Anomalies in the development of the urinary tract. Malformations of the skull, musculoskeletal system, digestive and genitourinary systems. Congenital heart defects. Clinical picture, diagnosis, treatment Malformations of the genitourinary system

According to statistics, anomalies in the development of the genitourinary system occur in every tenth person, and they lead to serious problems of a physiological and psychological nature. These anomalies are one of the most numerous, since they include malformations of many organs. They can only be diagnosed with medical equipment, such as x-rays or ultrasound.

What is the pathology of the development of the genitourinary system?

Malformations of the genitourinary system are genetic mutations expressed by the underdevelopment of a separate organ, or, conversely, its hyperdevelopment. Abnormalities include damage to the kidneys, bladder, ureters, urethra, and cause problems in the male and female genital area. Some may be considered minor, such as duplication of the ureters.

Malformations of the organs of the genitourinary system entail serious consequences, but there are also less dangerous pathologies.

Varieties of pathologies

Violation in the development of the urinary tract

Pathology of the urinary tract refers to a violation in the development of the kidneys, ureters, renal vessels and bladder. This type of pathology is divided into two groups - anomalies of structure and position. In case of violation of the proper development of the kidneys, its underdevelopment (aplasia), hypoplasia, ureters and pelvis, polycystic kidney disease, lumbar dystopia, occurs. Ureteral developmental disorders include:

  • doubling;
  • aplasia;
  • congenital narrowing;
  • pathology of the valves of the ureters;
  • retrocaval location;
  • ectopia (wrong location of the mouth).

Pathology of the bladder

Some of the anomalies may never manifest themselves, and some make themselves felt from the first days of life.

Aplasia, doubling, diverticulum, and exstrophy are common. A diverticulum is when the wall of the bladder bulges out. When there is no overgrowth of the urinary duct, a cyst is formed. At the same time, no symptoms of malaise are observed. But the most severe and dangerous defect is considered to be exstrophy - a pathology not only of the bladder, but also of the abdominal cavity, pelvic bones, and also the urinary canal. This deviation implies mandatory treatment, since if ignored, a fatal outcome is possible.

Pathologies of the urethra in women, men and children

Malformations of the urinary system (urethra) - a congenital pathology of the structure of the urethra and, as a result, dysfunction. In this case, there is a difficult passage of urine, which adversely affects the functioning of the kidneys, therefore, they require prompt treatment. It should be noted that pathologies of the urinary system are more common than other organs. These include doubling, hypospadias, epispadias. Hypospadias - the wrong location of the external opening of the urethra (is below the position). Hypospadias occurs only in men. In women, there is often a violation of the development of the clitoris. Epispadias is considered a defect of the penis, when the external opening of the urethra in women and men is located above the norm on the upper surface of the penis.

Causes of the development and occurrence of anomalies of the urinary system

During the bearing of a child, a woman and the condition of the fetus are affected by various harmful factors.

Anomalies of the genitourinary system are congenital malformations of the fetus (CMD). Some problems are inherited from parents. Pathology may lie in the mutation of the gene. The occurrence is affected by deviations from external factors. Congenital malformations begin to appear in the fetus in the first three months of pregnancy. During this period, the formation of internal organs takes place, and any external impact of adverse factors directly on the fetal body can provoke a delay or, conversely, excessively rapid development. Unfavorable factors include viral infections that a pregnant woman suffers from, the influence of harmful chemical factors (dyes, resins), the use of alcoholic beverages during pregnancy, as well as antibiotics, hormones and other medicines, and the influence of radiation.

Autosomal recessive polycystic kidney disease
Epidemiology
The frequency is 1 in 4000 newborns. The gene responsible for the inheritance of polycystic kidney disease is located on the short arm of chromosome 6, the risk of recurrence of the disease in the family if it is detected in one of the children is 25%. Therefore, it is advisable to carry out prenatal karyotyping and determine the karyotypes of the parents, in case of fetal death, a complete pathoanatomical study of it.

Etiology
In autosomal recessive infantile kidney disease, the lesion is symmetrical. Cystic formations in the tissue of the kidneys no larger than 1-2 mm in size, they are the result of dilatation and hyperplasia of normally formed collecting ducts.

Antenatal diagnosis
Sonography is a valuable method for antenatal diagnosis of polycystic kidney disease with an autosomal recessive type of inheritance, which makes it possible to diagnose this pathology starting from the 20th week of gestation.

Ultrasound reveals enlarged hyperechoic kidneys. The increase in the size of the kidneys is so significant that they occupy most of the volume of the abdominal cavity of the fetus. The combination with oligohydramnios, an empty bladder and pulmonary hypoplasia against the background of intrauterine malnutrition indicates the maximum degree of intrauterine damage and serves as an indication for termination of pregnancy.

Forecast
The prognosis for autosomal recessive infantile kidney disease is poor. Most fetuses with prenatally diagnosed disease die shortly after birth. In newborns with established pathology in the first month of life, renal failure increases for 6-8 months. Patients who survive the neonatal period have a better prognosis. However, in general, the outcome is unfavorable, since in the future hepatic fibrosis and portal hypertension join the existing manifestations of renal failure.

Most of the patients who survive the period of infancy require kidney transplantation.

Autosomal dominant polycystic kidney disease
Etiology
Autosomal dominant polycystic kidney disease is a consequence of disturbances in the process of the final stage of nephrogenesis. Cystic degeneration occurs in both the nephron and the collecting ducts. Inheritance occurs in an autosomal dominant manner, in the general population the frequency is 1:1000 people.

Prenatal diagnosis
Antenatally echographic signs appear rather late, even at the end of the II and the beginning of the III trimester, it is not always possible to identify such characteristic signs as nephromegaly and cysts of various diameters. The echogenicity of the parenchyma may be increased, the amount of amniotic fluid is usually normal or slightly reduced, the bladder is visualized.

Of the combined anomalies, it is necessary to exclude defects in the valvular apparatus of the heart, intracerebral vessels and the liver.

Forecast
The main factor in predicting the outcome of pregnancy is the amount of amniotic fluid. With a normal number of them, the prognosis for the life of the fetus is relatively favorable, but the development of arterial hypertension and chronic renal failure cannot be completely excluded. The risk of recurrence of this form of pathology in the family is 50%, especially if there is a renal pathology in the mother or maternal heredity is aggravated. Therefore, if signs of autosomal dominant polycystic kidney disease are detected in the fetus, a thorough examination of the urinary system of the parents, and often an invasive prenatal genetic diagnosis, is necessary.

Differential diagnosis is carried out with multicystic kidney disease, neoplasms and other types of renal dysplasia, as well as with congenital nephrotic syndrome and intrauterine infection. Autosomal dominant polycystic kidney disease is characterized by a family history of kidney disease: kidney cysts, pyelonephritis, manifestations of renal failure in relatives.

Multicystic kidney dysplasia
Epidemiology
More often one kidney is affected, the frequency of a unilateral process is 1:3000-5000, bilateral - 1:10,000 newborns. Multicystosis can be either an isolated defect or be part of a number of syndromes; boys are more likely to suffer (sex ratio 2.4: 1).

Etiology
Multicystic kidney dysplasia is a consequence of impaired nephrogenesis in the early stages of development. The reason for the degeneration of the renal tissue is an early developing obstruction of the urinary tract.

Antenatal diagnosis
Antenatal echographic signs of multicystosis appear already at the beginning of the second trimester of pregnancy. By the beginning of the third trimester, the kidney is a conglomerate of cysts of various diameters, between which there is hyperechoic tissue of varying severity, nephromegaly can reach sizes of more than 10 cm in diameter. With a unilateral process, the amount of amniotic fluid and the structure of the bladder are not changed. With a bilateral process, there is a sharp oligohydramnios, which progresses over time, while the size of the kidney cysts in dynamics can decrease, up to the complete absence of visualization of the kidney. Some authors believe that kidney aplasia is ultimately the result of its cystic dysplasia, which in some cases is confirmed histologically.

Treatment
Questions about the need and timing of surgery for a multicystic kidney are decided individually. If the size of this kidney is less than 5 cm, then in most cases the cysts can regress on their own.

A follow-up catamnesis showed that if the tissue of a cystically altered kidney is represented by small cysts, then after 6-12 months their size decreases at least 2 times, and by 2-3 years of age they can be completely reduced. In the case of persistent multicystic tissue over the age of 3 years, laparoscopic nephroureterectomy can be performed.

However, there are situations when nephroureterectomy is required in the neonatal period. Indications for nephrectomy in newborns are as follows:
- large sizes of the multicystic complex (its total size exceeds the size of a healthy kidney or the maximum diameter of the largest cyst exceeds 30 mm);
- clinical and ultrasound signs of suppuration of cysts.

Forecast
For isolated non-syndromic cases, a family renal history and combination with other malformations are uncharacteristic. The prognosis, of course, depends on the form of the lesion: in the case of a bilateral process, the outcome of the disease is absolutely unfavorable, while with a unilateral process, the prognosis for life is optimistic.

Solitary kidney cysts
Epidemiology
The frequency of detection of simple kidney cysts in children of the first year of life is not so rare, it is 1.6:1000 and, as a rule, is an ultrasound finding, no correlation with gender was noted. Solitary infantile renal cysts are usually represented by liquid, round, single-chamber formations. Histologically, the cyst wall consists of a single-layer epithelial layer, and the contents are serous fluid. The etiology of these formations has not been established.

Prenatal diagnosis
Echographically, the size of the kidneys correspond to age norms, and the cysts do not communicate with the collector system. Antenatally, infantile kidney cysts can be visualized from the second trimester of gestation as unilateral single-chamber anechoic formations of various diameters.

Treatment
There are few studies of patients with solitary infantile cysts, but the available data suggest that the size of the cysts does not increase with age and they do not cause renal impairment.

Forecast
The prognosis for single simple kidney cysts is considered favorable.

Obstructive uropathy
Epidemiology
Obstructive uropathy are among the most commonly diagnosed malformations of the urinary system, they account for more than 50% of all prenatally diagnosed anomalies. About 1% of all newborns have a prenatally diagnosed expansion of the renal collecting system.

Prenatal diagnosis
If the ultrasound shows an increase in the size of the pelvis of the fetus more than 4 mm in the II trimester and more than 7 mm in the III trimester of pregnancy, this is a sign of pyelectasis. Almost 70% of cases of antenatal detected pyelectasis are not the result of true urinary tract obstruction, but are associated with structural and functional immaturity. In this case, a stable echographic picture is characteristic, without an increase in the size of the pelvis and thinning of the parenchyma. However, in the case of an increase in pyelectasis, accompanied by a decrease in the kidney parenchyma, an urgent complete nephrourological examination of the child after birth is necessary to determine further treatment tactics, including surgery. The main strategy for managing patients with expansion of the pelvicalyceal system detected in the prenatal period: dynamic observation in the neonatal and infancy periods in order to determine the nature of pyelectasis, prevention of urinary infection, the development of nephrosclerotic processes and renal failure.

Fetal Interventions
Currently, there are more and more reports of prenatal surgical treatment of obstruction of the urinary system in the fetus. However, this method is not always justified. To determine the tactics of management and address the issue of the need for intrauterine interventions, it is first of all necessary to assess the functional state of the fetal kidneys. To do this, determine the osmolarity and the content of Na and Cl ions in fetal urine obtained by puncture above the site of obstruction. It has been established that a favorable outcome of intrauterine decompression of the urinary tract with good immediate and long-term results can be predicted with the following values ​​of prognostic criteria:
To determine the indications for antenatal invasive therapy aimed at correcting malformations of the urinary system in the fetus, a number of aspects should be taken into account:
- gestational age at which obstructive uropathy was first detected;
- the rate of progression of the pathological process;
- the amount of amniotic fluid;
- one- or two-sided nature of the lesion;
- biochemical analysis of urine, etc.

Despite the progressiveness of the method of antenatal therapy, information about a large number of complications of the procedure (according to various sources, up to 45%) and not always a long-term effect after unloading the urinary tract, do not allow unequivocally recommending invasive fetal interventions.

Diagnostics
Ultrasound of newborns and infants allows you to recognize malformations, confirm or exclude urological diseases detected prenatally. The choice of tactics for the treatment of newborns with defects should be based on the results of comprehensive studies conducted in the neonatal period. The volume of the research program for each specific newborn with suspected anomalies of the urinary system depends on the proposed diagnosis. Early examination and treatment of children in specialized departments is required, preventing infection of the urinary system. Indications for surgical treatment at an early age are all obstructive uropathy, leading to a delay in the maturation of the renal tissue, the development of secondary pyelonephritis.

Newborns with malformations of the urinary system can be divided into homogeneous clinical and functional groups according to the severity of the lesion and its clinical course:
Group 1 - newborns with suspected anomalies incompatible with life (children with prenatally or postnatally diagnosed kidney anomalies - bilateral renal agenesis, bilateral multicystosis, infantile polycystic kidney disease, bilateral hydronephrosis or ureterohydronephrosis with a complete absence of parenchyma). As a rule, the prognosis for confirmation of the diagnosis is unfavorable.

Group 2 - newborns with severe forms of obstructive uropathy (dilation of the renal pelvis more than 20 mm), infravesical obstruction, urinary tract infection, clinical and laboratory signs of renal failure, bladder exstrophy. Since untimely provision of qualified assistance can lead to irreversible consequences, such children should be transferred to a specialized department.

Group 3 - newborns with an expansion of the renal collector system from 10 to 20 mm, but with normal thickness and echogenicity of the parenchyma. These children need prophylaxis of urinary tract infection and outpatient follow-up by a pediatric urologist.

Group 4 - newborns with expansion of the renal pelvis no more than 10 mm, without expansion of the calyces and intact parenchyma. These patients should be observed on an outpatient basis by a pediatric urologist. Parents are advised to monitor the timely emptying of the bladder in a child, to prevent constipation (restorative massage of the anterior abdominal wall, rational nutrition of a nursing mother and child, etc.). Children belonging to the 2nd clinical and functional group need a specialized urological examination to assess the degree of damage to the renal tissue and the need for surgical treatment in the neonatal period.

hydronephrosis
Hydronephrosis is a persistent expansion of the pyelocaliceal system with atrophy of the parenchyma and a decrease in kidney function due to impaired patency of the pyelourethral junction.

Epidemiology
Obstruction in the pyeloureteral segment is twice as common in boys, left-sided kidney damage predominates. Bilateral process occurs in 5-15% of cases.

Etiology
Obstruction of the outflow of urine in the pyeloureteral segment leads to an increase in pressure in the renal pelvis and to an increase in the level of renin. This, in turn, leads to an increase in endogenous angiotensin, which causes renal ischemia with a decrease in glomerular filtration rate. Accession of the inflammatory process in the pelvis enhances pathological changes in the renal parenchyma. All this leads to serious violations of kidney function with the development of nephrosclerosis. Obstacles to the outflow of urine in the pyeloureteral segment can be internal and external. Embryonally formed stenosis at the point of origin from the pelvis is one of the most common causes of the formation of a defect. External reasons for the development of hydronephrosis include: the presence of an aberrant vessel, fibrous bands, high discharge of the ureter.

Prenatal diagnosis
Now, in most cases, hydronephrosis is diagnosed antenatally. If hydronephrosis is suspected on antenatal ultrasound, the examination should be repeated a few days after birth, as intrauterine hydronephrosis may be physiological and disappear by the time the baby is born.

Diagnostics
Ultrasound with hydronephrosis visualizes dilatation of the pelvis more than 20 mm with the expansion of all groups of cups, the parenchyma is thinned by more than 50%, increased echogenicity. Dopplerography of the renal vessels reveals a weakening of blood flow towards the periphery, the pressure in them is increased. The organic nature of the obstruction is confirmed by diuretic sonography. To exclude comorbidity, voiding cystourethrography is mandatory. Radionuclide renography reveals a decrease in kidney function by 40-50%. Magnetic resonance imaging also reveals an expansion of the pelvicalyceal system with signs of parenchymal atrophy.

Treatment
Hydronephrosis requires surgical treatment. Its timing depends on the degree of dysfunction of the kidney and signs of atrophy of the parenchyma. In the neonatal period, surgical intervention can be performed with severe dilatation of the pelvicalyceal system of the kidney, thinning of the parenchyma and a decrease in kidney function. The operation of choice, as a rule, is resection pyeloplasty according to Heinz-Andersen. In severe concomitant pathology and dysfunction of the affected kidney, the imposition of a puncture nephrostomy is indicated, followed by a radical operation to stabilize the patient's condition. In the case of a bilateral lesion, the reconstruction of the more affected kidney is performed first.

Forecast
With timely surgical correction of kidney function, as a rule, it is possible to save and restore. This is especially true in the case of bilateral lesions.

Megaureter
Megaureter - expansion and dysfunction of the ureter.

Epidemiology
Megaureter is one of the most common types of obstructive uropathy in children, accounting for 25-30% of their total number. A bilateral process is noted in 20-25% of patients, a left-sided lesion is detected 2.5 times more often. In boys, the defect is 3 times more common than in girls.

Etiology
Expansion of the ureter can be caused by both true obstruction (anatomical stenosis) in the distal region, and functional obstruction (violation of the muscular structure). Between these types of obstruction there are many intermediate stages, but the common thing for them is the absence of reflux, which emphasizes the localization of the process in the final part of the ureter. Along with this, massive reflux from the bladder into the upper urinary tract also causes a violation of urodynamics and is one of the forms of obstruction. That is why, in our opinion, the megaureter should be distinguished into refluxing and non-refluxing, indicating the functional state of the kidney and ureter.

A non-refluxing megaureter is an expansion of the ureter and the renal collecting system due to a violation of the outflow of urine in the distal ureter. The reason for the violation of urodynamics may be organic stenosis (ureterohydronephrosis) or disorientation of the muscle fibers of the distal ureter (functional disorders).

Etiology
Normally, the muscle tissue of the ureter has the character of a plexus due to the crossing of individual bundles in it, going in different directions, i.e. in fact, one layer is formed. In the distal section, longitudinal muscle fibers, fan-shaped and intertwining with the muscle fibers of the bladder and contralateral ureter, form a superficial trigonal layer. The coordinated work of the muscular apparatus of the ureter is ensured by the passage of the contraction wave. If the muscle wall is interrupted by collagen or scar tissue (organic stenosis), or if the muscle fibers lose their longitudinal orientation in the terminal section (functional form of obstruction), urodynamic disturbances of varying severity develop.

Diagnostics
In the process of diagnostic procedures, it is necessary to answer one question: is the obstruction in the zone of the ureterovesical segment organic or is it a manifestation of the functional immaturity of the ureter. For this, X-ray examinations are carried out. The functional immaturity of the ureter in the area of ​​the ureterovesical anastomosis is characterized by the following features: echographically, the kidney parenchyma is practically unchanged, the pelvis and calyces are not dilated, the ureter is dilated - as a rule, only in the distal section and peristaltizes well; gross changes in blood flow during Doppler sonography are not observed; there are no signs of vicarious hypertrophy of a healthy kidney and a decrease in the functions of a compromised kidney. The signs of organic obstruction are as follows: an ultrasound picture of gross violations in the structure of the kidneys; significant changes in the blood flow of the kidney during Doppler sonography; signs of vicarious hypertrophy of a healthy kidney; progressive decline in kidney function and ureter contractility.

Treatment
Functional disorders in non-refluxing megaureter respond well to conservative therapy aimed at improving the trophism and functions of the kidneys and maturation of the ureters. All children with a confirmed organic form of obstruction of the distal ureter require surgical correction of the defect.

Forecast
With a unilateral lesion, despite a more pronounced dysplasia of the renal tissue than with hydronephrosis, timely correction brings positive results.

Vesicoureteral reflux is a congenital anomaly in which the closing function of the ureterovesical fistula is impaired. This leads to a reverse flow of urine from the lower urinary tract to the upper ones, an increase in intraureteral and pelvic pressure, inhibition of the contractile activity of the ureter and pelvis, and pelvic-renal reflux.

Etiology
The cause of vesicoureteral reflux is a dysfunction of the vesicoureteral segment and, above all, the closure apparatus. An increase in intravesical pressure (during urination, in an upright position) causes resistance to the reverse flow of urine. In addition, the appearance of reflux can be explained by the weakness of the muscular wall of the bladder associated with caudal regression syndrome, malformations of the spine, anatomical defects of the bladder (diverticulum, ureterocele).

Antenatal diagnosis
Antenatal diagnosis of vesicoureteral reflux is based on sonographic signs of pyeloectasia, which has the character of unstable dilatation, the size of which varies depending on the filling-emptying phase of the bladder. Visualization of the ureter, which has similar patterns of change in cross-sectional dimensions or a transient pattern of expansion, increases the likelihood of diagnosing vesicoureteral reflux in the postnatal period.

Classification
Vesicoureteral reflux associated with congenital insufficiency of the vesicoureteral segment is primary, and developing against the background of obstruction (posterior urethral valve, bladder neck fibrosis) or neurogenic bladder dysfunction is secondary. Currently, an international classification based on a quantitative assessment of the degree of reflux is used, it distinguishes five degrees of vesicoureteral reflux. The international classification takes into account not only the level of backflow of urine and the presence of expansion of the ureter and renal pelvis, but also the anatomical changes resulting from reflux of the arch of the cups.

Diagnostics
The initial stage in the diagnosis of vesicoureteral reflux is ultrasound, which allows you to assess the size of the kidneys, the thickness of the parenchyma and the diameter of the ureter. With vesicoureteral reflux of I-III degrees, as a rule, no changes in the structure of the parenchyma, hemodynamics are detected, the ureter is determined in the lower third from 3 to 6 mm. At high IV-V degrees of reflux (refluxing megaureter), thinning of the parenchyma, expansion of the renal collector system and the ureter itself are noted. To assess the degree of vesicoureteral reflux, voiding cystourethrography is performed. If a bilateral refluxing megaureter is detected, it is necessary to perform cystourethroscopy - to exclude a malformation of the posterior urethra (valve) and to visualize the location and configuration of the orifices of the ureters. If a posterior urethral valve is detected, diagnostic cystourethroscopy turns into a therapeutic one: its electroresection is performed.

Treatment
If vesicoureteral reflux of I-III degree and there are no signs of reflux nephropathy, conservative treatment is carried out, consisting of the prevention of urinary tract infections, renoprotective therapy and physiotherapy aimed at improving urodynamics. Children with refluxing megaureter are shown to undergo surgical correction after the infectious process subsides, which is present to one degree or another in most patients with massive reflux.

Forecast
Since the lesion is usually bilateral, only careful monitoring and timely therapy will preserve kidney function.

11. Anomalies of the urinary system

Anomalies of the urinary system are the most common malformations. Some can lead to early death in childhood, others do not cause dysfunction of the urinary system and are found incidentally during an ultrasound examination or tomography, as well as an X-ray examination.

Some of the anomalies progress very slowly and can only become clinically apparent in old age. Some developmental anomalies can serve as a background for the development of another pathology (renal stones, chronic pyelonephritis, arterial hypertension).

The causes of anomalies may be hereditary predisposition, mother's diseases during pregnancy - rubella in the first months; ionizing radiation, syphilis, alcoholism, the use of hormonal drugs.

If an anomaly is found in one family member, then all family members should be examined.

Classification

There are the following groups of anomalies:

1) anomalies in the number of kidneys - bilateral agenesis (absence of kidneys), unilateral agenesis (single kidney), doubling of the kidneys;

2) anomalies in the position of the kidneys - momolateral dystopia (the lowered kidney is on its side); heterolateral cross dystopia (transfer of the kidney to the opposite side);

3) anomalies in the relative position of the kidneys (fused kidneys), horseshoe-shaped kidney, biscuit-shaped, S-shaped, L-shaped;

4) anomalies in the size and structure of the kidneys - aplasia, hypoplasia, polycystic kidney;

5) anomalies of the renal pelvis and ureters - cysts, diverticula, bifurcation of the pelvis, anomalies in the number, caliber, shape, position of the ureters.

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Anomalies in the development of the genitourinary system is a group of congenital diseases, a feature of which is an intrauterine malformation of the fetus, as a result of which one or more organs of the genitourinary system suffer. Diseases of this type are very common and account for approximately 40% of all developmental abnormalities that occur in utero.

Pathology of the bladder

Among congenital abnormalities, exstrophy of this organ is considered very serious and dangerous. This disease, like other anomalies in the development of the bladder, is often accompanied by other deviations of the entire system (they become pathologies of the formation of the kidneys, pubic bones or urethra). It is not without reason that such a disease is considered an extremely severe type of anomaly in the formation of the fetus inside the womb, because as a result of its development, the bladder does not have an anterior wall, and the same anterior abdominal wall is also missing, which causes incessant leakage of urine, as well as the appearance of ulcers on the surface of the perineum and genitals. This disease affects 1 out of 30-40 thousand newborns. Women are twice as likely to develop this pathology. In addition, if the first child was born with such an ailment, then in 1 case out of 100 the second will also suffer from a similar disease.

A very common congenital ailment associated with the organ under discussion is the diverticulum of the bladder. In this case, one of the walls will bulge outward. Diverticulum is not only congenital, but also acquired in adulthood. In addition, there are single and multiple diverticula (differing, respectively, in the number of protrusions), as well as false and true ones (the protrusion has all the layers characteristic of the wall of this organ according to its histology, and with a false one, there is a hernial protrusion of the mucous membrane, colliding with the muscular bladder tissue).

Bladder agenesis is also common. During the formation of this disease, the organ is not present at all in the body of the newborn. In a significant number of cases, such developmental abnormalities are accompanied by other anomalies, which together lead to death due to problems with the vital activity of the organism.

Bladder doubling is considered the absolute opposite of bladder agenesis. In such circumstances, which are quite rare, the organ develops with a partition inside, dividing it into two separate components. In both these cavities, an independent mouth of the ureter opens. In some cases, this defect is accompanied by the presence of urethra and necks in two copies. Interestingly, in rare cases, the septum does not fully develop, which serves to form the so-called “two-chamber bladder”.

But also the contracture of the bladder neck belongs to the diseases of this category. With the development of this scenario, too much fibrous tissue is formed in the bladder wall in the cervical region. Depending on the volume of this excess tissue, fibrosis resulting from cervical contracture can have varying degrees of severity. In mild cases, patients have only minor problems with urination. When fibrosis is in a severe stage, the process of leakage of fluid from the bladder is disrupted. The manifestation of such a deviation can be vesicoureteral reflux and the progression of inflammation (for example, urethritis) in the urinary system due to the penetration of a urogenital infection.

In addition, there are several types of urachus pathology. This organ is present in our body during fetal development and serves to connect the bladder and amniotic fluid. In a normal situation, by the time a person is born, the urachus is completely delayed. Otherwise, one of the pathologies associated with the release of urine through the opening of the umbilical cord may develop.

Anomalies of the urinary organs

The most common are malformations of the ureters. Diseases of this category account for approximately one-fifth of all ailments related to violations of the formation of the genitourinary system. Like other anomalies in the field of urology, these developmental disruptions are in many cases accidentally detected in children during examinations related to other diseases, and are also sometimes accompanied by a disorder in the functions of the renal system. Often, patients are diagnosed with a narrowing or expansion of the ureter. Despite the huge number of different types of anomalies associated with the ureter, they cause a violation of urodynamics (the movement of urine through the urinary system). Accordingly, interference with the flow of urine leads to the development of inflammatory processes in the kidneys (for example, cystitis), which is the cause of a violation of its functioning. In many cases, children are diagnosed with several anomalies of the ureters on both sides at once, which leads to irreversible changes in the kidney. Typically, these malformations of maturation are detected at the age of 6 to 10 years.

Anomalies in the development of the male genital organs are malformations of the genitourinary parts of the body. Most often, this category includes testicular abnormalities, with which 5 to 7 percent of babies are born. Among such deviations, several quantitative ones are distinguished: anorchism (in which both testicles are completely absent), monorchism (there is only one), polyorchism (the number is three or more). Among other anomalies in the development of the testicles, hypoplasia (underdevelopment) of this organ and cryptorchidism (does not descend into the scrotum) are noted. The last anomaly is considered the most frequent of all. Diseases in this category are monitored very closely by doctors, since their consequences determine whether a boy can have offspring as an adult.

Renal agenesis

Anomalies in the development of the kidneys is another type of malformation of the maturation of the genitourinary organs. Problems with their formation are typical for a third of all patients with urological ailments. Due to the frequent detection of such anomalies and the severity of the consequences of kidney diseases associated with malformations, doctors are increasingly attentive to signs that may indicate the presence of any problems. In many cases, timely treatment helps prevent the development of much more serious diseases and avoid serious, and sometimes irreversible consequences. In many cases, disorders of the development of the kidneys are accompanied by abnormal development of the renal vessels. Due to the fact that the kidneys with malformations have impaired blood circulation and urodynamics, chronic inflammatory processes begin to form in the body. This leads to the fact that kidneys with structural pathologies are more vulnerable to diseases such as pyelonephritis, hydronephrosis and nephrolithiasis. It is important to point out that in the absence of any symptoms and other noticeable manifestations of the disease, deviations are sometimes not detected throughout life.

In addition to deviations in the development of the kidneys, doctors distinguish problems with the formation of renal vessels. Pathologies of this type are common. The severity of such an ailment depends on whether it is accompanied by any kidney disease or whether it is the only type of anomaly of the genitourinary system. In some cases, abnormal renal vessels lead to various kidney ailments, however, the predominant number of such ailments does not make itself felt.

Causes of anomalies

Most often, diseases of the genitourinary system associated with developmental anomalies are due to a number of established factors. These include:

  • genetic predisposition (the disease is transmitted at the gene level);
  • infectious and venereal diseases suffered during pregnancy;
  • exposure to radiation, as well as ionizing radiation;
  • addiction to alcohol and tobacco products;
  • wrong diet;
  • contact with toxic substances;
  • the use of hormonal drugs.

If an anomaly in the development of the genitourinary system is detected, an examination of all members of the patient's family is required. In addition, consultation with a specialist will help clarify the situation and calculate the risks of repeating a similar scenario in future generations.

One of the most frequent deviations of maturation inside the womb, characteristic of the upper urinary tract, is their duplication. Mostly it is located on one side, in more rare cases it is present on both. In a situation with duplication of the upper urinary tract, two other malformations of the ureteral orifices are often present - these include ectopic orifice of the ureter and ureterocele. In general, pathologies of the upper urinary tract are divided into four fairly large groups:

  • anomalies associated with impaired communication of blood vessels with the upper part of the urinary tract;
  • partial dysplasia of the ureter wall;
  • ectopia of the mouths of the ureters;
  • ureterocele.

Moreover, congenital malformations of the urinary tract include all diseases associated with abnormalities in the development of the kidneys. Such a malaise, for example, is a doubling of the kidney. This often diagnosed anomaly is characterized either by duplication of some components of the kidney (pelvis, vessels or ureters), or all of these elements at once, which is called complete doubling of the kidney. In this case, both halves are usually considered independent organs, and the pathological process often affects only one of the organs.

The following developmental anomalies are characteristic of the kidneys: incorrect position and dystopia. These subtypes of deviations are divided into pelvic, iliac, lumbar, thoracic and cross. At the same time, patients with dystopia often have problems with bowel activity. In general, without certain clinical manifestations, these types of diseases can go unnoticed for a very long period of time.

One of the serious deviations in the formation of the urinary tract is an anomaly in the relationship of the kidneys, in which the organs grow together with each other. Depending on the type of fusion, they can take a different shape, changing the appearance of the organ to L-shaped, S-shaped and horseshoe-shaped. Kidneys fused with each other are easily exposed to inflammatory processes, and in addition, they can cause renal hypertension. Due to the development of other diseases caused by inflammatory processes (pyelonephritis, hydronephrosis, abnormal blood supply), arterial hypertension can also progress.

Another developmental anomaly that is of a serious nature is polycystic kidney disease. The disease is most often transmitted genetically and has a dominant type of inheritance. With this malaise, most of the kidney parenchyma is affected by a huge number of hollow tumors (cysts) of various sizes. In 7 out of 10 cases, a child whose organs are affected by polycystic disease is born dead. If the number of tumors is not so large, their performance (and hence the viability of the body) is preserved, but in many cases there is a high chance of developing kidney failure. Such a disease is often accompanied by polycystic disease of other parts of the body.

Another anomaly of a non-hereditary nature is the solitary cyst of the organ in question. Such a disease involves a single cyst in the kidney, which can be either congenital or acquired. With an increase in the size of the cyst, a violation of the renal functions occurs, while patients suffer from pain in the lower back.

The deviation, known as spongy kidney, is characterized by the formation of a large number of tumors in the renal pyramids. Such a disease usually does not lead to the development of organ failure.

Separately, it is worth considering a ureterocele - a protrusion, more like a hernia, inside the ureter. After all, it sometimes provokes the expansion of the upper urinary tract, pyelonephritis and urolithiasis.

Course of the disease

Polycystic. kidney cyst

The course differs for each individual anomaly in the development of the genitourinary system. Some of these deviations may not manifest themselves for a long time and are discovered quite by accident during examinations related to other ailments. Others become prerequisites for the development of urolithiasis, chronic renal failure and arterial hypertension. In specific cases, pathologies of the formation of the organs of the genitourinary system cause the death of a child in the womb or at a very early age, which is associated with a violation of the vital functions of the body. Sometimes such anomalies are characterized by slow progression, which ultimately affects the manifestation of symptoms of the disease in old age.

Diagnostics

When detecting malformations of the genitourinary system, doctors use differentiated diagnostic methods, which include:

  • radiography with the introduction of contrast fluid;
  • Magnetic resonance imaging;
  • CT scan;
  • retrograde urethrography;
  • insertion of a catheter into the ureter;
  • cystoscopy;
  • ultrasound procedure.

A timely diagnosis of diseases of this type, which is prescribed at an early age, is simply necessary. For example, with an undescended testicle, ultrasound helps to establish its location for sure, and taking contrast fluid when using X-rays makes it possible to determine the presence of kidney aplasia or bladder diverticulum.

Thanks to the timely diagnosis and necessary treatment, it is possible to avoid problems, both physical and psychological. For example, with a curvature of the penis, the anomaly causes problems with urination (more often found in a child), as well as a psychological barrier in the intimate sphere (typical for adult men). In addition, this disease causes infertility (which also leads to bilateral cryptorchidism). For example, cryptorchidism, with the diagnosis and treatment of which was delayed, causes testicular torsion.

Treatment of anomalies of the genitourinary system

Often, pathologies require surgical intervention in the body. Such operations vary in volume and complexity. With cryptorchidism (undescended testicle into the scrotum), the necessary surgical intervention can be performed both in childhood and in adulthood. Some medical interventions are prescribed exclusively in cases where traditional methods do not bring any results.

The most difficult to treat and correct are anomalies associated with hermaphroditism. In such cases, patients are advised to use surgery. Hormone therapy that corresponds to the gender definition of a person will not be superfluous.

During the formation of the fetus in the womb, various failures can occur, which are manifested by the presence of various anomalies of the genitourinary system. In such a situation, both one and several organs can be affected at the same time.

Such abnormalities can be detected by chance, since they do not cause any problems, or lead to the death of the baby before birth. They make up about 40% of all intrauterine anomalies.

Pathology of the bladder

Among the congenital diseases of the organ, anomalies are distinguished:

Some of the anomalies may never show up.

  1. Diverticulum. It is characterized by protrusion of the wall outward. In this case, accumulation and stagnation of urine occurs, which leads to various kinds of inflammation and the formation of stones. In some cases, diverticula exceed the size of the organ itself. They are congenital and acquired. Classify them into single and multiple, true and false. In the latter form, a herniated rounded formation of the mucous membrane is observed.
  2. Urachus anomaly. There is a stop of its formation in the prenatal period, which affects the process of its overgrowth. This can cause the formation of a fistula, from which urine or pus will be released, as well as cysts.
  3. . One of the dangerous deviations, since its occurrence leads to death due to the absence of an organ.
  4. Exstrophy. There are no anterior walls of the bladder, urethra and abdominal cavity, therefore, in the lower abdomen there is a hole with a diameter equal to several centimeters. It shows the back of the bladder and the mouth of the ureter, through which urine is excreted to the outside. This causes a lack of cartilaginous skeletal formation and a medial location of the hips. This causes urine to flow down them, the development of ulcers and inflammation. If a pathology occurs in the first child, it manifests itself in the second.
  5. Doubling. In this case, the bladder is divided by a septum into 2 halves, each of which has its own ureter. Pathology is rarely diagnosed. Distinguish between complete and incomplete doubling. At the first, the urethra and bladder neck double, which in 50% of cases is combined with a vaginal fistula and a doubled rectum. The second type of pathology is characterized by a two-chamber bladder.
  6. Neck contracture. Pathology is also rare and occurs with increased growth of fibrous tissue in the submucosal and muscular layers.

Anomalies of the genitourinary system

Pathologies of the ureters are recorded in a fifth of the population and are not detected immediately, but as a result of diagnostic measures:

  • Doubling. With a complete type, they intersect and open with mouths into the bladder, and with an incomplete type, they merge.
  • Ureterocele. Accompanied by protrusion of the most distal part of the ureter into the bladder.
  • neuromuscular dysplasia. It is characterized by underdevelopment of the neuromuscular apparatus and a narrow intromural section.
  • Retrocaval ureter. Diagnose the location of the middle part of the ureter (mostly right) behind the inferior vena cava.
  • The ectopia of the mouth implies its uncharacteristic location.
  • Spiral ureter.

The following deviations in the development of the urethra:

  • Congenital valves are single and multiple folded formations in the form of bridges within the organ.
  • Hypertrophy of the seminal tubercle. A rare anomaly that can lead to complete occlusion of the urethral lumen.
  • congenital narrowing.
  • Hypospadias. There is no posterior wall of the canal or its partial absence. According to the location of the opening for excretion, pathologies such as scrotal, stem, perineal and capitate are distinguished (50% of cases).
  • Epispadias. It is a non-closure of the anterior wall of the urethra. It happens total, stem and capitate (boys), clitoris (girls).

Deviations in the formation of the kidneys are classified depending on the number, size, location, structure and relationship with the organs. Diagnosed in 30% of patients with urological diseases.

In the third month of pregnancy, abnormalities in the development of the fetus can be detected

There are such types of disorders:

  • Agenesis is the complete absence of the kidneys. It is combined with anomalies of the male genital organs.
  • Aplasia. The absence of the pelvis, which does not allow it to produce urine. If the pathologies are bilateral in nature, then they lead to the death of the fetus.
  • Doubling. The full appearance of the anomaly implies the presence of pyelocaliceal systems in each part of the kidney, in the upper it is underdeveloped, and ureters. Doubling of the vessels and parenchyma of the kidney is an incomplete form.
  • The presence of a third kidney. Its value is less than that of the usual one, but this does not affect the functioning.
  • Hypoplasia. Due to the small caliber of the renal artery, it produces a smaller volume of urine.
  • Dystopia is the incorrect location of the kidneys relative to each other or the skeleton.
  • Fusion of the kidneys with the upper, lower (horseshoe-shaped) or opposite (L or S-shaped) poles. The biscuit-like form is observed when accreting along the inner rib.
  • With dysplasia, there is a decrease in the size of the organ and a non-standard structure of the parenchyma (rudimentary and dwarf).
  • Multicystic. The parenchyma is replaced by cysts, there is no renal artery, the ureter is closed at one end.
  • Spongy kidney. Bilateral pathology is associated with the formation of cysts.

Among the anomalies associated with changes in the structure of the kidneys, polycystic, solitary and dermoid cysts, diverticulum of the renal pelvis are distinguished. The first is the most common and is the cause of the development of renal failure.

Also, doctors separately classify the pathology of the formation of renal vessels. They differ in the number, shape and location of these veins and arteries, and can cause the development of dangerous diseases.

Deviations in the formation of the reproductive system

For boys:

  • narrowing of the foreskin (phimosis);
  • short bridle;
  • abnormal location of the testicle (cryptorchism and ectopia);
  • anorchism (lack of two testicles), monorchism (1 testicle) and polyorchism (3 or more testicles);
  • testicular hypoplasia - sizes less than 1 cm.

With the help of diagnostic methods, you can detect the problem in time

For girls:

  • abnormal structure of the vaginal septum;
  • infantilism - delayed development of the genital organs;
  • double uterus or vagina;
  • the hymen is devoid of an opening;
  • abnormal anatomical structure of the uterus (one- or two-horned, saddle-shaped).

These diseases cause infertility, and women develop an inability to bear a fetus.

Symptoms of kidney anomalies

The main signs of pathologies:

  • painful urination, urinary incontinence;
  • pain in the left (right) or both sides, pelvic region, with chest dystopia, they occur in the abdomen after eating;
  • the work of the intestines and stomach, blood supply to organs is disrupted;
  • bleeding;
  • increased blood pressure and body temperature;
  • frequent cold infections;
  • nausea and vomiting;
  • swelling of the lower extremities;
  • cloudy urine with impurities of blood or pus;
  • high levels of creatinine and urea in the blood.

Many symptoms are manifested by the addition of infections and the development of such renal pathologies as: pyelonephritis, urolithiasis.

Causes of anomalies

Physicians have established a number of factors that affect the pathological development of the organs of the genitourinary system in the fetus:

Types of kidney anomalies

  • heredity;
  • diseases of an infectious or venereal nature in the mother during pregnancy;
  • excessive use of drugs, alcohol and nicotine;
  • radiation exposure or poisoning with toxic poisons;
  • abuse of hormonal drugs;
  • harmful professional activity.

Diagnostics

Not every anomaly is noticeable at the birth of a child, with the manifestation of symptoms or suspicion of deviations in the formation of the genitourinary system, it is necessary to undergo a series of examinations:

The operation can get rid of the problem. This is the most reliable way to treat pathologies

  • x-ray with the introduction of a contrast agent;
  • cytoscopy and ureteroscopy;
  • excretory urography;
  • selective renal arteriography;
  • angiography;
  • retrograde pyelography or cystography;
  • Ultrasound of the pelvic organs;
  • CT or MRI;
  • scintigraphy;
  • fistulography.

Some anomalies can be determined by the doctor after examining the child, but most are detected during examinations for the presence of a disease of the genitourinary system.

Treatment of anomalies of the genitourinary system

Due to the fact that most pathologies affect the normal outflow of urine, the main method of treatment is surgery. Some operations are carried out only at a certain age, others are prescribed when medical treatment is ineffective. Such treatment does not always bring a 100% solution to the problem, sometimes repeated surgical interventions may be required. With anomalies of the genital organs, hormonal preparations are prescribed.

In the postoperative period, the patient must comply with all doctor's prescriptions, come to the examination in a timely manner, take tests to check the functioning of the kidneys.

Anomalies in the development of organs lead to the death of the fetus, if the identified pathology is not fatal, then it requires timely therapy.

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