. More often, congenital dilatation or narrowing of the ureter is observed.

Anomalies of the ureters are often multiple, bilateral, and lead to changes in the renal parenchyma. The more severe the anomaly, the earlier it manifests itself and is diagnosed.

All malformations of the ureters lead to impaired renal function, in particular urodynamics. Any disturbances in the formation of the ureteric sprout manifest themselves in the morphogenesis of the renal parenchyma, since a full-fledged nephron can only be formed under the condition of normal development of both the metanephrogenic blastema and the distal parts of the collecting ducts, calyces, pelvis, etc. Anomalies in the quantity of the ureter and renal pelvis are combined with abnormalities in the renal parenchyma.

According to the generally accepted classification, ureteral anomalies are divided into the following groups:

quantity anomalies - aplasia, doubling, tripling (complete or incomplete), etc.;

structural anomalies - hypoplasia, narrowing (stricture), valve, diverticulum, ureterocele, neuromuscular dysplasia, as well as achalasia, megaureter, hydroureteronephrosis;

shape anomalies - ring-shaped, corkscrew-shaped ureter;

location anomalies-retrocaval ureter, retroiliac ureter, ectopic ureteric orifice.

Clinical manifestations of various ureteral anomalies depend not so much on its morphological characteristics, but on the complications caused by developmental disorders. The main complications are inflammatory diseases, hydronephrosis, stone formation, nephrogenic hypertension, which often occur against the background of pyelonephritis. Infection due to abnormalities of the ureter most often occurs in early childhood. Its course can be active, or with latent periods, periodic exacerbations. More often, the infectious process is cyclical in nature due to intercurrent diseases or increased stress on the body (years of intensive growth, puberty, pregnancy). In rare cases, even pronounced anomalies are not accompanied by complications for a long time and the infection appears only in adulthood.

Abnormalities of the ureter often predetermine the development of hydronephrosis or ureterohydronephrosis. Depending on the degree of patency of the ureter and the level of obstruction, loss of function or its compensation is observed due to hypertrophy and increased contractility of the ureter in sections located above the level of the anomaly. Patients often develop progressive hydronephrosis, which leads to kidney failure.

If the outflow of urine is disrupted in the lower parts of the urinary tract, compensation processes last longer and hydronephrosis progresses more slowly. A certain role, in addition to hypertrophy of the ureteral wall over the obstacle, is also played by an increase in the reabsorption field, which is not limited to reflux at the level of the kidney, but appears throughout the entire ureter.

In the case of numerous anomalies of the ureter, conservative measures are very rarely limited. The threat of kidney death forces one to resort to surgical interventions, the consequences of which depend on the age of the child. The earlier the operation is performed, the greater the hope for functional compensation of the kidneys, the more favorable prognosis when combined with treatment with antibacterial agents, which allows achieving clinical and laboratory remission of the inflammatory process in only 50% of children. This is explained by the fact that most anomalies, except for urodynamic disorders, are accompanied by improper formation of renal tissue and immaturity of the immune system. After restoration of urodynamics, these changes remain the direct cause of maintaining the inflammatory process.

In the diagnosis of the main malformations of the ureters, the main role is played by urological methods - variants of excretory urography. Ascending pyelography is relatively rarely used. This study is performed only in cases where the ureteral anomaly causes complete loss of kidney function.

Cystoscopy is of particular importance, as it allows one to determine the location of the ureteric orifice, its contractility, shape, and length of the intravesical part. The high incidence of vesicoureteral reflux in all ureteral anomalies makes it possible to replace ascending pyelography with conventional voiding cystography.

Important information is provided by methods based on the capabilities of television observation and video recording of contraction of the ureters during excretory urography. Renal angiography, pharmacodiagnostic, immuno-morphological and cystochemical research methods make it possible to assess the morpho-functional state of the renal tissue, outline a treatment plan for the patient, avoid diagnostic errors, correct violations in the operation, monitor the further course of the disease and predict possible consequences.

QUANTITY ANOMALIES

Aplasia (agenesis) of the ureter It is very rare and accounts for 0.2% of kidney and urinary tract anomalies. Bilateral anomaly is usually combined with bilateral renal agenesis, less often with bilateral multicystic kidney. This anomaly has no clinical significance because it is incompatible with life.

Unilateral ureteral aplasia is also a component of renal aplasia and the result of the absence of a ureteric sprout. Sometimes the ureter is found in the form of a thin fibrous cord or process that ends blindly.

Diagnosis of ureteral aplasia is based on data from excretory urography, which makes it possible to establish the absence of function of one of the kidneys. Cystoscopy reveals hypoplasia or complete absence of half of the bladder triangle. The opening of the ureter may be located in its usual place, but be narrowed. With long-term observation, the absence of its contractions can be detected. Sometimes the hole looks like a blind depression, which is determined when the catheter is inserted, or ends blindly at any level. In these cases, cystography is quite informative. In case of a rudimentary opening of the ureter, it is recommended to perform an ultrasound examination and computed tomography.

The need for treatment arises only when the ureter ends blindly, since this anomaly can cause an inflammatory process, sometimes with suppuration (empyema), and stone formation. In the case of scarring, the peripheral opening of the ureter forms a closed cavity, resembling a cyst or tumor of the abdominal cavity.

Such complications are manifested by pain in the corresponding groin or epigastric region, dysuria, intermittent increase in body temperature, and phenomena of chronic intoxication. Urine contains a large number of leukocytes, protein, and bacteria. If a stone is present, macro- or microhematuria is detected.

Treatment consists of removing the ureteral stump.

Duplication of the ureter - one of the most numerous anomalies (1:140). It is caused by the simultaneous growth of two ureters from two ureteric shoots of the nephrogenic blastema or the splitting of a single ureteric shoot. One of the ureters may develop normally, while the other may develop pathologically. If several ureteral anlages are formed in the caudal section of the duct of the primary kidney, not only doubling, but also tripling of morphologically complete ureters is possible. The two ureters correspond to two renal pelvises, which are urine collectors for different ends of the kidney.

In such cases, the kidneys are rarely isolated. A third, additional bud is formed.

Sometimes two or more ureters extend from the pelvis of a non-duplicated kidney, or the proximal end of one of the ureters ends blindly. Both ureters usually pass through the same fascial sheath.

Complete (ureter duplex) and incomplete (ureter fissus) duplication of the ureters is observed.

With incomplete duplication, both ureters extend from the renal pelvis down to the bladder and merge into one at different distances from it. In this case, one hole appears in the bladder - a split ureter. Sometimes the ureters drain near the bladder, intravesically (intramurally), or even at the opening. One of the ureters flows into the other at an acute angle.

As a rule, the length of both ureters from the ureteropelvic segment to the confluence is different and the sections of both ureters above it are in different phases of peristalsis. With incomplete doubling, 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.

Duplication of the ureter - both complete and incomplete, is often unilateral. Localized on both sides with the same frequency.

With complete duplication, both ureters go separately to the bladder. They are closely adjacent to the walls, according to the Weigert-Meyer law, they intersect in the proximal and distal sections and open with two openings on the corresponding half of the bladder triangle (one above the other or next to each other), if there is no ectopia of one of them. In the bladder, the ureteric orifices of the superior pelvis are almost always contained below the ureteral orifices of the inferior pelvis.

Vesicoureteral reflux is more often observed with complete duplication of the ureters. This is due to the short intravesical section of the ureter, which opens more proximally. Occasionally, a blind ending of one of the duplicated ureters is observed. The anomaly is manifested by pain in the epigastric region and symptoms of the inflammatory process.

Duplication of the ureters is often combined with other malformations: the absence of both or one ureteric orifice, narrowing (ureteral stricture, ureterocele, ectopia of the opening of one of the ureters (usually the lower one), segmental or widespread dysplasia of the neuromuscular elements of the ureters, aberrant vessels, adhesions, fibrous cords, etc. .P..

With a double ureter, no characteristic symptoms are observed. For a long time, the anomaly has an asymptomatic course. Clinical manifestations occur when complications occur. Symptoms are determined by the nature and stage of the complication or associated anomalies.

The main diagnostic method is excretory urography. If the function of one half of the double kidney decreases, the necessary information can be obtained using infusion pyelography using delayed images. Radiological signs of duplication of the ureter include: absence of the upper cups in the lower pelvis; displacement of the lower pelvis downwards, outwards or rotation around the longitudinal axis; deformation of the higher located cups; the presence of a large “silent” zone between the edge of the pelvis and the end of the kidney; the occurrence of tubular pelvic-renal reflux in the area of ​​the upper cups; the presence of two renal pelvis or two ureters.

Cystoscopy can detect complete duplication of the ureter if both openings open into the bladder. The combination of cystoscopy with intravenous administration of indigo carmine makes it possible to determine the functional value of the upper and lower kidneys. Using serial voiding cystography, vesicoureteral reflux is detected.

Treatment. If duplication of the ureters is discovered by chance and the anomaly does not cause noticeable urodynamic disturbances, children do not require treatment. They are subject to observation by a pediatrician at their place of residence. If any complaints arise, consultation is required. In the case when duplication of the ureters is complicated by pyelonephritis, and uro-radiological examination fails to reveal significant urodynamic disturbances, children should be prescribed conservative anti-inflammatory treatment, stimulating, desensitizing therapy, and measures should be taken to increase immunological reactivity. If such treatment is ineffective, surgery is recommended.

Surgical intervention is required for such complications of the anomaly as the formation of stones, ureterohydronephrosis of one or both parts of the double kidney, severe vesicoureteral and interureteral reflux, ectopia of one of the openings of the double ureter, ureterocele.

The choice of treatment method depends on several factors: the degree of damage to the kidney segment, the type of ureteral duplication, the nature of changes in the terminal part of the ureter, the presence of reflux in the adjacent and contralateral ureter.

The operation of choice for ectopic orifice of the accessory ureter, segmental hydro- and ureteronephrosclerosis, vesicoureteral reflux and small ureterocele without obstruction and reflux into nearby ureters is heminefroureterectomy. In case of a large vesical or ectopic ureterocele, its membranes are removed, performing heminefrureterectomy and correction of the openings of the adjacent and contralateral ureters. If the functions of both segments of the kidney are preserved, organ-preserving operations are applicable - anastomosis of the ureters in the upper cystoid, anti-reflux operations on two ureters in one block.

To eliminate vesicoureteral reflux in case of duplication of the ureters, a Politano-Leadbetter operation is performed on one or both ureters. It is advisable to supplement heminefrureterectomy with antireflux surgery in cases where one of the kidney segments has completely lost its functional capacity, and the remaining ureter has a defective vesicoureteral orifice.

In case of complete duplication and vesicoureteral reflux in the lower segment of the kidney, ureterectomy should be used from two approaches: 1) in the lower back to remove the ureter and create a pyeloureteroanastomosis, 2) in the epigastric region to complete the ureterectomy and immerse the ureteral stump into the bladder wall.

Triplication of the ureters - a very rare anomaly. Cases of the presence of 4, 6, and even 12 ureters have been described.

Clinical manifestations, diagnosis and treatment of this anomaly are the same as for duplication of the upper urinary tract. In uncomplicated cases, the anomaly is not diagnosed. Clinically manifested in the case of an inflammatory process, ureterohydronephrosis or stone formation, as well as ectopia of one of the openings of the ureter.

ANOMALIES OF STRUCTURE AND FORM

Ureteral hypoplasia combined with hypoplasia of the corresponding kidney or its half (with doubling), multicystic kidney dysplasia. The ureter has the appearance of a thin tube with a sharply reduced diameter. It may be obliterated in some areas. More often the lumen of the ureter is preserved. Its wall is thin due to underdevelopment of muscle fibers, peristalsis is sharply weakened.

This malformation is often combined with vesicoureteral reflux. Clinical manifestations of ureteral hypoplasia are caused by concomitant renal anomalies.

Diagnosis of the anomaly is based on data from excretory urography and its infusion modification, since the hypoplastic ureter extends from the pelvis of a functioning kidney, its function is usually significantly reduced and a clear image of the urinary tract can only be achieved on delayed images.

The final diagnosis can be made using retrograde pyelography, if it is possible to insert a catheter into the ureter, or by cystography, when the malformation is complicated by vesicoureteral reflux.

Treatment depends on the condition of the corresponding kidney. When performing a nephrectomy, the ureter is also removed. Obliteration of the ureter at different levels or its blind ending due to aplasia, complications due to the inflammatory process in a diverticulum-like formation are indications for ureterectomy and removal of a non-functioning kidney.

Narrowing (stricture) of the ureter observed in 0.5-0.7% of children. Most often, the anomaly is localized in the vesicoureteral segment, then in the ureteropelvic segment, but can be observed in any part of the ureter.

The narrowing of the ureter can be unilateral or bilateral, single or multiple. In most cases, it refers to a congenital pathology, but it can also be acquired (as a result of trauma, damage during instrumental examinations, bedsores or inflammation due to prolonged presence of a stone in the ureter). A narrowing may develop after surgery on the ureter. If there is no history of any of these reasons, we can assume that the narrowing is congenital. Congenital and acquired narrowings are clinically and histologically indistinguishable.

Above the narrowing of the ureter and the collecting system expands due to a constant increase in pressure and stagnation of urine. If the obstruction is localized in the ureteropelvic segment, hydronephrosis develops. When the narrowing is located in the prostatic (juxtavesical) part or in the middle third of the ureter, it expands significantly and lengthens above the obstruction. The ureter becomes long, tortuous, and can be as thick as the colon. In young children with an asthenic body structure and decreased muscle tone, such ureters can be palpated through the anterior abdominal wall. This pathology is often mistaken for megaureter.

The clinical picture is caused by impaired patency of the ureter, the development of hydronephrosis or ureterohydronephrosis, and the addition of infection.

The main method for diagnosing narrowing of the ureter is excretory urography.

Treatment is surgical, its scope depends on the condition of the upper urinary tract and the location of the narrowing. It consists of removing the narrowed part of the ureter and restoring its patency, transplanting the ureter into the bladder, partial or complete replacement with a segment of the intestine. If the renal parenchyma is destroyed, nephroureterectomy is indicated.

Ureteral valve is a congenital transverse fold of the mucous membrane containing circular muscle fibers.

Until the onset of ureterohydronephrosis and the addition of infection, the anomaly does not manifest itself clinically. The inflammatory process in the affected and contralateral kidneys is early complicated by an obstruction (obstruction) at any level of the ureter. This is explained by increased pressure in the renal pelvis, venous and lymphatic stasis, and ischemia of the renal parenchyma.

The diagnosis is established on the basis of excretory urography and retrograde pyelography. Renal arteriography allows you to determine the stage of hydronephrosis, but does not determine the cause of obstruction in the ureter. Radionuclide methods (scanning, renography) provide information about the amount of remaining renal parenchyma. Often the type of anomaly is clarified only during surgery. If hydronephrosis causes a sharp expansion of the renal cavities and almost complete death of its parenchyma, antegrade percutaneous puncture pyelography is used to differentiate it from cystic unilateral kidney damage.

Treatment consists of removing (resection) the affected area and restoring the patency of the ureter by suturing its ends. If the valve is placed in the pelvic part of the ureter, then after its removal, according to indications, the ureter is transplanted into the bladder or its plastic surgery is performed using the Boari method.

Nephrectomy and ureterectomy for narrowing and ureteral valve are performed only when the kidney parenchyma has turned into a thin-walled sac or when hydronephrosis as a result of infection has become complicated by pyonephrosis. In other cases, indications for nephrectomy should be sharply limited.

Ureteral diverticulum As a congenital pathology, it is observed very rarely. This is a long spherical formation that connects to the lumen of the ureter. Its walls have all the same layers as the walls of the ureter. The diverticulum develops as an additional ureteric bud. In most cases, it forms in the lower third of the ureter. It is most often localized on the left and very rarely on both sides.

Congenital ureteral diverticulum is usually small and does not cause any problems. Sometimes it reaches large sizes, compresses the ureter, causing ureterohydronephrosis, and if infected, pyonephrosis. A common symptom is persistent pyuria.

The main method for diagnosing this anomaly is excretory and retrograde pyelography. On ureteropyelograms in the lower third of the ureter there is a noticeable spherical formation, one end of which ends blindly, and the second is combined with the ureter. Sometimes this formation is located close to the kidney, and then it is mistaken for an additional ureter. In such cases, excretory urography and ultrasound and magnetic resonance imaging help to clarify the diagnosis. On urograms, the diverticulum is filled with X-ray contrast agent only in its lower part, and sometimes it is not filled at all.

Treatment consists of removing the diverticulum. For ureteropyonephrosis, nephroureterectomy is effective.

Ureterocele - cyst-like protrusion into the bladder of the mucous membrane or all layers of the wall of the intravesical part of the ureter. It is observed more often on the right, often on both sides. The size of ureterocele varies from a few millimeters to several centimeters in diameter. Sometimes it reaches large sizes, fills most of the bladder, and in women it can fall out through the urethra, simulating signs of a bladder.

The cause of ureterocele is a narrowing of the opening of the ureter or a change in its intravesical part. Urine is retained in the ureter above the narrowing, making it difficult to remove. The mucous membrane of the lower edge of the ureter, weakly connected to the muscular wall, slides into the bladder cavity under the pressure of urine and peristaltic waves, creating sac-like protrusions. It consists of two layers of mucous membrane: inside - the ureteral mucosa, outside - the bladder mucosa. Often stones form in its cavity.

The following types of ureterocele are distinguished: 1) simple; 2) which protrudes into the urethra (in women); 3) which, due to ectopia, opens into the urethra, diverticulum of the bladder; 4) which ends blindly.

A small ureterocele has no clinical manifestations, but a large one can cause dysuria and even complete urinary retention. With prolonged existence of ureterocele, dilation of the ureter and renal pelvis develops with the formation of ureterohydronephrosis and even pyoyephrosis. In such cases, kidney function is impaired.

The main method for diagnosing ureterocele is cystoscopy. When examining the site of the ureteral opening, a protrusion is detected, which either decreases or increases. On cystograms, a filling defect in the form of a half ring is observed, covering the cavity with radiopaque liquid. Excretory urography allows you to clarify the condition of the upper urinary tract and identify characteristic changes. Ultrasonography is widely used to diagnose ureterocele. On echograms, a ureterocele is a fluid-filled space-occupying formation in the projection of the bladder, delimited from it by a thin, well-defined membrane. Many patients suffering from ureterocele develop ureterohydronephrosis. This can be determined using ultrasound and computed tomography.

Treatment. Transurethral dissection of the ureterocele is performed. If the ureterocele is large or protrudes from the bladder through the urethra, it is excised transvesically and the bladder mucosa is sutured to the ureteral mucosa. When excising a ureterocele, the closing apparatus (sphincter) of the ureter is often disrupted, which leads to reflux and ureterohydronephrosis.

If the renal parenchyma dies, nephroureterectomy is performed.

N er clearly -muscular dysplasia of the ureter - bilateral congenital narrowing of the opening and intravesical part of the ureter in combination with neuromuscular dysplasia of its lower third, which leads to dilation of the ureter - megaureter .

There are two points of view regarding the etiology and pathogenesis of this anomaly: I) insufficiency of development of the neuromuscular apparatus of the ureter, 2) the presence of functional or organic obstacles at the level of the prostate or intravesical part of the ureter.

There are two forms of this anomaly: 1) caused by a mechanical obstruction in the prostatic or intravesical part of the ureter, which impedes the outflow of urine from the pelvic cystoid, 2) associated with a mechanical or functional disorder of urodynamics in the area of ​​the ureteral opening, bladder neck or urethra. The difference between them is that the second form is combined with vesicoureteral reflux, which is the direct cause of the dilation of the ureter.

Stage one of neuromuscular dysplasia of the ureter (achalasia) characterized by expansion of its lower cystoid, the second (megaureter) - expansion of the ureter along its entire length; third (hydrouretherone e froz) - complete irreversible disruption of urodynamics and hydronephrotic transformation of the kidney.

Clinically, the anomaly is not manifested. Its symptoms are observed if stagnation of urine leads to severe complications - ureterohydronephrosis, pyelonephritis, pyonephrosis, etc.

The addition of an infectious process leads to the appearance of symptoms of pyelonephritis. Further, as urodynamic disorders and kidney destruction due to pyelonephritis and hydronephrosis progress, signs of chronic kidney failure appear. Children grow slowly, their blood biochemical parameters change. Therefore, symptoms of chronic pyuria in children that recur are an indication for a special urological examination.

The main method for diagnosing anomalies is x-ray examination. During cystography, due to incomplete contraction of the ureteral opening, one can notice the filling of a sharply dilated, often with bends of the ureter due to vesicoureteral reflux. Excretory urography data allows you to assess renal function. Using the urogram, you can trace the course of the ureter and determine the degree of its expansion. On ureteropyelograms, a tortuous and sharply dilated ureter is clearly visible with a relatively slight ectopia of the renal pelvis. Urokymographic study against the background of reflux allows us to judge the peristalsis of the ureter.

Treatment is only surgical. If the expansion is limited to the final segment of the ureter, then it is crossed over the obstacle and reimplantation is performed into the bladder. Some authors supplement reimplantation with removal of most of the dilated ureter.

Methods of surgical treatment can be divided into the following groups: 1) ureterocystoneostomy with the formation of an intravesical “proboscis”; 2) ureteroneocystostomy with submucosal tunnel; 3) lengthening of the intravesical section of the ureter while maintaining its opening; 4) lengthening of the intravesical section of the ureter due to movement or plastic surgery of its opening; 5) creation of a duplicate from the dilated ureter, antireflux ureterocystoanastomosis; 6) ureteral prosthesis.

The fight against infection and homeostasis disorders is of great importance. Antibacterial therapy is used before performing plastic surgery, in the postoperative period for the purpose of sanitation of the kidneys and urinary tract, and in the absence of indications for surgery - as an independent method.

Surgeries on the ureter are complex and not always effective, especially in the later stages of the disease.

Annular ureter - a rare anomaly in which the ureter in the middle third is twisted in the form of a ring. The embryogenesis of this anomaly is probably related to the inability of the pelvic ureter to rotate with the kidney. This malformation does not cause a sharp disturbance in urodynamics, so its clinical manifestations are scarce. If inflammation occurs, the bend of the ureter becomes fixed, and disturbances in the outflow of urine begin to manifest themselves in the form of renal colic. The pathology can be complicated by the formation of stones and ureterohydronephrosis.

To diagnose this anomaly, excretory urography with delayed images is used. In rare cases, there is a need for retrograde pyelography.

Treatment. If an annular ureter causes urinary stasis, ureteric resection is performed with end-to-end anastomosis.

ANOMALIES OF LOCATION

Retrocaval ureter - the result of an anomaly of the inferior vena cava, in which the ureter in the upper third spirally covers the inferior vena cava, and starting from the middle third, goes in the usual direction - into the bladder. This unusual location of the ureter leads to impaired urine outflow and hydronephrotic transformation.

The development of hydronephrosis and infection of the retrocaval ureter does not have clinical manifestations, and it is diagnosed by chance. Persistent pyuria is often caused by chronic pyelonephritis.

The diagnosis is established on the basis of excretory urography data, and if kidney function decreases, retrograde pyelography. To clarify the diagnosis, cavography is sometimes used in combination with ureteropyelography.

The treatment for this anomaly is surgical - intersection and movement of the ureter forward from the vena cava with restoration of its patency. If the renal parenchyma dies, nephroureterectomy is performed.

Retroiliac ureter - a very rare anomaly. Characterized by the location of the ureter behind the common iliac artery.

Clinic. Diagnosis and treatment of this anomaly are the same as for retrocaval ureter.

E photocopy of the ureteral orifice - the location of one or both openings of the ureters in an atypical place. The ectopic opening usually belongs to one of the duplicated ureters. In most cases, the ureter, which drains the superior renal pelvis, opens at a lower level. In girls, the opening of the ectopic ureter usually opens in the vaginal vault or other parts of the external genitalia, in the urethra near its external opening, in the rectum, cervix or corpus of the uterus; in boys in the back of the urethra, vas deferens, seminal vesicles, perineum, rectum, etc.

Excretory or infusion urography, cystoscopy, uretero- and colpography help to correctly establish the diagnosis. Indirect information about the localization of the ectopic opening of the ureter can be provided by retrograde pyelography performed through a normally located opening. Obtaining a retrograde pyelogram characteristic of a double renal pelvis (an image of the main pelvis on the side of the anomaly, slightly lower and lateral than on the opposite side) makes it possible to determine with a high probability the side of the ectopic ureteral orifice.

In the absence of a kidney anomaly, the localization of the ectopic opening is determined on the basis of a sharp decrease in kidney function (according to data from infusion urography and scanning) and the absence (during cystoscopy) in the bladder on one side of the ureteral opening.

If the possibility of localizing the opening of the ureter in the family outflow tract is suspected, vesiculography should be used.

Treatment for ectopic ureteric orifice is surgical. In the presence of a large opening without accompanying duplication of the upper urinary tract, ureterocystoneostomy is indicated. If the function of the double kidney is preserved, a ureterourethroanastomosis or ureteropyeloanastomosis is created. With a sharp decrease in the function of the corresponding half of the kidney, heminephrectomy with ureterectomy is effective. If the kidney is not functioning, nephroureterectomy is performed. Removal of a kidney in case of ectopic ureteric orifice is carried out strictly according to indications.

Vesicoureteral reflux may be due to congenital insufficiency of the obturator apparatus of the ureteral orifice or impaired patency of the vesicoureteral segment and a chronic inflammatory process.

There are diametrically opposed opinions regarding the connection of reflux with anomalies of the vesicoureteral segment. Some authors believe that reflux occurs due to chronic urinary retention and inflammation. Others - which is caused by a violation of the normal anatomical and physiological state of the vesicoureteral segment. The frequency of reflux with duplication of the ureters indicates that its occurrence is due to one and other reasons.

So, vesicoureteral reflux should be considered as a condition bordering on anomalies of the ureter and bladder. The closure of the opening of the ureter is ensured by an increase in intravesical pressure by two factors: a) the oblique direction of the ureter as it passes through the muscular wall of the bladder and compression of the ureter during contraction of the muscle that pushes urine, b) the submucosal location of the final, intravesical part of the ureter, for about 11 mm. When pressure in the bladder increases, this section of the ureter is pressed against the muscle wall.

The closure of the ureteral opening is disrupted when the muscular wall is located in a perpendicular or close to it direction, as well as when the submucosal (intravesical) part is shortened. This position is created with complete doubling of the ureter, but can occur without this anomaly. In children with a short submucosal part of the ureter, maturation may occur before puberty with the disappearance of reflux. However, this may be prevented by infection and progressive obstruction of the vesicoureteral segment, as evidenced by the disappearance of reflux when the infection is controlled. In effect, a vicious circle is created: reflux promotes an ascending urinary tract infection, and the infection maintains the reflux. This circle can only be broken with targeted antibacterial therapy with the administration of large amounts of fluid.

The diagnosis is made based on the results of cystography and radionuclide research methods. Be sure to determine the condition of the vesicoureteral segment. If its patency is impaired, reflux can be eliminated only after correction of the said anomaly.

Depending on the severity of the reflux of the radiopaque fluid and the dilation of the upper urinary tract, five degrees of vesicoureteral reflux can be traced on the urogram: I - reflux of the radiopaque substance into the distal part of the ureter; II-filling of the ureter and calyx-pelvis system with a radiopaque substance; III - moderate dilation of the ureter with pyeloectasia and rounding of the calyx arches; IV - pronounced expansion and tortuosity of the ureter, deformation of the pyelocaliceal system; V - hydroureter and sharp thinning of the kidney parenchyma.

Treatment. In most cases, vesicoureteral reflux in children disappears with age. This is explained by the completion of the development of the corresponding section of the ureter due to its achievement of sufficient length in the submucosal layer of the bladder. Therefore, if the congenital inferiority of the obturator apparatus of the ureter is moderate (I, II, sometimes III degree), and the corresponding process has not yet begun or is benign, it is advisable to carry out conservative therapy (uroantiseptics, external electrical stimulation of the bladder with sinusoidal or diadynamic currents). If it is ineffective, anti-reflux operations are performed. Before this, the causes of obstruction of the bladder neck and urethra, if any, should be eliminated. At stages IV and V, resection of the distal ureter with ureterocystoanastomosis is recommended.

Children who have undergone surgery for vesicoureteral reflux are subject to supervision by a urologist and nephrologist.

The urinary tract begins with the renal pelvis and ends with the urethral opening.

Congenital malformations (anomalies) of the urinary tract

Congenital anomalies of the urinary tract are closely related to identical kidney anomalies. If a patient has two kidneys, there are three ureters on one side. In the case of unilateral renal agenesis, the ureter itself is correspondingly absent. Along with the presence of a double kidney, urethrocele is also quite common - protrusion of the urethra through the vaginal wall, resulting in the formation of a bulbous swelling. The ureter may have narrowings, possibly also in the case of a blind branch. In addition, it is possible that the ureter does not end in the ureter, but, for example, flows into the seminal vesicles or into the urethra itself.

Congenital malformations (anomalies) of the bladder

When the urinary duct is not closed during development, quite often a cyst begins to form in it. It is also possible to form a bladder diverticulum - a funnel-shaped or sac-like protrusion of the organ wall. All of the above anomalies are not accompanied by illness. One of the most severe malformations of the urinary tract is exstrophy of the bladder, which is manifested by damage not only to the bladder, but also to the anterior wall of the peritoneum, urethra and pelvic bones. If there is no treatment for these anomalies, the patient dies.

Congenital anomalies of the urethra

Epispadias is a developmental defect (anomaly) of the penis, in which the opening of the urethra, which is intended for urine output, is located on the upper part of the penis. Female representatives also often have malformations of the bladder and clitoris. Hypospadias is a disease in which the external (excretory) opening of the urethra is located on the lower part of the penis.

Symptoms of kidney abnormalities:

urinary disturbance;

growth disorder;

pain in the side and abdomen;

frequent urinary tract infections.

Causes of urinary tract abnormalities

All of the above anomalies of the urine extraction system from the body are congenital. Accordingly, they can arise as a result of a genetic defect or as a result of exposure to harmful factors on the fetus during gestation.

Treatment of anomalies of the genitourinary system

In most cases, treatment is surgical. Of course, when the congenital anomaly does not interfere with the flow of urine, treatment is not critically necessary.

Self-medication in the presence of congenital anomalies of the urinary tract is strictly prohibited.

Diagnosing anomalies in young children is extremely difficult. Parents should remember the main symptoms of congenital anomalies of this body system and monitor them as the child grows. This is an increase in body temperature of unknown origin, lack of appetite. These and other ailments, when identified, must be discussed with the pediatrician and, if necessary, diagnostics performed.

If there is a suspicion of a congenital anomaly of the urinary system, a blood test and ultrasound examination of the pelvic organs are prescribed. In addition to a blood test, an X-ray examination with the introduction of a contrast agent may also be prescribed to accurately determine the location of the anomaly.

Course of the disease

The vast majority of congenital malformations of the urinary tract are diagnosed accidentally (except for those that can be visualized with the naked eye) during examination of the patient for other reasons; usually such pathologies do not cause negative consequences in the future, but there are a number of cases of severe pathologies, for example, bladder exstrophy, which require emergency complex treatment.

Modern therapy for anomalies of the urinary system is possible only if the pathology is diagnosed early, so every child must undergo all mandatory preventive examinations.

ABSTRACT

Prevalence and epidemiology

anomaly kidney urinary polycystic disease

Anomalies of the urinary system are among the most common human developmental defects. According to the literature, from 10 to 15% of children are born with various malformations of the genitourinary organs, and the most common of them are kidney anomalies. It is believed that kidney anomalies account for about 40% of all human developmental defects.

Malformations of the kidneys and urinary tract can be single or multiple. Sometimes they are so severe that they are incompatible with life (eg, bilateral renal aplasia), while in other people they do not manifest themselves clinically throughout life. However, in most cases, with kidney anomalies, there is a violation of the outflow of urine from this organ and a violation of intrarenal hemodynamics, which is a pathogenetic factor for the development of pyelonephritis, stone formation, hydronephrosis and other pathological processes. Kidney defects are usually diagnosed as these complications progress.

Classification

The variety of renal malformations and the variety of their clinical manifestations make it necessary to systematize them, which is necessary both for choosing a method for their recognition and for determining the characteristics of treatment.

One of the first classifications of renal malformations belongs to the domestic urologist I. X. Dzirn, who identified:

Congenital absence of one of the kidneys (aplasia);

Congenital underdevelopment of the kidney (hypoplasia);

S-shaped kidney, etc.;

Congenital abnormal position of the kidneys (dystopia).

The classification of I. X. Dzirné reflected all the malformations of this organ known at that time.

E.I. Gimpelson combined all the malformations of the kidneys into four main groups: anomalies of quantity, anomalies of position, anomalies of relationship and anomalies of structure. The principle of this classification forms the basis of all modern classifications of renal malformations.

In our country, in order to unify numerous classifications of renal anomalies, at the II All-Union Congress of Urologists, a classification was adopted that included group VI of developmental defects, including renal vascular anomalies and combined renal anomalies.

I. Abnormalities in the number of kidneys:

1. Aplasia.

2. Complete and incomplete duplication of the renal pelvis and ureters.

3. Accessory (third) kidney.

II. Anomalies in kidney size: hypoplasia (rudimentary, dwarf kidney).

III. Anomalies in the location and shape of the kidneys:

1., Kidney dystopia:

a) one-sided (thoracic, lumbar, iliac, pelvic);

b) cross.

2. Kidney fusion:

a) one-sided (L-shaped kidney);

b) bilateral symmetrical (horseshoe-shaped, biscuit-shaped buds) and asymmetrical (L- and S-shaped buds).

IV. Abnormalities of the kidney structure:

1. Multicystic kidney.

2. Multilocular cyst.

3. Polycystic kidney disease.

4. Solitary kidney cysts, including:

a) simple solitary cyst;

b) dermoid cyst.

5. Parapelvic cyst, calyceal and pelvic cysts.

6. Calyceal-medullary anomalies:

a) megacalyx, polymegacalyx;

b) spongy bud.

V. Anomalies of the renal vessels:

a) accessory renal artery;

b) double renal artery;

c) multiple arteries.

2. Anomalies in the shape and structure of the arterial trunks (unilateral and bilateral renal artery aneurysms, fibromuscular stenosis of the renal artery.

2. Congenital arteriovenous fistulas.

3. Congenital changes in the renal veins:

a) anomalies of the right renal vein (multiple veins,
the confluence of the gonadal vein into the renal vein on the right);

b) anomalies of the left renal vein (annular left renal vein, retroaortic left renal vein, extracaval entry of the left renal vein).

VI. Combined kidney anomalies:

1. With vesicoureteral reflux.

2. With bladder outlet obstruction.

3. With vesicoureteral reflux and bladder outlet obstruction.

4. With anomalies of other organs and systems.

Some clarifications have been made to this classification in recent years. Thus, N.A. Lopatkini A.V. Lyulko (1987) believe that the first place in the classification should be given to the group of malformations of the renal vessels, and point “b” of the group “anomalies in the number of kidneys” should not be designated “doubling of the renal pelvis” , and “doubling of the kidney - complete and incomplete.”

Etiology and pathogenesis

There is still a lot of uncertainty about the origin of human developmental defects. Usually, the causal (causal) and formal genesis of malformations of any organs and systems are distinguished, which can be considered, respectively, as the etiology and pathogenesis of the malformation. According to A.V. Ayvazyan and A.M. Voino-Yasenetsky, in relation to the organs of the urinary system, causal genesis and etiology can be equated, while the formal genesis of a kidney malformation is not always its pathogenetic mechanism.

Based on their origin (causal genesis), all kidney malformations are divided into congenital and hereditary. The former are a consequence of intrauterine disease of the fetus and are most often associated with such exogenous damaging factors as exposure to ionizing radiation, medicinal and chemical agents, and steroid hormones. The second (hereditary) malformations are associated with pathology of the chromosomal apparatus. There are about 40 known types of hereditary kidney malformations, including such a common developmental defect as polycystic kidney disease.

The formal genesis of kidney malformations is currently considered in connection with the embryogenesis of this organ, distinguishing between developmental defects that are determined at the stages of the pronephros. primary kidney (mesinephros) and the formation of a permanent kidney (metanephros). It should be remembered that the kidneys and the upper urinary tract develop from completely different rudiments, which are initially not connected with each other. It is precisely this circumstance that many authors explain the extreme diversity of renal malformations.

Clinical symptoms. Diagnostics

Some kidney malformations have their own characteristic symptoms (for example, polycystic kidney disease), while others do not have any clinical manifestations at all, since they do not impair the function of this organ and are not a prerequisite for the development of complications. Quite often, kidney malformations are detected during a urological examination undertaken in connection with the clinical manifestation of their complications, because with many types of kidney anomalies there is a violation of the outflow of urine from this organ or a violation of its blood supply, which contributes to the occurrence of inflammatory processes, stone formation, atrophy of the kidney tissue, renal failure, hypertension. In this regard, from a practical point of view, it is advisable to characterize the clinical symptoms and methods for recognizing each type of renal malformation separately.

Kidney abnormalities

Aplasia (agnesia) of the kidney is the result of the absence or arrest in the development of the Wolffian duct and, according to autopsies, occurs with a frequency of 1: 610. With aplasia of the kidney, there is an absence

Rice. 1. Scheme of various variants of kidney aplasia:

The corresponding half of the vesical triangle or one of the ureteric orifices (Fig. 1). Renal aplasia usually:

a - aplasia of the left kidney, the ureter is absent; b - the ureter in the form of a small passage ends blindly; c - cross dystopia of a single kidney is accompanied by hypertrophy of the opposite kidney, with normal function of which chronic renal failure does not occur. Bilateral renal aplasia is incompatible with life, but is extremely rare. It has been noted that kidney aplasia in men is quite often (15-25%) combined with malformations of the genital organs: the absence or underdevelopment of the seminal vesicle, vas deferens, testicle or epididymis.

Aplasia of one of the kidneys with a healthy opposite kidney does not manifest itself clinically. Only rarely do people with a single congenital kidney notice dull pain in the lumbar region from this kidney due to its compensatory hypertrophy. This malformation acquires clinical significance only when disease (or damage) of the solitary kidney appears.

Unilateral renal aplasia is diagnosed using cystoscopy, excretory urography, renal scanning, radioisotope renography and renal angiography. During excretory urography, there is no release of contrast agent on the aplasia side, but this research method provides only tentative data on this malformation, since the excretory function of the kidney may be absent in many diseases.

The absence of kidney function during radioisotope renography can only lead the doctor to think about the possibility of this malformation, but not confirm the diagnosis. Renal aplasia is definitively determined using renal arteriography or aortography.

Duplication of the kidney is quite common and, according to autopsies, is observed with a frequency of 1: 125. The double kidney is longer than the normal one. The upper part of the double bud is usually smaller than the lower one, and between them there is a distinct furrow to varying degrees. With renal duplication, there may be duplication of the renal pelvis, duplication of the renal vessels, or duplication of both the renal pelvis and the renal vessels at the same time. The latest version of this malformation is called “complete doubling of the kidney.” With complete doubling of the kidney, each of its halves has a separate pyelocaliceal system, and a ureter departs from each pelvis. The ureters of the double kidney can either join together at the level of their upper or middle third and end in the bladder with one mouth, or continue with two ureters to the bladder, where they end with two mouths on one side of the bladder triangle.

In this case, in the bladder, the orifice of the ureter coming from the upper pelvis is located below the orifice of the ureter coming from the lower pelvis. However, the ureter of the upper half of the double kidney can end not only in the bladder, but also be ectopic, that is, open in other parts of the genitourinary organs:

in the vagina or urethra in women, in the urethra or seminal vesicles in men. The blood supply to both parts of the double kidney is usually carried out by two renal arteries, caliber

Rice. 2. Retrograde pyelogram

Complete duplication of the right kidney, the left kidney without features proportional to the vascularized mass.

Clinically, kidney duplication may not manifest itself in any way. However, in many people with this developmental defect, the outflow of urine from the kidney is disrupted, which is accompanied by hydronephrosis, the addition of pyelonephritis, the formation of stones and other pathological conditions that manifest themselves clinically. In patients with ectopia of the ureteral orifice, urinary incontinence during normal urination comes first.

A double kidney should be diagnosed based on excretory urography and cystoscopy. In doubtful cases, they resort to retrograde pyelography (Fig. 2), ultrasound scanning, and computed tomography.

An accessory (third) kidney is extremely rare. With this malformation, in addition to two normal kidneys, there is one more. The accessory kidney has its own capsule, independent blood supply and its own ureter. It is completely separate from the normal kidney, and between the accessory and normal kidneys there is loose adipose tissue. The size of the accessory kidney is smaller than the normal one. The accessory kidney is most often located at the level of the lumbar vertebrae or in the iliac region, less often in the pelvis.

The accessory kidney usually does not manifest itself clinically. In diseases of the accessory kidney, the same symptoms occur as in diseases of the main kidneys, but the clinical picture becomes atypical due to its abnormal location. Sometimes an accessory kidney is mistaken for a tumor.

Abnormalities in kidney size

Hypoplasia is characterized by a kidney that is reduced in size.

There are two variants of this developmental defect: rudimentary kidney and dwarf kidney. In a rudimentary kidney, which arises as a result of stopped growth of the organ in the early period of embryogenesis, its parenchyma consists of connective tissue with a small number of glomeruli and tubules. A dwarf kidney is a normal organ in structure, but reduced in size. Renal hypoplasia is most often unilateral and very rarely bilateral. With unilateral hypoplasia, the second kidney is usually increased in size.

A hypoplastic kidney is often affected by pyelonephritis and can cause hypertension. This developmental defect is identified
based on excretory urography. When function decreases
hypoplastic kidney is used to clarify the diagnosis
scintigraphy or retrograde pyelography, sometimes -
computed tomography. A hypoplastic kidney should be differentiated from a secondary shriveled kidney, and renal angiography can clarify the diagnosis.

Anomalies in the location and shape of the kidneys

Kidney dystopia. If in the embryonic period the primary kidney does not move from the pelvis to the lumbar region and the kidney does not rotate 180° around its axis, then its dystopia (congenital unusual position) is observed. According to autopsies, renal dystopia occurs on average once in 600-1200 autopsies. Depending on the location of the kidney, pelvic, ileal, lumbar and thoracic dystopias are distinguished. The dystopic kidney differs from the normal one in its pronounced lobulation, the renal pelvis is located on the anterior surface, the calyces are turned towards the spinal column. With pelvic, iliac and lumbar dystopia, the ureter is shortened, while with thoracic dystopia, on the contrary, it is longer than usual. Kidney dystopia can be homolateral or heterolateral; with the latter version of this development, one of the kidneys moves to the opposite side. Kidney dystopia can be unilateral or bilateral. Thoracic kidney dystopia is extremely rare. With this malformation, the kidney is located above the diaphragm. This type of malformation is most often detected during preventive examinations during X-ray examinations of the chest cavity and sometimes the visible shadow is mistaken for a tumor or cyst of the lung or mediastinum.

Lumbar dystopia is characterized by the location of the kidney in the lumbar region, but 1-2 vertebrae lower than usual. Lumbar dystopia differs from a prolapsed kidney in that it has a shortened ureter.

Rice. 3. Ileal dystopia of the left (Fig. 3.) kidney. The medial location of the glasses and the lateral origin of the ureter are noted.

Ileal dystopia is a relatively common anomaly of the location of the kidney. The kidney is located at the level of the ilium wing, and its cups are directed towards the spine

The clinical course of ILIAC DYSTOPIA is characterized by pain in the iliac region due to the pressure of the dystopic kidney on neighboring organs and nerve plexuses, as well as impaired urodynamics. With this type of anomaly, the kidney can be felt in the abdomen and mistaken for a tumor. The literature describes observations when such a kidney, mistaken for a tumor, was removed, and the correct diagnosis was made only after a morphological examination of the removed organ.

Pelvic dystopia is characterized by the location of the kidney in the pelvis and sometimes it is located between the bladder and rectum in men and between the uterus and rectum in women. The shape of the kidney in pelvic dystopia differs significantly from the usual one, sometimes it is round, sometimes square and flattened. The pelvis of the pelvic-dystopic kidney is rudimentary, and the ureter is sharply shortened. The vessels of such a kidney are of the loose type. This malformation manifests itself as pain in the pelvis or abdomen, which usually appears during movement. When the kidney puts pressure on the bladder, dysuria is possible.

In women, due to the pressure of the dystopic kidney on the uterus, pregnancy often occurs with various complications. A pelvic-dystopic kidney is diagnosed during a bimanual examination, when a dense, almost motionless or sedentary formation is identified in the vicinity of the rectum in men and the posterior vaginal vault in women, which is most often mistaken for a tumor. The diagnosis is clarified using x-ray methods. Very rarely observed cross dystopia of the kidneys.

For any type of renal dystopia, the differential diagnosis is made with nephroptosis and an abdominal tumor. The examination package must include ultrasound, excretory urography in the supine and standing position, and sometimes renal angiography is performed.

Renal fusion is quite common and accounts for 14% of all renal anomalies. This type of malformation is characterized by the fusion of two kidneys into one organ, but the ureters of the fused kidneys end in the bladder in the usual places, and each of the fused kidneys has its own blood circulation. Fusion can occur either symmetrically with the upper or lower poles (horseshoe kidney), or asymmetrically, when the lower pole of one kidney fuses with the upper pole of a vertically rotated (S-shaped kidney) or horizontally located (L-shaped kidney) other kidney. Both kidneys can grow together completely (biscuit-shaped kidney).

E.M. Gimpelson (1936) proposed the following classification of this type of renal malformation:

1. Horseshoe kidney.

2. Symmetrical:

Biscuit-shaped kidney;

Fused kidneys;

L-shaped kidney.

3. Asymmetrical - S-shaped kidney.

Rice. 4. Horseshoe kidney (diagram)

The most common type of this group of anomalies is the horseshoe kidney. It occurs once every 700-800 autopsies and is formed as a result of fusion of nephrogenic blastomas in the embryonic period.

Most often (95%) the kidneys are fused at the lower poles (Fig. 4). The isthmus of the kidney, connecting the lower poles, consists of either dense fibrous tissue or renal tissue. It is usually located in front of the large vessels (aorta, inferior vena cava) and the solar plexus, which the isthmus presses against the spine. The pelvis of the horseshoe kidney faces anteriorly and medially and is sometimes located intrarenal. The ureters of the horseshoe kidney are directed inward and anteriorly, spreading across the isthmus or lower poles of the kidneys. Each of the kidneys, fused by the same poles, has its own blood circulation, and most often there are several (3-5) arteries that can flow into the parenchyma of the horseshoe kidney in addition to the renal sinus.

The clinical manifestations of horseshoe kidney are varied. Due to the pressure of the isthmus on the nerve plexuses, a pain symptom complex occurs. The pain may be intermittent, but usually worsens during trunk extension (Rovsing's sign) and after exercise. Compression of the aorta is observed less frequently and is accompanied by symptoms of coarctation of the aorta. Compression of the inferior vena cava can lead to venous hypertension of the lower half of the body, which is accompanied by swelling of the lower extremities and a feeling of numbness.

Due to abnormal circulation of the horseshoe kidney, renovascular hypertension may occur, and due to intrarenal venous hypertension, gross hematuria may occur.

Rice. 5. Horseshoe kidney

The pelvis is located at the level of the second and third lumbar vertebrae. The cups are directed towards the spinal column

With a horseshoe kidney, the outflow of urine from this organ is almost always impaired, which is due to the high discharge of the ureter, its compression when thrown over the isthmus or lower poles of the kidneys and bends. Impaired urodynamics is one of the factors causing abdominal pain.

Violation of the outflow of urine from the horseshoe kidney and the peculiarities of its blood circulation contribute to the development of various complications. It is believed that various diseases of the horseshoe kidney (hydronephrosis, pyelonephritis, nephrolithiasis, etc.) occur in 85-90% of people with this malformation, and often the symptoms of complications predominate in the clinical picture. According to N.A. Lopatkin and A.V. Lyulko (1987), the most common complication is pyelonephritis, followed by hydronephrosis and nephrolithiasis.

Recognition of a horseshoe kidney is based on data from x-ray and radioisotope research methods. The examination begins with a survey and excretory urogram. Sometimes in the photographs you can see the silhouette of a horseshoe kidney. The pelvis on excretory urograms is located laterally, they are most often dilated, and the calyces are directed towards the spinal column (Fig. 5). The ureters depart from the lower pole of the kidney and go to the spine. The scans reveal a typical accumulation of nucleides in the form of a horseshoe. Renal arteriography is used to study the angioarchitecture of the horseshoe kidney.

Indications for surgical treatment arise in case of complications of a horseshoe kidney (stones, hydronephrosis, etc.).

Abnormalities of kidney structure

This group of anomalies includes polycystic kidney disease, spongy kidney, solitary kidney cyst, multicystic kidney, parapelvic renal cyst, diverticulum of the pelvis, calyx-medullary anomalies and some other kidney malformations in which there is a violation of the structure of the renal tissue. In recent years, some authors include dysplastic kidneys in this group of anomalies: simple dysplasia, cortical dysplasia, oligomeganephronia, segmental dysplasia.

Polycystic kidney disease is a fairly commonly observed bilateral kidney malformation. Polycystic disease is characterized by the replacement of the renal parenchyma with multiple cysts of various sizes (Fig. 8.7) and occurs on average once in 400 autopsies. However, polycystic kidney disease is not always recognized during life, so the frequency of clinical recognition of this defect is 2-3 times less frequent than pathological recognition.

Rice. 6. Polycystic kidney disease (scheme) of provisional urine from the proximal nephron

Polycystic disease is a hereditary disease and is equally common in men and women. According to current theories, this anomaly is a consequence of a violation of kidney embryogenesis at the stage of fusion of the excretory and secretory apparatuses, that is, straight and convoluted tubules. As a result, the outflow is disrupted

The contents of the resulting cysts contain uric acid, urea, cholesterol, mineral salts, and sometimes blood. Large cysts put pressure on the renal parenchyma, causing its atrophy and replacement with connective tissue. At the same time, normal nephrons are formed in the kidney during embryonic development, so chronic renal failure develops slowly, and in many patients polycystic kidney disease proceeds throughout their lives without clinical manifestations.

In the clinical course of polycystic kidney disease, most authors distinguish 3 stages: compensated, subcompensated and decompensated.

In the compensated stage of polycystic kidney disease there are no clinical manifestations of chronic renal failure and most patients do not show any complaints for a long time. Only some patients report dull pain in the kidney area. The first clinical manifestations of this malformation are observed by the age of 40-45 and are characterized by arterial hypertension, pyelonephritis, and abdominal pain. According to our data, the first clinical manifestation of polycystic kidney disease in 40% of patients was arterial hypertension, in 24% - abdominal pain and digestive disorders, and in 36% - exacerbation of pyelonephritis.

The subcompensated stage of this malformation is characterized by symptoms of renal failure. Patients complain of fatigue, headache, nausea, dry mouth, and thirst. Many patients at this stage experience increased blood pressure, frequent exacerbations of pyelonephritis, and gross hematuria as a result of venous hypertension. Suppuration of cysts may occur.

The decompensated stage of polycystic disease is accompanied by a sharp decrease in the functional capacity of the kidneys - the terminal stage of chronic renal failure. It is characterized by vomiting, severe thirst, oligoanuria and anuria.

For most patients with polycystic kidney disease, a slow course is characteristic, and deterioration occurs with exacerbation of pyelonephritis.

To recognize polycystic kidney disease, anamnesis and study of the patient's pedigree are important. When examining patients, pay attention to dry and yellow skin. When palpating the abdominal cavity, in many patients it is possible to palpate the kidneys in the form of lumpy, dense formations, since the kidneys with polycystic disease are enlarged in size. Sometimes the kidneys increase to such a size that the bulging of one of the hypochondriums becomes noticeable when examining the patient. When examining urine, hypoisosthenuria is noted, and with the addition of pyelonephritis, leukocyturia is noted. A blood test reveals anemia, an increase in creatinine levels, and with exacerbation of pyelonephritis or suppuration of cysts - leukocytosis with a shift in the blood count to the left. X-ray examination methods are of decisive importance in the diagnosis of polycystic disease. Identification of several radiological signs of polycystic kidney disease: changes in the outline of the kidney, deformation of the pelvis and calyces in the form of their semilunar and spherical changes, widely spaced enlarged calyces, narrowing and lengthening of the necks of the calyces, an increase in the number of calyces, lengthening and displacement of the pelvis, changes in the vascular system of the kidneys, displacement of the ureter inside (Fig. 7).

Rice. 7. Bilateral retrograde pyelogram for polycystic kidney disease. There is deformation of the pelvis and calyces, lengthening of the necks of the calyces, and an increase in the height of the renal shadow.

If renal function decreases, it is advisable to perform infusion modification of excretory urography. Angiograms of polycystic kidneys are characterized by avascular zones, thinned and elongated main vascular zones, thinned and elongated main vessels.

Polycystic kidney disease should be differentiated from a kidney tumor, multicystic kidney, and, less commonly, from a solitary kidney cyst.

Treatment of patients with polycystic kidney disease consists of preventing and treating its complications. When cysts suppurate, they are punctured or dissected with drainage of the kidney.

Multicystic kidney disease is a rare anomaly that resembles polycystic disease, but is a unilateral pathological process. A valuable method for recognizing this renal malformation is angiography, in which the renal vessels are not differentiated. Computed tomography is also used.

Multilocular renal cyst is a multilocular thin-walled cyst located in one of the poles of the kidney. Rarely seen. Solitary renal cyst. There are simple and dermoid solitary renal cysts. This malformation is characterized by the presence of a single cystic formation. Enlargement of the cyst gradually leads to atrophy of the renal parenchyma and causes disruption of intrarenal hemodynamics. This developmental defect most often manifests itself at the age of 40-60 years. The most characteristic clinical symptom of a solitary kidney cyst is a dull pain in the lumbar region. In 70% of patients, this malformation has no subjective symptoms. The most common complications of a simple kidney cyst are its suppuration, less often - rupture and hemorrhage into the cyst.

Diagnosis of solitary renal cyst is based on data from ultrasound examination of the kidney, excretory urography, percutaneous cystography, and renal angiography. Sometimes they resort to performing computed tomography.

Treatment consists of puncture of the cyst or its opening and excision of the walls.

Spongy kidney is characterized by the presence of multiple small cysts in the renal pyramids. Cysts have the appearance of a finely porous sponge, hence the name of this malformation. The cavity of some cysts contains small stones. The relatively benign course of this anomaly without the presence of chronic renal failure is explained by the fact that the glomeruli of the cortical layer remain morphologically and functionally complete.

Abnormalities of the renal vessels

The accessory renal artery is quite common and in many people it is combined with other malformations of this organ. Sometimes the number of accessory arteries reaches 4-8. The accessory renal artery, as a rule, goes to the upper or lower segment of the kidney and has a smaller caliber compared to the main one. Accessory arteries going to the upper segment of the kidney do not disturb urodynamics, while arteries going to the lower pole are one of the main causes of the development of hydronephrosis due to impaired outflow of urine from the kidney. In this case, the accessory vessel compresses the ureter. This malformation of the renal vessels is recognized by X-ray examination. Excretory urograms can reveal narrowing in the area of ​​the ureteropelvic segment (Fig. 8), and renal angiograms can reveal the number and location of accessory arteries. Treatment is surgical.

Intrarenal constriction vessel. This type of renal vascular malformation is characterized by compression of one of the vessels of the isthmus of the upper calyx (Fraley's symptom). This causes stagnation of urine in this cup and its expansion. Diagnosis is based on data from excretory urography and renal arteriography.

Renal artery aneurysms are usually located in the main renal artery or its main branches and are characterized by sac-like dilatation of the vessel. This developmental defect manifests itself mainly in young people. Its complication is thrombosis of the aneurysm with possible subsequent dispersion of emboli and the formation of renal infarctions. Renal angiography plays a decisive role in the diagnosis of renal artery aneurysm. Treatment is surgical. Blood pressure does not normalize when using antihypertensive drugs. Kidney function is not impaired. Typically, such patients are observed for a long time by therapists for high blood pressure.

Renal angiography is used for recognition. Treatment is surgical.

Accessory renal vein and multiple renal veins refer to abnormalities in the number of renal vessels. Accessory veins are more common on the right than on the left. The accessory renal vein, compressing the urinary tract, can lead to disruption of the outflow of urine from the kidney and the development of hydronephrosis.

Rice. 8. Accessory vessel of the right kidney. A narrowing is detected in the area of ​​the ureteropelvic segment

The flow of the vein of the right testicle into the right renal vein is a malformation of the vascular bed of the kidney, since normally it should flow into the inferior vena cava. Accompanied by the appearance of symptomatic varicocele on the right.

An annular left renal vein is a common anomaly of the left renal vein. According to the literature, it accounts for 15-18% of all causes of renal vein stenosis. Clinically, this malformation manifests itself as varicose veins of the spermatic cord and bleeding from the left kidney due to the development of venous hypertension. Extracaval entry of the left renal vein is accompanied by venous hypertension in the kidney due to impaired blood outflow. With this malformation, the left renal vein does not flow into the inferior vena cava, but into the left iliac vein and, in such an unusual direction, intersects with the horizontal arteries of the kidney. The main clinical manifestation of the extracaval drainage of the left renal vein is recurrent hematuria. Diagnosis is based on vascular studies. Characteristic signs of this defect on angiograms are the premature appearance of a nephrogram and venogram due to fistula blood flow. The images also determine the direction of the renal vein going down into the left common iliac vein. Treatment is conservative. Only with profuse and frequently recurring hematuria is kidney decapsulation or, as an exception, nephrectomy indicated.

Forecast. Criteria for determining disability. Disability due to renal malformations is usually observed with the addition of complications (pyelonephritis, chronic renal failure, arterial hypertension, nephrolithiasis, etc.). Permanent or long-term disability occurs with increasing symptoms of chronic renal failure, which is most often observed with polycystic kidney disease.