Complications of anomalies of the genitourinary system presentation. Anomalies in the development of the organs of the genitourinary system Department of Urology BSMU

Frequency: occur in a population of approximately 1%, are diagnosed prenatally in approximately 0.2% of children.

Attention: 30% of prenatal diagnoses are not confirmed, so follow-up after delivery is necessary.

Forms:

Hydronephrosis: Impaired emptying of the bladder (eg, urethral valve).
Polycystic kidney disease.
Congenital megaloureter, doubling.
Vesicoureteral reflux.
Agenesis/aplasia of the kidneys.
Prune-Belly syndrome (syn.: aplasia of the membranes of the abdominal wall, plum belly syndrome),
Bladder exstrophy.

Complications and problems: severe and prolonged intrauterine obstructive uropathy leads to kidney shrinkage (sometimes already in utero).

First aid if you suspect a defect in the urinary system:

Forced postnatal diagnosis.
Diuresis count and urine collection. Urine in the first 24 hours is excreted in 92% of mature and 90% of immature newborns, within 48 hours - in 99%.
With the valve, if necessary, a suprapubic puncture of the bladder is performed (unloading the bladder and kidneys).

Attention: on the part of the kidneys, there are practically no emergency conditions in the delivery room, however, there may be lung hypoplasia (with oligo- or ahydramnios). With kidney disease, spontaneous pneumothorax can occur even on spontaneous breathing.

First aid with exstrophy of the bladder:

Immediately put on a sterile diaper.
Humidification of eventriated parts.
Constant assessment of the eventrated bladder: color, hemorrhages, edema.
Wrapping eventriated tea! in a sterile plastic bag or moisturizing with sterile vaseline.
Don't forget to weigh your baby.
Emergency (up to 24-48 hours) operation.
Examination of the proximal urinary tract.

hypospadias

Definition: A malformation of the penis with incomplete development of the urethra and an abnormally located opening on the underside of the penis, on the scrotum, or on the perineum. Malformation of the spongiform urethra with or without curvature, ventral foreskin defect, and dorsal foreskin hypertrophy.

Tactics depends on the location of the external opening:

Capitate, stem, scrotal, perineal forms.
With or without stenosis.
With or without head deformation.
"Hypospadias without hypospadias" is a curvature without significant dislocation of the foramen.

Associated with vices:

Cryptorchidism and inguinal hernia - about 9% of cases.
Utriculus prostaticus masculinus - about 11% of cases (with severe forms).
Kidney and urinary system defects - 3%.

Differential diagnosis with intersexuality, hypogenitalism; if necessary, determination of the karyotype.

Treatment:

Correction is indicated in all cases of curvature of the trunk, stenosis of the foramen, and localization of the foramen proximal to the coronary sulcus.
With distal forms, indications are determined by aesthetic disorders, but not in the newborn period!

Hydrocele (dropsy)

Hydrocele (dropsy): Persistence of an open processus vaginalis can lead to accumulation of peritoneal fluid in it.

Definition: A fluid-filled cyst in the area of the testicles or spermatic cord.

Forms:

Dropsy cord.
Dropsy of the testicle: the testicle is most often not palpable.
Inguinal-scrotal dropsy: the upper pole of the cyst reaches the abdominal cavity. A dense formation is palpated medially to the inguinal ligament in the small pelvis. When pressing on the scrotum, its volume increases.
With communicating dropsy (changes in size), there is a connection with the abdominal cavity. The clinic and treatment corresponds to an inguinal hernia.
Nukov's canal cysts (in girls): circumscribed collections of fluid in the area of the round ligament outside the inguinal canal.

Clinic:

Asymptomatic unilateral or bilateral tumor-like formations in the testicles of various sizes.
Often occurs in newborns. Spontaneously regresses during the first 3-4 months of life.
They may appear spontaneously for no apparent reason outside the neonatal period, and may also regress.
After the first year of life, dropsy occurs in less than 1% of boys.

Diagnostics:

Examination, palpation.
Diaphanoscopy does not allow distinguishing a hydrocele from a strangulated hernia.

Differential diagnosis: strangulated hernia, scrotal hernia, varicocele, testicular torsion.

Important: testicular torsion can also be congenital; This is an indication for immediate surgery.

Treatment:

The operation is indicated after six months with persistent hydrocele.
In the first 3 months, the operation is performed only with a large size, a rapid increase in volume and an inguinal-scrotal hydrocele.

Inguinal hernia

It is formed due to the persistence of the vaginal process of the peritoneum. A sufficiently large hole size allows the intestinal loops to penetrate into the appendix.

Definition: parts of the intestine exit the abdominal cavity through a congenital or acquired opening (hernial orifice), surrounded by the parietal peritoneum. The content of the hernia is limited to the hernia membranes (subcutaneous tissue, skin or scrotal wall). Children's inguinal hernias are always indirect. They form along the inguinal canal (persistence of processus vaginalis).

Clinic:

More commonly, asymptomatic, soft, reducible tumors in the groin medial to the inguinal ligament (inguinal hernia) that may reach the scrotum (scrotal hernia).
When infringed, the hernia becomes painful and irreducible.

Diagnostics:

Clinical difference from a hydrocele: with an inguinal hernia, the hernial sac is palpated above the inguinal ring, and with a hydrocele it does not reach it. In doubtful cases, treatment is always carried out as with a strangulated hernia.
Sonography.

Differential diagnosis: testicle in the inguinal canal, lymphadenitis, dropsy of the testicle or cord, testicular torsion, varicocele.

Complications:

Potentially life-threatening condition.
Intestinal strangulation or peritonitis.
Loss of the testicle/ovary or part of the intestine.

Infringement: in about 12% of cases, but 70% - in the 1st year of life:

Clinic: sudden onset of the disease, severe pain, anxiety, symptoms of peritoneal irritation.
Findings: elastic, tense, inactive inguinal or inguinal-scrotal tumor.

Treatment and indications for surgery:

The reduction of an inguinal hernia often occurs on its own.
Active reduction (under sedation) is possible no later than 6-8 hours from the onset in the absence of shock symptoms
At unsuccessful reposition urgent operation is shown.
Elective surgery after successful reduction.
In all other cases, early hernia repair is optimal so that the risk of anesthesia does not increase.

Umbilical hernia

Umbilical hernia: a defect in the fascia of the umbilicus covered by skin. Surgical intervention is required very rarely, since the defect closes over time (at 1 year of life). Infringement or damage to the skin due to pressure is extremely rare.

Introduction

The urinary system is a collection of organs that produce and excrete urine. The urethra is the tube that carries urine from the productive areas of the kidneys to the bladder, where it is stored and then expelled to the outside through a channel called the urethra. With congenital abnormal development (anomalies) of the urinary system, either the production or excretion of urine is impaired.

Urinary system defects vary in severity from minor to life-threatening. Most are serious, requiring surgical correction. Other defects do not cause dysfunction of the urinary system, but make it difficult to control urination.

The greatest number of complications occurs with a mechanical obstruction to the outflow of urine; urine stagnates or returns (is thrown) into the upper urinary system. Tissues in the area of mechanical obstruction swell and, as a result, tissues are damaged. The most serious damage to the renal tissue, leading to impaired renal function.

Obstruction (mechanical obstruction) is a relatively rare pathology. Boys get sick more often. In girls, the obstruction is more likely to occur at the branch of the urethra from the kidney or between the urethra and the bladder wall; in boys - between the bladder and the urethra. Obstruction in the upper urinary tract is more often on the right side.

Among the complications of obstruction, infectious processes and the formation of kidney stones are the most common. Surgical intervention is indicated to avoid their development. Surgical interventions in infancy are more successful. In the absence of surgery up to 2-3 years, kidney damage and impaired function are inevitable.

Another type of developmental anomaly is the absence or duplication of some organs of the urinary system, their incorrect location, the presence of extra holes. In second place among the anomalies is exstrophy (defect of the bladder, anterior abdominal wall, umbelical ligaments, pubic region, genitals or intestines) and epispadias (defect of the penis and urethra).

Most children who develop urinary tract obstruction are born with structural abnormalities of the urinary tract; there may be overgrowth of tissue in the urethra or a pocket in the bladder, an inability of any part of the urethra to move urine to the bladder. Possible damage to the urethra in trauma to the pelvic organs.

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

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

Quality anomalies

urogenital agenesis kidney dystopia

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

It is believed that most often anomalies of the urinary system occur due to the influence of hereditary factors and various negative effects on the fetus during fetal development. Anomalies of the urinary system can develop in a child due to rubella and syphilis transferred by the mother in the first months of pregnancy. Alcoholism and drug addiction of the mother, her use of hormonal contraceptives during pregnancy, as well as medications without a doctor's prescription can provoke the occurrence of anomalies.

Anomalies of the urinary system are divided into the following groups:

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

Ø 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);

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

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

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

Many of these anomalies cause the development of nephrolithiasis, inflammation (pyelonephritis), arterial hypertension.

The influence of various forms of anomalies of the urinary system on the child's body can be expressed in different ways. If some disorders most often lead to intrauterine death of a baby or his death in infancy, then a number of anomalies do not have a significant effect on the functioning of the body, and are often detected only by chance during routine medical examinations.

Sometimes an anomaly that does not disturb a child can cause serious functional disorders in adulthood or even old age.

It is believed that the risk of developing such disorders is highest in the first months of pregnancy, when the main organs are laid, including the urinary system. The expectant mother should not take any medication without a doctor's prescription. In case of colds and other diseases in which there is a high temperature and intoxication, you should immediately go to the hospital.

When planning a pregnancy, young parents are advised to undergo a medical examination, which will exclude various diseases that lead to abnormalities in the fetus. If there have already been cases of anomalies in the family, a consultation with a geneticist is necessary.

Cloacal exstrophy

Exstrophy (eversion of a hollow organ outward) of the cloaca is a defect in the development of the lower part of the anterior abdominal wall. (The cloaca is the part of the germ layer from which the abdominal organs eventually develop.) A child with cloacal exstrophy is born with multiple defects in internal organs. Part of the large intestine is located on the outer surface of the body, on the other side there are two halves of the bladder. In boys, the penis is short and flat; in girls, the clitoris is split. Cases of such a gross anomaly occur: 1 in 200,000 live births.

Despite the severity of the defect in cloacal exstrophy, newborns are viable. The bladder can be reconstructed surgically. The lower colon and rectum are underdeveloped, so a small stool receptacle is surgically created from the outside.

Bladder exstrophy

Bladder exstrophy is a congenital anomaly of the urinary system, characterized by eversion of the bladder outward from the abdominal wall. This pathology occurs in 1 out of 25,000 children, in boys 2 times more often than in girls.

In all cases, exstrophy of the bladder is combined with an anomaly of the external genitalia. Epispadias occurs in boys, in 40% of boys with exstrophy of the bladder, the testicles are not descended into the scrotum, the penis is short and flat, thicker than usual, attached to the outer abdominal wall at the wrong angle.

In girls, the clitoris is split, the labia (protective skin folds around the vaginal and urethral openings) may be widely separated, and the vaginal opening may be very small or absent. Most girls with this pathology are capable of conceiving a child and giving birth naturally.

Exstrophy of the bladder in both boys and girls is combined, as a rule, with an anomaly in the location of the rectum and anus - they are significantly displaced forward. The prolapse of the rectum is a consequence of its location, when it can easily slip out and also be easily reduced. Bladder exstrophy may be associated with a low navel and lack of cartilage that connects the pubic bones. The latter circumstance usually does not affect gait.

The progress of surgical technique makes it possible in most cases to reliably correct this type of developmental defects.

epispadias

Epispadias is a developmental defect characterized by an abnormal location of the opening of the urethra. In boys with epispadias, the opening of the urethra is located on the upper side of the penis, at the root, where the anterior abdominal wall begins. In girls, the opening of the urethra is normally located, but the urethra is widely opened. Epispadias is often associated with bladder exstrophy. As an isolated defect, epispadias occurs in 1 out of 95,000 newborns, in boys 4 times more often than in girls.

Pyelectasis

Pyelectasis is an expansion of the renal pelvis. The explanation of the origin of the term "pyeloectasia" is quite simple. Like most complex medical names, it comes from Greek roots: pyelos - "trough", "tub", and ektasis - "stretching", "stretching". It is clear with stretching, but with what is called a "tub", you need to figure it out.

Pyelectasis is one of the most common anomalies of the structure that are detected by ultrasound examination of the urinary system. An ultrasound doctor usually does not have specialized information in the field of nephrology, he works in his area of competence, therefore, in his conclusion there is the phrase: “A consultation with a nephrologist is recommended,” and you get an appointment with a nephrologist. Most often, pyelectasis is found during ultrasound during pregnancy, before the birth of the baby, or in the first year of life. Therefore, it will not be a big mistake to attribute the expansion of the pelvis to the innate features of the structure.

But the expansion of the pelvis may occur later. For example, at the age of 7, during the period of intensive growth of the child, when there is a change in the arrangement of organs relative to each other, it is possible that the ureter is clamped by an abnormally located or additional vessel. In adults, the expansion of the pelvis may be due to the blockage of the lumen of the ureter by a stone.

Causes of pyelectasis: expansion of the pelvis occurs when there is an obstruction (difficulty) in the outflow of urine at any stage of its excretion. Difficulty in the outflow of urine may be due to:

· with any problems associated with the ureters, such as: developmental anomaly, kink, compression, narrowing, etc.;

· with permanent or temporary (with improper preparation for ultrasound) overfilling of the bladder. With constant overfilling of the bladder, the child goes to urinate very rarely and in large portions (one of the types of neurogenic bladder dysfunction);

· with the presence of an obstacle in the passage of urine into the bladder from the ureter or when it is removed through the urethra;

· with blockage of the ureter with a stone, tumor or clot of pus (more often in adults);

· with some physiological, i.e. normal processes in the body (for example, excessive: fluid intake), when the urinary system simply does not have time to remove all the absorbed fluid;

· with a normal, but rarer type of location of the pelvis, when it is not inside the kidney, but outside it;

· reflux of urine from the bladder back into the ureters or kidney (reflux);

· with an infection of the urinary system due to the action of bacterial toxins on the smooth muscle cells of the ureters and pelvis. According to researchers, in 12.5% of patients with pyelonephritis, the pelvicalyceal system expands. After treatment, these changes disappear;

· with a general weakness of the muscular apparatus during prematurity of the baby (muscle cells are part of the ureters and pelvis);

· with neurological problems.

Pyelectasis is curable if correctly diagnosed and adequately treated. Another thing is that in some cases, with pyeloectasia, an independent recovery is possible, associated with the growth of the baby, a change in the position of the organs relative to each other and the redistribution of pressure in the urinary system in the right direction, as well as with the maturation of the muscular apparatus, which is often underdeveloped in premature babies.

The first year of life is the period of the most intensive growth: organs grow at a tremendous speed, their position relative to each other changes, and body weight increases. The functional load on organs and systems is growing. That is why the first year is decisive in the manifestation of most malformations, including malformations of the urinary system.

Less intense growth, but also significant for the manifestation of developmental anomalies, is noted during the period of the so-called first stretching (6-7 years) and in adolescence, when there are sharp increases in height and weight and hormonal changes. That is why pyeloectasia detected in utero or in the first months of life is subject to mandatory observation in the first year of life and during the listed critical periods.

Should be of concern:

· the size of the pelvis is 7 mm or more;

· change in the size of the pelvis before and after urination (during ultrasound);

· change in size throughout the year.

Quite often, after discovering in a child after 3 years the size of the pelvis is 5-7 mm and observing it for a year or two, experts come to the conclusion that this is just an individual deviation from the generally accepted norms of the structure, which is not associated with a serious problem.

The question is completely different if this deviation is determined in the baby in utero or immediately after birth. If the size of the pelvis in a child in the 2nd trimester of pregnancy was 4 mm, and in the 3rd trimester - 7 mm, constant monitoring is required. Although it must be said that in most babies, the expansion of the pelvis disappears after birth. Accordingly, there is no need to worry, but it is simply necessary to be observed.

Pyelectasis is primarily associated with an increase in pressure in the pelvis, which cannot but affect the tissue of the kidney adjacent to it.

Over time, part of the kidney tissue under the influence of constant pressure is damaged, which, in turn, leads to a violation of its functions. In addition, due to the high pressure in the pelvis, the kidney requires additional efforts to excrete urine, which “distracts” it from direct work. How long will the kidneys be able to work in such an enhanced mode?

With low-grade pyelectasis (5-7 mm), control ultrasound of the kidneys and bladder is performed at intervals of 1 time in 1-3 months. (frequency is determined by a nephrologist) in the first year of life, and in older children - 1 time in 6 months.

When an infection is attached and (or) with an increase in the size of the pelvis, an X-ray urological examination is performed in a hospital. Usually it is excretory urography, cystography. These surveys allow you to establish the cause of pyelectasis. Of course, they are not absolutely harmless and are carried out strictly if there are indications and by the decision of the supervising doctor - a nephrologist or urologist.

There is no single, universal treatment for pyelectasis, it depends on the established or suspected cause. So, if there is an anomaly in the structure of the ureter and (or) with a sharp increase in the size of the pelvis, your baby may need surgical (surgical) treatment aimed at eliminating the existing obstruction to the outflow of urine. In such cases, the wait-and-see approach adopted by some parents may result in the loss of a kidney, although it can be saved.

In the absence of a sharp deterioration and visible disturbances (according to ultrasound, urinalysis, etc.), another tactic may be proposed: observation and conservative treatment. Usually it includes physiotherapy, taking (if necessary) herbal preparations, ultrasound control.

Let's summarize:

· Pyelectasis is not an independent disease, but can only serve as an indirect sign of a violation of the outflow of urine from the pelvis as a result of any anomaly in the structure, infection, reflux of urine, etc.

· During the period of intensive growth, mandatory monitoring of changes in the size of the pelvis is required. The frequency of follow-up examinations is determined by the nephrologist.

· Pyelectasis may be the result of a urinary tract infection and, conversely, may itself contribute to the development of inflammation.

· With general immaturity of the body (in premature babies or babies with CNS problems), the size of the pelvis may return to normal as problems with the CNS disappear. In this case, the terms "pelvic hypotension" or "agony" are sometimes used.

· Pyelectasis requires mandatory observation by a nephrologist and ultrasound control.

· In most cases, pyelectasis is transient, that is, a temporary condition.

· Treatment is prescribed depending on the cause of pyelectasis, together with a nephrologist (urologist).

Increased renal mobility and nephroptosis

Anomalies in the development of the kidneys may not make themselves felt, but may be manifested by persistent pain in the abdomen. Finding the source of the problem can be difficult even for a specialist, since in many cases nothing can be found during the examination, and all tests are normal. But thanks to ultrasound, it became possible to quickly and painlessly detect violations in the functioning of the kidneys, although they are often detected by accident.

Currently, increased kidney mobility and nephroptosis (more pronounced kidney mobility) are often diagnosed. As the name suggests, the problem is due to excess movement of the kidney.

Normally, the kidneys can make a certain amount of movement during breathing, moreover, the lack of such mobility of the kidneys can be a sign of a serious illness.

The kidneys are located behind the peritoneum, coming closest to the surface of the body from the back. They lie in a special fat pad and are fixed with ligaments. Why has the number of cases of excessive mobility of the kidneys and nephroptosis increased so much? The point, of course, is not only that new methods of examination have appeared, in particular ultrasound. Most often this problem occurs in thin children and adolescents. It is in them that adipose tissue, which creates a bed for the kidney, is practically absent, just as there is little of it throughout the body. Therefore, first of all, it is necessary to find out the cause of low body weight in a child, especially if he complains of headaches, fatigue, etc. In teenage girls, a sharp decrease in body weight is often associated with a desire to be like supermodels: girls go on diets, almost they don’t eat anything, although it is during the period of hormonal changes in the body that fasting is extremely harmful. Often, the appearance of nephroptosis is associated with a sharp jump in growth at 6-8 and at 13-17 years old, when enhanced nutrition is required.

Increased mobility of the kidneys is most often manifested by discomfort and (or) heaviness in the lumbar region, periodic headaches.

Nephroptosis is a more pronounced form of mobility. There are 3 degrees of kidney mobility. With the most pronounced III degree, the kidney is located at the level of the bladder or slightly above it.

The child complains of frequent and persistent abdominal pain! In addition, improper outflow of urine from the kidneys leads to infection of the urinary system. Often, with nephroptosis, there are pressure surges, and therefore adolescents are often diagnosed with "vegetative-vascular dystonia". Although, in fact, the increase in pressure is due to the constant stretching of the vessels that feed the kidneys. In addition to everything, a highly mobile kidney itself is poorly supplied with blood and tries to compensate for the impossibility of working during the day at night, when it gets into its normal place, and therefore more urine is produced at night than necessary.

Since a highly mobile kidney changes its location, including periodically returning to its “original position”, it is necessary to conduct an extended ultrasound of the kidneys with the determination of their mobility: examination of the patient in the supine position, then standing, and in some cases after physical exertion (for example, after a series of jumps).

If ultrasound showed the presence of nephroptosis, an X-ray urological examination is required to determine the degree of nephroptosis and possible anomalies in the structure of the urinary tract.

Quantity anomalies

Renal agenesis

The mention of kidney agenesis is found in Aristotle: he wrote that an animal without a heart cannot exist in nature, but they meet without a spleen or with one kidney. The first attempt to describe aplasia in humans belongs to Andreas Vesalius in 1543. In 1928, N. N. Sokolov revealed the frequency of aplasia in humans. As a result of his research, he analyzed 50198 autopsies and found renal agenesis in 0.1% of cases. According to him, the frequency of occurrence does not depend on the gender of the person. Modern scientists, based on a fairly large sample, give slightly different numbers. According to their data: the incidence of agenesis is 0.05%, and it occurs three times more often in males.

general information

Agenesis (aplasia) of the kidneys is a malformation of an organ during embryogenesis, as a result of which one or both kidneys are completely absent. The rudimentary structures of the kidney are also absent. The ureter at the same time may be developed almost normally or absent altogether. Agenesia is a common malformation and occurs not only in humans, but also in those animals that normally have two kidneys.

There is no reliable evidence that agenesis is a hereditary disease that is transmitted from parent to child. Quite often, the cause of this disease is multisystem malformations due to exogenous influences during the embryonic stage of fetal development.

Along with agenesis, other malformations of the genitourinary system are often encountered, provided that the ureter and vas deferens are completely absent on the same side. Often, along with agenesis in the female, malformations of the female genital organs are also found, which have a general underdevelopment. The urinary system and the female reproductive system develop from different rudiments, so the simultaneous appearance of these defects is irregular. From all of the above, there is reason to believe that renal agenesis is a congenital and not hereditary defect, and is the result of exogenous influences in the first six weeks of fetal development. Mothers with diabetes are a risk factor for developing agenesis.

Types of kidney agenesis:

Bilateral renal agenesis

This defect belongs to the third clinical type. Newborns with this defect are mostly born dead. However, there have been cases when a child was born alive and full-term, but died in the first days of his life due to renal failure.

To date, progress does not stand still, and there is a technical possibility of transplanting a kidney to a newborn and performing hemodialysis. It is very important to timely differentiate bilateral renal agenesis from other malformations of the urinary tract and kidneys.

Unilateral renal agenesis

Unilateral renal agenesis with preservation of the ureter

This defect belongs to the first clinical type and is congenital. With unilateral aplasia, the entire load is taken by a single kidney, which in turn is often hyperplastic. An increase in the number of structural elements allows the kidney to take on the functions of two normal kidneys. The risk of severe consequences in case of trauma to one kidney increases.

Unilateral renal agenesis with no ureter

This defect manifests itself at the earliest stages of the embryonic development of the urinary system. A sign of this disease is the absence of the ureteral orifice. Due to the structural features of the male body, renal agenesis in men is combined with the absence of a duct that removes seminal fluid and changes in the seminal vesicles. This leads to: pain in the groin, sacrum; painful ejaculation, and sometimes to sexual dysfunction.

Treatment of renal agenesis

The method of treating a kidney depends on the degree of impairment of the functionality of the kidney. The most commonly used surgery is kidney transplantation. Along with surgical methods, there is also antibiotic therapy.

Doubling of the kidney

According to sectional statistics, it occurs in 1 case per 150 autopsies; 2 times more common in women than in men. It can be unilateral (89%) or bilateral (11%).

Causes of duplication of the kidney:

Kidney duplication occurs when two foci of differentiation induction are formed in the metanephrogenic blastema. In this case, two pelvicalyceal systems are formed, but the complete separation of the blastema does not occur, and therefore the kidney is covered with a common fibrous capsule. Each of the halves of the doubled kidney has its own blood supply. The renal vessels may depart separately from the aorta, or they may depart in a common trunk, dividing at or near the renal sinus. Some intrarenal arteries pass from one half to the other, which can be of great importance during kidney resection.

Kidney duplication symptoms

More often the upper half is underdeveloped, very rarely both halves are functionally identical or the lower half is underdeveloped. The underdeveloped half in its morphological structure resembles kidney dysplasia. The presence of parenchymal renal dysplasia in combination with impaired urodynamics due to splitting of the ureter creates the prerequisites for the occurrence of diseases in the abnormal kidney. Most often, the symptoms of duplication of the kidney duplicate the symptoms of the following diseases: chronic (53.3%) and acute (19.8%) pyelonephritis, urolithiasis (30.8%), hydronephrosis of one of the halves (19.7%). Doubling of the kidney can be suspected with ultrasound, especially with dilatation of the upper urinary tract.

Diagnosis of duplication of the kidney

Excretory urography helps to diagnose duplication of the kidney. However, the most difficult task is to determine the complete or incomplete doubling. The use of magnetic resonance urography and MSCT greatly simplifies this task, but does not solve it completely. The presence of an ureterocele is a factor that makes it difficult to diagnose complete or incomplete doubling of the kidney. Cystoscopy in the vast majority of cases helps to establish the diagnosis<#"justify">Dystopia

Dystopia - the location of an organ, tissue or individual cells in an unusual place for them, due to dysembryogenesis, trauma or surgery.

Kidney dystopia heterolateral cross (d. renis heterolateralis cruciata) - congenital D. kidney with its location on the opposite side, next to the second kidney.

Dystopia of the kidney homolateral (d.renis homolateralis) - congenital D. of the kidney with its location above or below normal.

Dystopia of the kidney chest (d. renis thoracica) - D. kidney with congenital diaphragmatic hernia with its location in the chest cavity subpleural.

Iliac dystopia of the kidney (d. renis iliaca) - homolateral D. of the kidney with its location in the large pelvis.

Kidney dystopia lumbar (d.renis lumbalis) - homolateral D. of the kidney with its location in the lumbar region below normal.

Kidney dystopia pelvic (d. renis pelvina) - homolateral D. p. with its location in the small pelvis.

Literature

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

Sapin M.R. - ANATOMY AND PHYSIOLOGY OF THE HUMAN with the age characteristics of the child's body. - publishing center "Academy" in 2005

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

N. V. Krylova, T. M. Soboleva Genitourinary apparatus, anatomy in diagrams and drawings, publishing house of the Peoples' Friendship University of Russia, Moscow, 1994.

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The meaning and methods for determining individual compatibility (av0) and Rh compatibility. biological compatibility. Responsibilities of a Blood Transfusion Physician.

Classification of adverse effects of blood transfusions

Water-electrolyte disorders in surgical patients and principles of infusion therapy. Indications, dangers and complications. Solutions for infusion therapy. Treatment of complications of infusion therapy.

Trauma, injury. Classification. General principles of diagnostics. stages of assistance.

Closed soft tissue injuries. Bruises, sprains, tears. Clinic, diagnosis, treatment.

Traumatic toxicosis. Pathogenesis, clinical picture. Modern methods of treatment.

Critical disorders of vital activity in surgical patients. Fainting. Collapse. Shock.

Terminal states: pre-agony, agony, clinical death. Signs of biological death. resuscitation activities. Efficiency criteria.

Skull injuries. Concussion, bruise, compression. First aid, transportation. Principles of treatment.

Chest injury. Classification. Pneumothorax, its types. Principles of first aid. Hemothorax. Clinic. Diagnostics. First aid. Transportation of victims with chest trauma.

Abdominal trauma. Damage to the abdominal cavity and retroperitoneal space. clinical picture. Modern methods of diagnostics and treatment. Features of combined trauma.

Dislocations. Clinical picture, classification, diagnosis. First aid, treatment of dislocations.

Fractures. Classification, clinical picture. Fracture diagnosis. First aid for fractures.

Conservative treatment of fractures.

Wounds. Classification of wounds. clinical picture. General and local reaction of the body. Diagnosis of wounds.

Wound classification

Types of wound healing. The course of the wound process. Morphological and biochemical changes in the wound. Principles of treatment of "fresh" wounds. Types of seams (primary, primary - delayed, secondary).

Infectious complications of wounds. Purulent wounds. Clinical picture of purulent wounds. Microflora. General and local reaction of the body. Principles of general and local treatment of purulent wounds.

Endoscopy. History of development. Areas of use. Videoendoscopic methods of diagnosis and treatment. Indications, contraindications, possible complications.

Thermal, chemical and radiation burns. Pathogenesis. Classification and clinical picture. Forecast. Burn disease. First aid for burns. Principles of local and general treatment.

Electrical injury. Pathogenesis, clinic, general and local treatment.

Frostbite. Etiology. Pathogenesis. clinical picture. Principles of general and local treatment.

Acute purulent diseases of the skin and subcutaneous tissue: furuncle, furunculosis, carbuncle, lymphangitis, lymphadenitis, hydroadenitis.

Acute purulent diseases of the skin and subcutaneous tissue: erysopeloid, erysipelas, phlegmon, abscesses. Etiology, pathogenesis, clinic, general and local treatment.

Acute purulent diseases of cellular spaces. Phlegmon of the neck. Axillary and subpectoral phlegmon. Subfascial and intermuscular phlegmon of the extremities.

Purulent mediastinitis. Purulent paranephritis. Acute paraproctitis, fistulas of the rectum.

Acute purulent diseases of the glandular organs. Mastitis, purulent parotitis.

Purulent diseases of the hand. Panaritiums. Phlegmon brush.

Purulent diseases of serous cavities (pleurisy, peritonitis). Etiology, pathogenesis, clinic, treatment.

surgical sepsis. Classification. Etiology and pathogenesis. The idea of the entrance gate, the role of macro- and microorganisms in the development of sepsis. Clinical picture, diagnosis, treatment.

Acute purulent diseases of bones and joints. Acute hematogenous osteomyelitis. Acute purulent arthritis. Etiology, pathogenesis. clinical picture. Medical tactics.

Chronic hematogenous osteomyelitis. Traumatic osteomyelitis. Etiology, pathogenesis. clinical picture. Medical tactics.

Chronic surgical infection. Tuberculosis of bones and joints. Tuberculous spondylitis, coxitis, drives. Principles of general and local treatment. Syphilis of bones and joints. Actinomycosis.

anaerobic infection. Gas phlegmon, gas gangrene. Etiology, clinic, diagnosis, treatment. Prevention.

Tetanus. Etiology, pathogenesis, treatment. Prevention.

Tumors. Definition. Epidemiology. Etiology of tumors. Classification.

1. Differences between benign and malignant tumors

Local differences between malignant and benign tumors

Fundamentals of surgery for disorders of regional circulation. Arterial blood flow disorders (acute and chronic). Clinic, diagnosis, treatment.

Necrosis. Dry and wet gangrene. Ulcers, fistulas, bedsores. Causes of occurrence. Classification. Prevention. Methods of local and general treatment.

Malformations of the skull, musculoskeletal system, digestive and genitourinary systems. Congenital heart defects. Clinical picture, diagnosis, treatment.

Parasitic surgical diseases. Etiology, clinical picture, diagnosis, treatment.

General issues of plastic surgery. Skin, bone, vascular plastics. Filatov stem. Free transplantation of tissues and organs. Tissue incompatibility and methods of its overcoming.

What Causes Takayasu's Disease:

Symptoms of Takayasu's Disease:

Diagnosis of Takayasu's Disease:

Treatment for Takayasu's Disease:

Malformations of the skull, musculoskeletal system, digestive and genitourinary systems. Congenital heart defects. Clinical picture, diagnosis, treatment.

Anomalies or malformations - the occurrence of abnormalities in the structure of organs or tissue systems with a change or complete absence of function as a result of a violation of intrauterine development of the fetus.

According to WHO, malformations occur in 0.3 - 2% of births.

As a rule, malformations appear immediately at the birth of a child, much less often they appear later, when they progress with the growth of the child.

Factors contributing to the development of anomalies are called teratogenic. These factors are divided into internal and external. The action of teratogenic factors is manifested in the first weeks of pregnancy, especially from the 3rd to the 5th day and from the 3rd to the 6th week (periods of zygote implantation and organogenesis).

Internal factors - genetic defects (associated with gene and chromosomal mutations).

External factors:

1. Infectious - diseases of the mother during pregnancy (viral - measles, chickenpox, herpes; bacterial - scarlet fever, syphilis, diphtheria; protozoal - toxoplasmosis).

2.Physical - vibration, ionizing radiation, trauma during pregnancy.

3.Chronic diseases of the mother - diabetes mellitus.

Classification of malformations:

1. Changes in the size of organs:

a) hypergenesis - excessive development of an organ or part of the body;

b) hypoplasia (hypogenesis) - underdevelopment;

c) complete absence of the organ (aplasia, agenesis).

2. Change in the shape of organs - torticollis, clubfoot, horseshoe kidney.

3. Anomalies in the location of organs (ectopia, heterotopia - cryptorchidism, aberrant thyroid gland).

4. Increase in the number of organs: polydactyly, hermaphroditism.

5. Atavisms - median and lateral cysts of the neck.

6.Duplicating anomalies - "Siamese twins".

Malformations of the skull and brain.

Hydrocephalus (dropsy of the brain). Excessive accumulation of cerebrospinal fluid between the membranes of the brain or in the ventricles. The result is brain compression and atrophy.

Clinic: the head is significantly enlarged due to the vault, the fontanels are open, the seams are open, the high overhanging forehead. Progressive increase in intracranial pressure. The sharpest paroxysmal headaches are periodically noted.

Surgical treatment is the creation of an outflow from the ventricles using bypass surgery.

In emergency cases - ventricular puncture.

Craniocerebral hernia - protrusion of the brain and its membranes through a defect in the bones of the skull. 1 case per 4000 - 5000 newborns.

There are: anterior, posterior and basilar.

The meninges containing cerebrospinal fluid or brain tissue may protrude through the defect.

Treatment: surgical. The most favorable terms of treatment - up to 1 year. The results after the operation, carried out in a timely manner, are favorable. Patients after surgery physically and mentally develop correctly.

Craniostenosis. Premature fusion of the cranial sutures and discrepancy between the volume of the cranial cavity and the volume of the brain.

It occurs in about 1 in 1,000 newborns.

The skull changes its shape (tower-shaped, laterally narrowed, in half of the cases - 2-sided exophthalmos, increased intracranial pressure, headaches, decreased vision (optic nerve atrophy).

Treatment: surgical, bilateral flap craniotomy is performed. The results are usually favorable.

Malformations of the spine and spinal cord.

Spina bifida - incomplete closure of the spinal canal, most often characterized by splitting of the vertebral arches, in which spinal hernias are formed. Usually a protrusion in the lumbar region, there may be a violation of the function of the pelvic organs, the function of the lower extremities. Treatment is surgical. Sometimes the splitting of the arches is not accompanied by a hernia, it can often be accompanied by hypertrichosis, dermoids, enuresis.

Malformations of the face.

Upper lip cleft (cleft lip). This malformation accounts for 12-14% of all childhood malformations. 1 patient per 1000 - 1500 newborns.

There are incomplete and complete forms of non-closure, in which breathing and nutrition are disturbed. Food constantly enters the nasal passage, leading to asphyxia, pneumonia.

Operation: plastic replacement of the defect is performed in the first days or at the 3rd month of life. The results are good.

Macrostomia - non-closure of the corner of the mouth on one or both sides, an excessively wide oral fissure leading to the auditory duct. Usually accompanied by constant salivation.

Treatment is operative, best between 1 and 1.5 years.

Non-closure of the palate (cleft palate). One patient per 1000 - 1200 newborns. The history of treatment goes back several centuries. Previously, obturators were used to close the defect of the palate. The main feature is the communication of the oral and nasal cavities through a gap. Food, especially liquid, enters the nose, flows out of it: colloquial speech is unclear, nasal.

Respiratory and nutritional disorders previously led to 50% mortality in such children in the first year of life. Currently, the most appropriate time for surgical treatment is 3-4 years. Before the operation, special floating obturators are used.

Neck malformations.

Congenital cysts and fistulas of the neck.

Median cysts and fistulas occur as a result of a violation of the development of the middle lobe of the thyroid gland. It is always located in the midline, manifests itself at the age of 1 - 5 years. Usually it is possible to feel a dense cord going to the hyoid bone. Fistulas are usually formed with inflammation and suppuration of cysts.

Treatment is surgical, usually at the age of 3-4 years. The fistula is usually excised to the root of the tongue with resection of the hyoid bone.

Lateral cysts and fistulas. They are less common than the median ones, located along the inner edge of the sternocleidomastoid muscles.

Surgical treatment at 3-5 years. The fistula is excised completely up to the pharyngeal wall.

Malformations of the chest.

Funnel chest. The deformity appears immediately after birth. A characteristic symptom of the “paradox of inspiration” is the retraction of the sternum and ribs during inspiration, which is most pronounced when screaming and crying.

At school and adolescence, changes caused by chest deformity are more pronounced. Violation of posture increases, thoracic kyphosis is pronounced, fatigue, tachycardia, pain behind the sternum, a distinct decrease in lung excursions, pneumonia, bronchiectasis, and displacement of the mediastinal organs develop.

Surgical treatment - more often resection of costal cartilage segments, T - shaped osteotomy of the sternum and after correction - fixation with metal or bone structures.

congenital heart defects.

There are 3 groups of congenital heart defects, depending on the mixing of arterial and venous blood and, accordingly, changes in the color of the skin and mucous membranes.

1. Skin color is normal. Arterial and venous blood do not mix. Defects: coarctation of the aorta, stenosis of the aorta, pulmonary artery.

2. White-type defects: atrial septal defects, non-closure of the ductus arteriosus.

3. Vices of the blue type - are characterized by the discharge of venous blood into the arterial bed.

Atrial septal defects.

They make up about 10% of all heart defects. There is a discharge of arterial blood from the left to the right atrium. Hypertension develops in the system of a small circle, children usually lag behind in development, shortness of breath, sometimes cyanosis, hypertrophy of the right heart.

The treatment is surgical, preferably up to 3-4 years.

Ventricular septal defect. According to the statistics of the Institute of Cardiovascular Surgery, this defect is observed in 17% of patients with congenital malformations.

Hemodynamic disturbances are associated with the reflux of arterial blood from the left ventricle into the right ventricle (arterial discharge).

Treatment is surgical.

Non-closure of the arterial (botallian duct).

The child lags behind in development, frequent pneumonia, pallor of the skin, systole - diastolic murmur.

Treatment is surgical.

Coarctation of the aorta (congenital stenosis of the isthmus of the aorta). According to statistics, it occurs from 6 to 14% of all birth defects. The average life expectancy with this defect is up to 30 years.

Most often, the hemodynamic picture includes two different modes: the upper one, including the coronary system of the shoulder - head vessels, and the lower one, including the trunk, lower limbs and internal organs.

The state of decompensation can develop already in children of 1 year of age. Surgical treatment is from 3 to 10 years, but surgery is also performed at the 1st year of life.

Operation: resection and plasty of the site of narrowing..

Tetralogy of Fallot is a blue (cyanotic) type of defect.

The defect is classic among all the defects of the cyanotic type. It makes up 14% of all birth defects and 75% of blue defects.

Anatomically:

1) narrowing of the pulmonary artery;

2) ventricular septal defect;

3) displacement to the right (dextraposition) of the aortic orifice and its location above both ventricles (aorta sitting astride the interventricular septum);

4) hypertrophy of the wall of the right ventricle.

The defect in 1888 was first described by the French pathologist Fallot. Cyanosis develops from the first days or weeks. Dyspnea. By the age of 2, the fingers are “drum sticks.” Children rest while squatting (a very characteristic symptom) - pressure in the upper aorta increases due to compression of the large arteries of the lower extremities, this makes the collaterals between the large and small circles of blood circulation work more intensively . During attacks - loss of consciousness.

Shortness of breath - cyanotic attacks !!!

Surgical treatment: elimination of pulmonary artery stenosis, elimination of a ventricular septal defect, excision of the fibrous ring of the right ventricle in the region of the pulmonary artery mouth.

Pentade of Fallot: tetrad of Fallot + atrial septal defect.

Malformations of the digestive organs.

The yolk duct connects the midgut with the yolk sac, from which the embryo receives nutrition for the first 2-3 weeks of life. It becomes empty by the 8th week of intrauterine life, and by the 3rd month it completely atrophies. In cases where the yolk duct remains open, an umbilical fistula occurs.

The urinary tract (urachus) occurs at 2-3 months of intrauterine life during the differentiation of allantois. The upper part of the allantois forms the urinary tract, through which the urine of the fetus passes into the amniotic fluid. Obliteration begins from the 5th month of intrauterine life. With non-closure, a urinary fistula is also formed in the umbilical region.

Meckel's diverticulum - a blind process extending from the ileum - a variant of incomplete fusion of the distal part of the vitelline duct.

Hernia of the umbilical cord. Part of the abdominal wall in the area of the umbilical ring is covered with a thin transparent membrane. The protrusion passes into the umbilical cord.

Surgical treatment in the first hours.

Pyloric stenosis - hypertrophy of the pylorus muscles and a violation of their innervation Manifested at 3-4 weeks.

Surgical treatment - pyloroplasty.

Hirschsprung's disease is a congenital underdevelopment of the nerve plexuses in the recto-sigmoid colon with the expansion of the overlying sections. Hypertrophy, a sharp expansion of the large intestine, an increase in the volume of the abdomen, constipation.

Treatment is surgical.

Atresia of the anus and rectum - 1 in 10,000. Surgical treatment in the first hours.

Malformations of the genitourinary system

Cryptorchidism is a delay in intrauterine movement into the scrotum of one or both testicles, which are delayed in the retroperitoneal space or inguinal canal.

Treatment - operational.

Aplasia is the absence of a kidney.

Hypoplasia is a decrease in the size and functional ability of the kidney.

Dystopia - moving the kidney (into the pelvis, gr. cell, etc.).

Horseshoe kidney - fusion of the upper or lower poles of the kidneys

Epispadias - cleft of the anterior wall of the urethra. 1:50.000.

Hypospadias is the absence of the distal urethra. 1: 200 - 400 newborns.

Malformations of the musculoskeletal system.

Congenital dislocation of the hip - from 3 to 8 per 1000 newborns. In girls it occurs 4-7 times more often. Bilateral hip dislocation occurs 3-4 times less often, while left-sided dislocation occurs 2 times more often than right-sided.

Etiology:

1) developmental delay at the embryonic stage;

2) a defect in the primary backfill. In 98 - 99% of children with this disease at birth, predislocation is noted - hip dysplasia. As the head of the femur grows in the first months, it shifts outwards and upwards, the cavity of the joint flattens, is replaced by connective tissue. The head outside the acetabulum is deformed, flattened, the acetabulum is flattened, the articular bag is stretched

Inspection should be carried out in the first days.

The most reliable symptoms:

a symptom of repositioning or dislocation, or a slipping symptom.

a symptom of abduction restriction, which is determined by abduction of the legs bent at the knee and hip joints.

Asymmetry of the skin folds of the thigh.

At 1-1.5 years, lameness is detected with unilateral dislocation and "duck gait" with bilateral dislocation, a shortening of the limb appears on the side of the lesion. Later lumbar lordosis comes to light. R - graphic!

Treatment: it is necessary to start from the first days. Up to 1 year, special tires, spacers are used to fix the lower limbs in a state of abduction. Such fixation for 3-4 months allows to achieve normal development of the femoral head and articular cavity, and hip dislocation is completely cured.

At the age of 1 - 3 years, treatment is used in a plaster cast - bed (M.V. Volkov). After 3 years - surgical reduction of dislocation.

Congenital clubfoot. On average 1:1000 newborns.

Supination (turning the feet inward).

Adduction (adduction of the forefoot).

Equinus (plantar flexion of the foot).

Accompanied by atrophy of the muscles of the lower leg, gait based on the rear of the foot.

Treatment - from the first days of life.

In the first 2 - 3 weeks - 6 - 7 times a day - therapeutic exercises and bandaging with a soft flannel bandage. Starting from the 20th - 30th day of life - stage plaster bandages up to 5 months, changing the plaster every 7 days. In children older than this age, the dressings are changed after 2 weeks, until the foot is in a normal position.

In 60 - 70% of children, the deformity can be eliminated by 6 - 7 months of age.

If conservative treatment fails - at 2.5 - 3 years - Zatsepin's operation on the tendon-ligamentous apparatus, followed by a plaster cast for 5 - 6 months.

Syndactyly - fusion between the fingers (skin or bone). Operation in 2 - 3 years.

Polydactyly is an increase in the number of fingers or toes.

Macrodactyly - an increase in the volume of the fingers.

Malformations of the genitourinary system - one of the most numerous groups of congenital anomalies, including: damage to the kidneys (organs that purify the blood and form urine), ureters (channels leading from the kidneys to the bladder), bladder (the organ that contains urine), the urethra (the channel through which urine is released from the bladder), and the female and male genitalia. The male reproductive organs are the penis, prostate and testicles. Female genitalia - vagina, uterus, fallopian tubes, ovaries.

Malformations of the genitourinary system is perhaps the most numerous, they occur in every tenth newborn. Some of them are minor (for example, doubling of the ureters coming from one kidney to the bladder). They can be diagnosed only with the help of X-ray, ultrasound, or during surgery for a related or unrelated problem. Others can cause complications such as urinary tract infections, obstruction, pain, and kidney failure.

What causes malformations of the genitourinary system?

Some problems and diseases of the genitourinary system are inherited from parents who have such a disease, or are carriers of its gene. However, the specific causes of most of the anomalies are unknown. Environmental and genetic factors likely play a role in shaping the organs of the genitourinary system. A family in which there is a child with such a defect must be consulted by a geneticist. The specialist will acquaint you with the information known about the disease and the risk of repeating it in future generations.

How are genitourinary malformations diagnosed?

Many genitourinary deficiencies are diagnosed before or immediately after birth using an ultrasound examination (US). After birth, ultrasound or other diagnostic methods are recommended to get more information about the shape and function of the kidneys and other organs of the genitourinary system.

What are the most common malformations of the genitourinary system?

The following deficiencies of the genitourinary system are more often diagnosed: agenesis of the kidneys, hydronephrosis, polycystic kidney disease, multicystic kidney disease, urethral stenosis, exstrophy of the bladder and epispadias, hypospadias, intersexualism.

What is renal agenesis?

Renal agenesis or renal agenesis is the congenital absence of one or both kidneys. Approximately 1 in 4,000 babies are born without a single kidney (bilateral or bilateral renal agenesis). Since the kidneys are vital organs, their absence is incompatible with life. Therefore, a third of these children are born dead or die in the first days of life.

Children with bilateral renal agenesis usually have other malformations of the heart and lungs. In the absence of kidneys, urine is not formed - the main component of amniotic fluid. The lack of amniotic fluid leads to a violation of the development of the lungs, causes the formation of abnormal facial features and limb deficiencies.

Approximately 1 in 550 babies are born with unilateral renal agenesis. These children can lead healthy lives, although they are at risk of kidney infections, kidney stones, high blood pressure, and kidney failure. Some newborns have other malformations of the genitourinary system, which can later significantly affect overall health.

What is hydronephrosis?

Hydronephrosis is an expansion of the pelvis of one or both kidneys due to the accumulation of urine in them while preventing its free outflow. Severe hydronephrosis is diagnosed in 1 in 500 pregnancies during an ultrasound examination. The cause of impaired outflow may be the posterior urethral valves (they are located at the junction of the bladder into the urethra).

The obstruction that results in hydronephrosis is often caused by a piece of tissue where urine is released from the bladder. From the overflowing bladder of the fetus, urine returns back to the kidneys, presses on them, seriously damaging them. If hydronephrosis is diagnosed prenatally, the doctor will do several follow-up ultrasounds to see if the condition is getting worse. Some newborns with hydronephrosis are frail at birth, have difficulty breathing, kidney failure, and infections. In severe cases, surgery is recommended to remove the blockage, although there is no cure. Many moderate forms of hydronephrosis can be cured without the help of a surgeon.

Sometimes hydronephrosis can seriously threaten the life of the fetus, so in such cases, a shunt is inserted into the bladder, which would release urine into the amniotic fluid before birth. Prenatal treatment of such malformations has become the most successful form of fetal surgery in our time.

A block for the outflow of urine is also possible in places where the ureter connects to the kidney. The severity of obstruction varies widely: from minor to the development of renal failure. Surgery is recommended in the first or second year of life to clear the obstruction and prevent further complications.

What is polycystic kidney disease?

Polycystic kidney disease is a hereditary disease, manifested by the formation of multiple cysts in the kidneys, impaired renal function. There are two forms of this disease: autosomal dominant and autosomal recessive. In addition to kidney failure, it can be complicated by kidney infections, pain, high blood pressure, and other complications.

Autosomal dominant form of polycystic kidney disease is one of the most common genetic diseases, with an incidence of approximately 1 in 200-1000. Most patients have a family history of the disease, i.e. inherited from one of the parents, but in a quarter of patients, polycystic kidney disease occurs for the first time. Symptoms usually appear in their 30s or 40s, but they can occur in children.

Autosomal recessive polycystic kidney disease is relatively rare, but kidney cysts appear before the baby is born. Approximately 1 out of 10,000-40,000 babies is born with this pathology. Newborns with severe polycystic kidney disease die in the first days of life. Children with moderate polycystic disease live up to 10-20 years. This form of polycystic kidney disease is inherited from both parents, who pass the disease gene on to their child.

Drug treatment can treat additional problems that may arise as a result of polycystic kidney disease - high blood pressure and genitourinary infections. If kidney failure develops, dialysis is prescribed - a blood purification procedure. Sometimes a kidney transplant is needed.

Kidney cysts are also characteristic of other diseases: multicystic kidney disease, which is diagnosed in about 1 in 40,000 babies, a number of genetic syndromes. Multicystic disease can cause death in the neonatal period if both kidneys are affected. It is believed that multicystic kidney disease is the result of blockage of the urinary canal in the early stages of fetal development. Children with only one kidney affected may have other complications, such as urinary infections.

Even if the diseased kidney does not perform its functions (which may be an indication for its removal), the child can live a normal life with one healthy one.

What is bladder exstrophy and epispadias?

Bladder exstrophy is an anomaly characterized by underdevelopment of the anterior wall of the bladder and its placement outside the abdominal cavity. In addition, the skin of the lower abdomen is not fully formed, with widened spaces between the pelvic bones. Anomalies of the genitals are almost always combined with exstrophy of the bladder. Bladder exstrophy, occurs in 1 in 30,000 newborns, boys are affected 5 times more often than girls.

Epispadias combines the imperfections of the urethra and genitals. Often it is diagnosed together with bladder exstrophy, but it can also develop independently. In boys, the urethra is usually short and bisected, with an opening on the upper surface of the penis. The penis in such cases is also short and flattened. In girls, the clitoris may be bifurcated, and the urethral opening may be placed abnormally. About half of children with epispadias have urinary problems (enuresis).

Bladder exstrophy and epispadias are corrected surgically. Some sick children require multi-stage surgical interventions in the first years of life to normalize bladder function and correct the appearance of the genitals. Children with exstrophy of the bladder, surgery is performed in the first 48 hours of life. During the operation, the bladder is placed in the pelvis, the anterior wall of the abdomen is closed, and the pelvic bones are reduced to their normal position. Girls at the same time undergo surgery on the genitals. However, boys undergo a similar procedure at the age of 1 to 2 years. Additional surgery may be performed before the age of 3 years to normalize urination. Studies show that 85% of operated babies go on to live healthy lives.

What is hypospadias?

hypospadias is a fairly common penis deficiency affecting approximately 1% of all male newborns. The urethra (urethra) does not reach the top of the penis, but vice versa - the urethral opening is located anywhere on the surface of the penis.

Hypospadias is usually diagnosed when examining a newborn. Affected boys should not have their foreskin circumcised, as this may be necessary for surgical repair of the defect. Appropriate surgery is performed between the ages of 9 and 15 months. Without surgical treatment, boys will have problems urinating, and as adults they will feel sharp pain during intercourse.

What is indeterminate genitals or intersex?

Babies who are diagnosed with indeterminate genitalia have external genitalia that look neither male nor female, or have some features of both. For example, a girl may be born whose clitoris is so large that it resembles a penis, or a boy may have testicles along with female-type external genitalia. Approximately 1 in 1000-2000 newborns are affected.

There are many causes of indeterminate genitalia, including chromosomal and genetic disorders, hormonal disorders, enzyme deficiencies, and undetermined abnormalities in fetal tissue that later becomes genitalia. Most often, the cause of this is a hereditary disease called congenital adrenal hyperplasia. Some of its forms are accompanied by a violation of the kidneys, which sometimes causes death. Congenital adrenal hyperplasia at an early age is caused by an enzyme deficiency that leads to excessive production of male hormones (androgens) in the adrenal glands. An increased amount of male hormones causes changes in the female genitalia according to the male pattern. In such cases, hormonal therapy is carried out throughout life. Sometimes surgical correction of the clitoris is used. Congenital adrenal hyperplasia can be diagnosed prenatally using a special technique. Prenatal treatment can have positive results.

Another cause of intersexism is androgen insensitivity syndrome. Affected babies have a male set of chromosomes (XY), but due to genetic disorders, their cells are sensitive to androgens - male hormones. Children with complete androgen insensitivity syndrome have testicles that are located predominantly in the abdominal cavity and external female genital organs, but they do not have ovaries and uterus. Such babies grow and develop like women, but during puberty they require hormonal treatment. Babies with partial syndrome have cells, are partially responsive to androgens, and tend to have indeterminate genitalia.

A number of chromosomal abnormalities are also the cause of indeterminate genitalia. So, for dysgenesis dysgenesis, a characteristic male set of chromosomes (XY), external and internal female genitalia, or indeterminate genitalia and some combinations of male and female internal genital organs.

When a baby is born with indeterminate genitalia, a series of diagnostic tests must be performed to determine the sex of the newborn. These include a general examination, a blood test (including ultrasound and certain hormone levels), a urinalysis, and sometimes an ultrasound or surgery to look at internal organs. The results of the analyzes will indicate what type the child will develop in and, possibly, which gender should be indicated in the metric. Hormone therapy or surgical correction of the genitals is recommended. Sometimes doctors advise surgical correction for boys who have too underdeveloped penis, and recommend that they be raised like girls. In some cases, doctors advise raising the child as a girl and postponing surgery until later to see how the external genitalia develops and check how the child feels - like a boy or a girl. This is quite difficult for both the child and the family as a whole, so consultations with a psychologist are necessary.

Publication date: 2.11.10