Malformations of the genitourinary system. Malformations of the urinary system in newborns. Anomalies of the genitourinary organs

Developmental anomalies genitourinary system are common and can have varying severity of manifestations. Most often, these are congenital defects associated with disruptions in the formation of the fetus during intrauterine development. Some of these pathologies are incompatible with life, and the baby dies in the womb or immediately after birth. Others are treated conservatively or surgically. Still others do not cause any concern to a person at all, and are discovered by chance during an examination using laboratory and hardware diagnostic methods.

The reproductive and urinary organs are closely related anatomical location and the functions performed. Together they form the genitourinary system. In women and men, the structure of this system differs to a certain extent due to their different reproductive roles.

The formation of the organs of the urinary system begins in the first weeks of the embryo’s existence, and at this time the fetus is especially vulnerable.

A number of things can pose a threat to the proper development of the internal organs of the unborn child: external factors:

• unfavorable environmental situation (increased background radiation, emissions of toxic substances into the atmosphere and water, and others);
• constant contact of a pregnant woman with chemicals, high temperatures ( professional activity);
• infectious diseases in the first trimester of pregnancy (toxoplasmosis, rubella, cytomegalovirus, herpes, syphilis);
• self-medication and uncontrolled use medicines;
• bad habits – alcohol abuse, smoking, drug addiction.

Plays an important role in the occurrence of abnormalities in the development of the baby. genetic predisposition. Gene mutations or other errors in the genetic apparatus can cause improper formation and development of the internal organs of a future person.

In more than thirty percent of cases, congenital pathologies of the urinary organs are inextricably linked with deviations in development and functioning reproductive system.

The following may be subject to abnormal changes:

• kidneys – pathology can be unilateral or bilateral;
• one of the ureters (less often a pair);
• bladder and urethra;
• reproductive organs (male more often than female).

Defects can affect the structure of tissues and the structure of the organ itself, as well as its blood supply system. An organ may have an atypical location in the body and, accordingly, influence the functioning of all its systems in a certain way.

Deviations in the structure and function of the kidneys

Congenital kidney pathologies may be associated with their location inside the body, the number of organs and their structure, as well as the atypical structure of their circulatory system.

Congenital pathologies of blood vessels providing blood supply to the kidneys:

1. The number and location of the renal arteries. In this case, there may be an accessory, double or multiple renal artery.
2. Anomalies in the structure and shape of the arterial trunks. These include an aneurysm - a modification of the walls of blood vessels, characterized by the absence of muscle fibers and thickening. Fibromuscular stenosis is an excess of muscle tissue. Arteriovenous fistulas are “bridges” between the venous and arterial systems.
3. Congenital modifications of the renal veins - by number: accessory and multiple, by shape and position - ring-shaped, retro-aortic, extracaval.

These renal vascular anomalies are not accompanied by painful symptoms and are discovered during examination of the patient.

However, they can become a “time bomb”, since the rupture of an aneurysm can lead to massive internal bleeding, kidney infarction, fibromuscular dysplasia - to a decrease in lumen renal artery, hypertension, kidney atrophy, nephrosclerosis and other negative phenomena.

There are five groups of deviations:

• number of kidneys;
• sizes;
• location;
• organ tissue structure;
• relationships with other bodies.

Kidney defects:

1. Aplasia is the absence of a kidney and its vessels. The bilateral form of this pathology is incompatible with life. With unilateral aplasia, one kidney functions and is enlarged.
2. Kidney doubling. The organ consists of two parts fused vertically - upper and lower. It is much longer than normal in length. The half of such a double bud, located on top, is often underdeveloped. Each part of the duplicated organ has its own blood supply system. Doubling can be complete or incomplete, one-sided or two-sided.
3. Accessory (third) kidney - has its own blood supply system. The size is smaller than normal, and the location is in the pelvis or iliac region (lower than normal). The third kidney itself is often abnormal. Has its own ureter.
4. Hypoplasia is a kidney that is reduced in size, but has normal structure and functionality. “Dwarf kidney” can be one-sided or two-sided. In unilateral cases, the opposite kidney is enlarged in size.
5. Dystopia is a deviation from the norm regarding the internal placement of the kidney. Normally, the kidneys are located in the retroperitoneal space; in the case of dystopia, the organ may be in the thoracic or pelvic cavity, in the iliac or lumbar region.
6. Spliced ​​kidney. It can be bilaterally symmetrical (“biscuit-shaped” - both kidneys are completely fused, “horseshoe-shaped” - fusion occurs at the upper or lower poles), bilaterally asymmetrical L, S - shaped, unilaterally - L-shaped.
7. Dysplasia is a structural anomaly in which the kidney has a reduced size and an abnormal structure of the parenchyma (dwarf, rudimentary).
8. Polycystic kidney disease - normal parenchymal tissue is modified in the form of cysts. Only small healthy areas of the organ parenchyma that are not replaced by cysts function. The pathology is bilateral.
9. Multicystic kidney - the tissues of the organ are replaced by multiple cysts containing fluid. This kidney does not function.
10. Megacalycosis is expansion of the calyces and thinning of the parenchyma.
11. Spongy kidney - multiple small cysts in the renal pyramids. In most cases it is bilateral.

Many of these pathologies are associated with abnormalities of the genital organs. In some cases, congenital anomalies become known after infection, when they appear negative symptoms. Kidney dystopia may be accompanied periodic pain in a stomach.

The fusion of the kidneys and their abnormal location, as well as the peculiarities of their forms, can provoke a mechanical effect on the ureters, vessels and nerve endings, which becomes the cause pain and disturbances in the blood supply to organs. Polycystic kidneys present with multiple symptoms characteristic of renal failure.

Congenital pathologies of the bladder

This organ plays vital role in the functioning of any living organism. It is designed to collect urine and then remove it from the body.

Anomalies in the development of the bladder are the result of certain disruptions during the intrauterine formation of the unborn child under the influence of a number of unfavorable external or internal factors:

1. Agenesis. The bladder and urethra are absent in the fetal body, which is incompatible with life.
2. Doubling. The organ is divided into two parts by a longitudinal septum. With complete bifurcation, each part of the bladder has its own urethra and one ureter. Incomplete duplication, called a “two-chamber” bladder, is characterized by the presence of one common urethra and a single neck.
3. Diverticulum. This disease is characterized by the presence of sac-like “protrusions” of the bladder walls. Urine accumulates and stagnates in these formations, which contributes to the development inflammatory processes and stone formation. These types of bladder abnormalities can be either congenital or acquired. The most characteristic symptoms are urinary retention, absence of urination, or urination in two stages.
4. Exstrophy. The hardest birth defect, in which the bladder has no anterior muscle wall, and in the lower abdomen there is a hole several centimeters in diameter. The back half of the bladder with the ureters protrudes into this open cavity, from which urine is released out. This anomaly is combined with defects of other organs and splitting of the pubic bones. It can only be treated surgically.
5. Anomaly of the urachus (urinary duct between the fetus and amniotic fluid), which should close by birth, but sometimes this does not happen. In such cases, there is an umbilical or vesico-umbilical fistula, cystic formations of this duct, and vesical diverticula.
6. Narrowing of the lumen of the bladder neck. Significant growth of fibrous tissue in the neck of the organ, which prevents the outflow of urine from the bladder.

Congenital pathologies of the ureters

These pathologies cause disruption of the processes of urine excretion from the body. Anomalies of the ureters are quite common among all congenital malformations of the genitourinary system.

Deviations may be as follows:

• the number of ureters is different from normal;
• there is an atypical location and relationship with other organs;
• the shape, structure and size of these organs are abnormal;
• the structure of muscle fibers is different from normal.

Abnormalities of the ureters are usually accompanied by congenital pathologies other elements of the urinary system - kidneys, bladder, urethra and reproductive organs:

1. Agenesis. The urinary organ is absent on the right or left side. Unilateral – accompanied by the absence of a kidney.
2. Doubling. Tripling. Characterized by a double (triple) pelvis. It can be complete or incomplete, one-sided or two-sided.
3. Retrocaval, retroiliac ureter - rare position anomalies when the ureter intersects with vessels, which lead to compression and obstruction of the ureter.
4. Ectopic orifice. Displacement of the ureteric orifice into the bladder neck (intravesical). Extravesical ectopia - displacement of the ureteric orifice into the urethra, rectum, vas deferens, uterus.
5. A spiral-shaped annular ureter leads to its compression and the development of hydronephrosis and pyelonephritis.
6. A ureterocele is a protrusion of the wall of the ureter into the bladder.

Anomalies of the ureters associated with changes in their structure - hypoplasia (the lumen of the ureter is narrowed, the wall is thinned), neuromuscular dysplasia (lack of muscle fibers in the walls of the organ), achalasia, ureteral valves, ureteral diverticulum.

These anomalies are quite rare and are not always diagnosed in childhood. However, the pathologies associated with them can be very serious. Treatment is most often carried out surgically.

Anomalies in the development of the urethra lead to both difficulty in passing urine and disruption of reproductive functions in men.

Birth defects of this body include the following states:

1. Hypospadias. Atypical location of the urethral opening due to the replacement of the anterior portion of the urethra with a chord. This phenomenon is accompanied by deformation of the reproductive male organ.
2. Epispadias. Characterized by the presence of a split anterior wall of the urethra. In boys it is observed more often and, depending on the length of the “cleft” and its location, it can be capitate, stem, or total. In girls - clitoral or subsymphyseal.
3. Congenital valves. Folded formations of the mucous membrane inside the urethra, shaped like bridges. They make urination difficult, cause stagnation of urine, infection, development of pyelonephritis and hydroureteronephrosis.

Very rare are congenital anomalies of the urethra, such as:

• obliteration (fusion) of the urethra;
• narrowing with impaired patency (stricture) of the urethra;
• urethral diverticulosis;
• double urethra;
• urethro-rectal fistulas;
• loss of all layers of the mucous membrane of the urethra to the outside.

There are also such anomalies in the development of the urethra as hypertrophy (increase in size) of the seminal vesicle in men and congenital cystic formations of the urethra.

Treatment of this type of congenital defects is carried out surgically in the first months and years of an infant’s life.

Anomalies in the development of the genitourinary system usually arise due to genetic mutation and can be expressed as underdevelopment individual organs, and in hyperdevelopment, up to doubling. Despite the fact that anomalies of this kind occur quite rarely in people, they can lead to serious psychological and physiological complications, and in most cases require treatment.

Medical Center "Energo" is a clinic that provides services in the field of diagnosis and treatment of many health problems. Correct, accurate diagnosis developmental anomalies of the genitourinary system allows us to establish their characteristics and recommend the correct course of treatment.

Developmental anomalies: types and symptoms

Among the existing anomalies of the development of the genitourinary system, it is customary to distinguish anomalies of external and internal organs, which are most often diagnosed in early age in male and female children.

Thus, among the anomalies that represent underdevelopment of the genitourinary organs, hypospadias and epispadias stand out. Hypospadias in men is underdevelopment of the penis and urethra, causing the urethra to be abnormally displaced.

Depending on the type of hole displacement, there are different types this disease:

• capitate hypospadias: the opening of the urethra is located on the head, but lower than necessary for normal urination in a child, capitate hypospadias in children and adults is considered mild degree indicated anomaly and is quite easy to treat;
• hypospadias truncal (distal-trunk form): the urethral opening is located at the bottom of the penis, which leads to its severe curvature;
• scrotal hypospadias: the opening is on the scrotum;
• coronal hypospadias: the urethral opening is located on the coronary sulcus;
• hypospadias without hypospadias: congenital shortening of the urethra with normal location urethra.

In turn, epispadias is a very large urethra (total epispadias), which in girls is located in the area of ​​the pubic joint (the anomaly can also occur in boys), due to which a person cannot control the process of urination.

Another type of developmental abnormality in boys is cryptorchidism, which is the failure of one or two testicles to descend into the scrotum. Most often, cryptorchidism in children is diagnosed on the right, less often on the left - that is, left-sided cryptorchidism.

In accordance with the location of the testicle, the following main classification of cryptorchidism has been developed, including such types of disease as:

• inguinal cryptorchidism: an undescended testicle is located in the groin, above or below inguinal ring;
• abdominal cryptorchidism: the location of the testicle is difficult to determine without special examination.

Very rare, but the most complex anomalies in the development of the reproductive system in humans include hermaphroditism, which is the simultaneous presence in humans of the primary genital organs of a man and a woman. In nature, in some animal species, hermaphroditism occurs as a normal feature, but in humans it is considered unnatural. Unlike other types of anomalies of sexual development, hermaphroditism may not be detected immediately, since its main signs in children sometimes appear only during puberty.

In addition to genitals, there are also anomalies of the urinary system, which mainly affect the kidneys and bladder.

Renal anomalies include:

• duplication of the kidney (right or left), which can be complete or incomplete. Incomplete duplication assumes that there are two organs with one ureter on one or both sides. With complete doubling, the doubled kidneys are absolutely independent, have their own circulatory system, ureters, renal pelvis, etc.;
• horseshoe kidney, or anomaly of fusion of the left and right kidney between themselves from above or below, occurs in children due to deviations at the stage of fetal development, can be diagnosed in both a child and an adult, usually during an ultrasound.

One of the most common types of bladder anomalies, in turn, is a bladder diverticulum - a kind of additional “pocket” (cavity) from the muscle tissue of the bladder.

Manifestations of abnormalities of the reproductive system are most often very obvious, which makes it possible to diagnose them in early childhood, usually almost immediately after birth. The exception is hermaphroditism, the signs of which may be inconsistency appearance gender status, which becomes obvious during puberty.

Problems with testicular prolapse may not be immediately identified, since this process occurs gradually. In this case, the symptoms can be both visual (the scrotum on one side may be underdeveloped, and the testicle cannot be palpated) and painful - in the form of aching pain on the undescended side.

Anomalies of the urinary organs are also most often diagnosed accidentally, usually when inflammatory processes occur, which can also be accompanied pain syndrome and frequent urination.

Developmental anomalies: causes of occurrence

It is generally accepted that most anomalies in the development of the genitourinary system occur due to a genetic mutation, the triggers of which have not yet been fully identified, however, experts usually include among the risk factors:

• negative impact of external factors on a woman during pregnancy (primarily radioactive radiation, if a woman lives in areas with high radioactivity);
• improper or insufficient nutrition;
• smoking and alcohol abuse during pregnancy;
• frequent use of toxic cosmetics(hairspray, for example) during pregnancy.

Developmental anomalies: complications

Anomalies in the development of the genitourinary system require diagnosis and appropriate treatment, as they can lead to serious consequences, physiological and psychological. Thus, a curvature of the penis can become not only the cause of problems with urination, but also psychological problems in the intimate sphere, as well as acquired infertility.

Infertility in adult men can also develop against the background of cryptorchidism (in the case of a bilateral type of disease, even with treatment and surgery, infertility is almost always inevitable), as a consequence of inhibition of the secretory functions of the testicle and cessation of sperm production. In addition, the longer cryptorchidism is left untreated, the more dangerous it is, as it can cause testicular torsion.

Hermaphroditism, in turn, causes a number of physiological and psychological problems, as it can interfere with a person’s self-determination.

Anomalies of internal organs, kidneys and bladder most often do not cause such problems to a person. serious problems, however, may increase the risk of developing inflammatory diseases, including chronic pyelonephritis and cystitis, as well as the occurrence of malignant neoplasms.

All of the above determines the need for timely diagnosis and treatment of developmental anomalies of the genitourinary system, which is recommended to be done at an early age, so you should contact a specialist as quickly as possible. Most often, one or another type of external anomaly can be determined by visual inspection and palpation, but in some cases additional diagnostics are required.

IN medical center"Energo" diagnoses developmental anomalies using modern General Electric equipment - CT, MRI and ultrasound machines.

Diagnosis using CT, MRI and ultrasound aims not only to differentiate the anomaly, but also to establish its severity. Thus, in the case of an undescended testicle, ultrasound allows one to accurately determine its location, and the use of contrast during the procedure makes it possible to determine the presence of a bladder diverticulum (in other cases, the diverticulum cannot be detected on ultrasound) or hypospadias.

Ultrasound and MRI are also used in the diagnosis of hermaphroditism, as they allow accurate assessment of deviations in the development of both types of genital organs. Additionally, for this type of anomaly, karyotyping and hormone tests are performed, which make it possible to determine which sex is genetically predicted.

Treatment plan

Properly carried out high-quality diagnostics developmental anomalies of the genitourinary system (hypospadias and other types) allows us to recommend the most effective treatment in St. Petersburg In most cases, treatment of this type of anomaly involves surgery. varying degrees complexity and volume. In the case of pathological curvature of the penis due to hypospadias, surgery is recommended to be performed within a period of six months to one and a half years, as this avoids serious psychological consequences for the young patient (gender self-determination usually occurs a little later in the child). Surgery for undescended testicles can be performed in both childhood and adulthood.

Surgery for abnormal development of the kidneys and bladder (for example, removal of a bladder diverticulum) may be indicated only if conservative treatment chronic diseases does not give results.

Hermaphroditism is one of the most difficult types of anomalies to correct: most often, surgery is combined with hormonal therapy in accordance with the patient's gender definition.

Developmental anomalies: prevention

Since this kind of anomaly is a consequence of a genetic mutation, it is quite difficult to predict its occurrence. However Special attention to diet, foods consumed and environment, as well as refusal bad habits, during pregnancy can significantly reduce the risk of developing abnormalities. Otherwise, it helps to solve the problem timely diagnosis, which can be obtained at the Energo medical center.

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

Congenital malformations (anomalies) of the urinary tract

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

Congenital malformations (anomalies) of the bladder

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

Congenital anomalies of the urethra

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

Symptoms of kidney abnormalities:

urinary disturbance;

growth disorder;

pain in the side and abdomen;

frequent urinary tract infections.

Causes of urinary tract abnormalities

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

Treatment of anomalies of the genitourinary system

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

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

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

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

Course of the disease

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

Modern therapy for urinary system anomalies is only possible if early diagnosis pathologies, therefore every child must undergo all mandatory preventive examinations.

CONTENTS:

INTRODUCTION
Renal vascular abnormalities
Kidney abnormalities
Ureteral abnormalities
Bladder abnormalities
Anomalies of male genital organs

Kidney anatomy

Kidneys:
paired organ
located according to
both sides of
spine in
lumbar
region

Embryogenesis:

Kidney development is based on three
structures:
Pronephros is an ontogenetic remnant of the excretory system
lower vertebrates, formed by 6-10 pairs of tubules,
connected by the mesonephric duct - Wolffian duct or
primary channel.
Mesonephros – develops from mesoblastic cell mass
and has functioning glomeruli and tubules. For 12-14 weeks.
its atrophy occurs.
Metanephros - consists of secretory and collecting systems.
The secretory system of the kidney consists of the mesonephrogenic blastema,
and the excretory one is from the remnant of the Wolffian duct.
The renal cortex is from the metanephrogenic blastema.

Main features of embryogenesis of the kidneys and ureters

nephrotomies
mesonephritic duct
urogenital ridge
1.
2.
3.
4.
5.
6.
7.
8.
Develop from mesoderm;
The forebud and primary kidney are rudimentary;
Prerenal ducts give rise to Wolffian ducts and, therefore,
ureters;
The Wolffian duct does not disappear and is involved in the development of the reproductive system in
male fetus;
Vessels approach the tubules of the primary kidney from the aorta, forming
capillary tangle. As a result, a renal corpuscle is formed, consisting
from the capillary glomerulus and capsule from the tubule of the primary kidney;
The final kidney functions from the second half of embryogenesis;
From the protrusion of the Wolffian duct, the ureter, renal
pelvis, renal calyces, collecting ducts;
The glomerular capsule is formed from metanephrogenic tissue, convoluted and straight
nephron tubules.

Merged Müllerian ducts (9 weeks)

By 7 weeks Wolffian embryogenesis
ducts and ureters open into
urinary
sinus
separate
holes. appears between them
accumulation of mesoderm (triangle
Bladder).
Wolffian (vas deferens) ducts
move downwards, and the ureters
up.
Merged Müllerian ducts (9 weeks)
The urogenital sinus is divided into 2
segment:
The ureters flow into the first one,
from it are formed: urinary
bubble, female and part of male
urethra;
In the second - Wolff and
fused Müllerian ducts,
from
him
is formed:
Part
male
urinary
canal, distal part and
vestibule of the vagina
Müllerian tubercle (9 weeks)

The muscular layer is formed from the surrounding mesenchyme.
The prostate is formed from epithelial outgrowths. At 3 months
embryogenesis of the ventral section of the urogenital sinus
expands to form the bladder.
The bladder moves towards the navel, connects with the allantois,
which is at 15 weeks. obliterated. From 18 weeks bladder
moves down and pulls the allantois (urinary duct) with it. WITH
20 weeks urinary duct - median umbilical ligament.
From the narrow pelvic section of the urogenital sinus is formed
part of the male urethra

DEVELOPMENT OF THE GENERAL SYSTEM The Wolffian canal takes part in the development of the male reproductive system, and the Müllerian canal takes part in the development of the female reproductive system.

MULLER (PARAMESONEPHRAL) CANAL
at the 3rd week of embryogenesis along the Wolffian
channel, a cellular cord is formed, gradually
it separates and a gap appears in it;
this formation is called
Müllerian canal or duct
in its upper part it ends blindly, and
caudal ends of opposite
Müllerian canals grow together and form one
they flow into the genitourinary duct through a common duct
sinus

development of gonads in both sexes
early stages proceeds the same way
(indifferent stage)
the surface of the primary bud is covered
coelomic epithelium (splanchnotoma)
on the medial surfaces of the primary
kidney
is happening
thickening
coelomic
epithelium,
which
called genital ridges
in the area of ​​the genital ridges of their endoderm
primary cells migrate from the yolk sac
germ cells - gonoblapsts
subsequently the genital ridges are significantly
develop, begin to protrude into the cavity
bodies, separated from the primary kidney,
acquire oval shape and turn
into the gonad
V
process
development
sexual
glands
coelomic cells and gonoblasts of reproductive
ridges grow into the underlying mesenchyme and
forms sex cords (cords) in it
then, depending on gender, sex cords
turn either into closed follicles (in
female) or into tubes (for males)
sex), where the primary reproductive organs are located
cells, which will later be
form
gametes,
And
cells
coelomic epithelium, of which there will be
take shape
follicular
And
interstitial cells of the ovary, cells
Leydig and Sertoli cells of the testis

Developmental anomalies

1. If the cranial displacement is disturbed, RENAL DYSTOPIA occurs;
2. Fusion of metanephrogenic tissue leads to UNION OF THE KIDNEY
(formation of a horseshoe kidney);
3. Splitting of the ureteric process leads to INCOMPLETE DUPLICATION
URETER, and with an accessory ureteric outgrowth - COMPLETE
URETER DUPLICATION;
4. ACCESSORY KIDNEY is formed as a result of the presence of an additional
area of ​​metanephrogenic tissue;
5. If the additional ureteric process is located far from the main one,
then, accordingly, the mouth of the ureter will open either in the neck
bladder, or in the urethra (ECTOPY
URETER);
6. In the absence of a ureteral outgrowth on one side, it develops
unilateral RENAL AGENESIS and only half is formed
triangle;

7. If the urinary-rectal septum is for 5 weeks.
does not separate the cloaca – CONGENITAL CLOACA;
8. Due to incomplete separation - CONGENITAL
FISTULAS in combination with posterior atresia;
9.
Obstruction of the urinary duct leads to
formation of vesico-umbilical fistula
(cyst);
10. Impaired downward displacement of the bladder leads to the formation
BLADDER DIVERTICULA;
11. Violation of the formation of the genital tubercle leads to the fact that the genitourinary
the groove is partially or completely open (on the dorsal surface
cavernous bodies) – EPISPADIA;
12. Violation of the fusion of the genitourinary folds causes – HYPOSPADIUS;

13. Absence of gonads –
AGENESIS,
incomplete
–
HYPOPLASIA;
14. Impaired descent of the testicles
scrotum
–
ONEor
BILATERAL CYPTORCHISM;
15.
Lowering
testicles
along
guides
fibers
the guide ligament causes
ECTOPY OF THE TESTIS;
16.
AZOOSPERMIA
(infertility)
arises
V
result
nonunion
networks
testicles
With
efferent tubules;
17.
MICROPENIS
at
pseudohermaphroditism
18. No front wall
Bladder - EXTROPHY
BLADDER.

Kidney anatomy

Each of the kidneys
has a front and
rear
surfaces,
lateral
And
medial edge,
top and bottom
ends (poles).

Kidney anatomy

There are veins in the sinus of the kidney
lie in front
arteries and nerves
behind the veins, and
renal pelvis and
ureter posterior to
arteries.

Kidney anatomy

The kidneys consist
from the brain and
cortical
substances

Kidney anatomy

Renal vascular abnormalities

1.
Anomalies in the number and position of the renal vessels:
a) Accessory renal artery
b) Double renal artery
c) Multiple arteries
2.
Anomalies of the shape and structure of arterial trunks
a) Aneurysms of the renal arteries
b) fibromuscular stenosis of the renal arteries
3.
4.
Arteriovenous fistulas
Renal vein anomalies
a) Anomalies of the right vein: multiple veins, confluence of veins
testicle into the renal vein on the right
b) Anomalies of the left vein: annular, retroaortic
left renal vein, extracaval confluence of the left
renal vein

84,6%

Additional
artery

Accessory and multiple renal veins:

occur in 17-20% of cases, which
are coming
To
lower
pole
kidneys,
accompanying the corresponding artery,
intersect with the ureter, thus
thereby causing disruption of the outflow of urine from
kidneys and the development of hydronephrosis.

Double renal artery

Multiple arteries

O.11%

Renal artery aneurysm
O.11%

Fibromuscular stenosis:

Fibromuscular stenosis:
More common in women.
The disease leads to a narrowing
renal artery lumen
Is high diastolic and
low pulse pressure and
refractoriness to antihypertensive
therapy.
On
basis
renal
angiography.
Surgical balloon treatment
dilatation
installation of an arterial stent
perform reconstructive
surgery

Fibromuscular stenosis
arteries

Arteriovenous fistula and renal aplasia

Kidney abnormalities

Occurs in 3-5.5% of patients
Make up 10% of all anomalies
Ministry of Railways
Behind last years Dont Have
downward trend

Kidney abnormalities are divided into 5 groups:

quantity anomalies
magnitude anomalies
location anomalies
relationship anomalies
structural anomalies

Classification (N.A. Lopatkina)

1 Abnormalities in the number of kidneys:
a) Aplasia
b) Kidney duplication
c) Accessory kidney
2 Abnormalities in kidney size: hypoplasia

Aplasia:

occurs relatively often - in
4-8% of patients with anomalies
kidney
the absence of not only kidneys, but also its
vessels
lack of
appropriate
half
interureteral
folds and orifices of the ureter
Excretory urography and ultrasound
allow
discover
the only one enlarged
kidney size

0,083% 1:1200

Kidney aplasia
0,083%
1:1200

Kidney number abnormalities

Kidney duplication
a common quantity anomaly. Double bud in length
significantly more than normal, it is often pronounced
embryonic lobulation. Between the upper and lower kidneys
there is a furrow varying degrees expressiveness. Upper
half double kidney most often smaller than the lower one.
The blood supply to the double kidney is carried out by 2 renal
arteries. Lymph circulation in each half of the double kidney
also separate. With complete doubling of the bud, in each half
there is a separate pyelocalyceal system, and in the lower
it is developed normally, but in the upper part it is underdeveloped. From every pelvis
leaves along the ureter. Duplication of the parenchyma and vessels of the kidney without
duplication of the pelvis should be considered incomplete duplication of the kidney.
Diagnostics - cystoscopy, excretory urography, scanning
kidneys This anomaly does not require treatment. Clinical manifestations
depend on various pathological processes developing in
one or both halves of the kidney.

Kidney duplication
10,4%
Accessory kidney

Kidney number abnormalities

Accessory kidney
This anomaly is extremely rare. Additional
the kidney has a separate blood supply that drains
the main kidney, or opens independently
mouth into the bladder. Sometimes she can be
ectopic and accompanied by constant
urine leakage. The accessory kidney is located
below normal and at the level of lower
lumbar vertebrae or in the iliac region, less often
in the pelvis. Its sizes vary, but most often
significantly reduced.
Diagnostics - excretory urography, scanning
kidneys, renal arteriography (aortography).
Indication for surgical treatment- execution
nephrectomy - hydronephrosis, nephrolithiasis, pyelonephritis,
as well as a tumor.

Accessory (third) kidney:

One of the rarest kidney anomalies (2%). The third kidney develops
due to splitting of the nephrogenic germ.
Has a separate blood supply from the ureter and is often located
below the normal kidney (in the iliac region, in the pelvis, in front of the pubic
symphysis).
Sizes are usually significantly reduced except in cases
hydronephrotic transformation.
The accessory kidney has its own capsule and sometimes has a loose
connective tissue fusion with a normal kidney.
The accessory kidney is often abnormal (hypoplasia, duplication
pelvis and ureters, dystopia) and is combined with various defects
development of the main kidneys.
The ureter of the accessory kidney can open independently in
the bladder is lateral and superior to the orifices of the main ureters or
open extravesically.
The accessory kidney usually has clinical manifestations only when
the development of pyelonephritis, hydronephrosis, stones, tumors or
ectopia of the ureter.
Chronic pyelonephritis, urolithiasis
indication for nephrectomy
disease
And
others,
serves

Accessory kidney

Full doubling kidneys

Duplication and hydronephrosis of the lower half of the kidney

Incomplete bud duplication

Magnitude anomalies

Renal hypoplasia is characterized by normal histological
structure and absence of renal dysfunction. Hypoplasia
Most often it is unilateral, but can be observed on both sides.
Diagnostics - excretory urography, radioisotope and
Ultrasound scan of the kidneys.
Renal arteriography allows differentiation of hypoplasia
from a kidney reduced in size due to pathological
process (nephrosclerosis).
With hypoplasia, the lumen of blood vessels both in the renal pedicle and inside
kidneys are uniformly reduced, and with secondary atrophy there is
sharp
decrease
lumen
intrarenal
vessels,
their incorrect distribution in the kidney, significant decrease
their quantities, especially in the renal cortex, with normal caliber
vessels
renal
legs.
With unilateral renal hypoplasia, the patient needs treatment
only if available pathological process in it. Usually this
pyelonephritis, which is often complicated by shrinkage of the kidney and
arterial
hypertension.
IN
this
case
perform
nephrectomy.

Renal hypoplasia

Classification

3. Anomalies in the location and shape of the kidneys
a) Kidney dystopia
- Unilateral (thoracic, lumbar,
iliac, pelvic)
- Cross
b) Kidney fusion
- Unilateral (L-shaped kidney)
- Bilateral (symmetrical –
horseshoe-shaped, biscuit-shaped buds;
asymmetrical – L and S shaped kidneys)

2,8%

Thoracic dystopia of the kidney
Rarely encountered, may appear unclear
pain in the chest, often after eating.
Diagnosis - chest X-ray,
fluorography - detect a shadow in the chest
cavities
above
diaphragm.
WITH
with help
excretory urography and kidney scan
the correct diagnosis can be made. U
thoracic
dystopian
kidneys
the ureter is longer than usual and marked
high
departure
vessels
kidneys

Thoracic dystopia of the kidney

Anomalies in the location of the kidneys (dystopia)

Lumbar kidney dystopia
the artery of the dystopic kidney is usually
departs from the aorta lower, at level II-III
lumbar vertebrae, pelvis facing
anteriorly. This anomaly manifests itself as pain.
The kidney is palpated in the hypochondrium and
may be mistaken for a tumor or nephroptosis

Lumbar kidney dystopia

Edge

Anomalies in the location of the kidneys (dystopia)

Ilial dystopia
the kidney is located in the iliac fossa,
renal arteries are usually multiple,
arise from the common iliac artery.
Shows
myself
pain
V
stomach,
caused by dystopic pressure
kidneys on neighboring organs and nerve plexuses,
as well as signs of impaired urodynamics.

Ilial dystopia

Anomalies in the location of the kidneys (dystopia)

Pelvic dystopia
characterized by deep location
kidneys in the pelvis.
Clinical manifestations are associated with displacement
border authorities, which causes a violation
their functions and pain.

Pelvic dystopia

Anomalies in the location of the kidneys (dystopia)

Cross Dystopia
characterized by the displacement of one kidney behind
the midline, causing both kidneys
turn out to be located with one
sides. With dystopia of the kidneys, blood vessels
short, extend lower than usual, kidney
lacks mobility. The operation is carried out
only in the presence of a pathological process
in a dystopic kidney.

Pelvic dystopia of the cup (medially).

Ileal dystopia of the cup (medially) and incomplete rotation of the kidney

Cross dystopia.

Cross Dystopia

ANOMALIES OF KIDNEY RELATIONSHIP:

Fusion may occur
symmetrically upper or
lower
poles
(horseshoe kidney),
as well as the middle parts or
asymmetrically when the bottom
the pole of one kidney fuses with
top pole vertically
rotated (S-shaped kidney)
or
horizontally
located
(L-shaped
kidney) of another kidney.
Sometimes both kidneys are fused
fully
And
have
biscuit-shaped.

Relationship anomalies

Fusion between both kidneys is considered
as relationship anomalies. Kidney fusion
on their medial surface is called
biscuit-shaped kidney. When connecting the upper
the poles of one kidney with the lower pole of the other
An S-shaped or L-shaped bud is formed. At
1st form ureteropelvic segment
one kidney is facing medially, and the other is facing laterally; in form 2, long axes of the kidneys
perpendicular
Friend
to a friend.

Biscuit bud

S-shaped and L-shaped kidneys

Relationship anomalies

Horseshoe
bud
characterized
connection
kidney
namesake
poles.
The horseshoe bud is almost motionless. More
durable
fixation
is
result
her
numerous vascular connections and peculiar
forms. The isthmus of the kidney connecting the lower
segments of both halves, usually located
in front of large vessels (aorta, inferior vena cava,
common iliac vessels) and solar plexus,
which presses against the spine. Very rarely
possible retroaortic position of the isthmus.

J-bud

0,25%

Horseshoe kidney:

With this kidney anomaly
merge with each other
with their upper ones or, more often,
lower poles.
This
promotes
more
frequent
traumatic
kidney damage
pressure on neighboring kidneys
organs
urolithiasis disease,
Developmental hydronephrosis,
there is a tumor process in it,
more often in the isthmus area..
when occurring in the kidney
diseases
requiring
surgical treatment.

Horseshoe kidney

Classification

4. Abnormalities of the kidney structure
a) Dysplastic kidney (rudimentary,
dwarf)
b) Multicystic kidney
c) Polycystic kidney disease
d) Kidney cysts
e) Calyceal-medullary anomalies
- megacalyx
- spongy bud
5. Combined kidney anomalies

Abnormalities of the kidney structure:
Dysplastic kidney (rudimentary, dwarf kidney).
Multicystic kidney.
Polycystic kidney disease:
adult polycystic disease.
polycystic childhood.
Parapelvic cyst, calyceal and pelvic cysts.
Calyceal-medullary anomalies:
a) megacalyx, polymegacalyx.
b) spongy bud.
Combined kidney anomalies:
a) with vesicoureteral reflux.
b) with bladder outlet obstruction.
c)c
vesicoureteral
reflux
And
bladder outlet obstruction.
d) with anomalies of other organs and systems - reproductive, musculoskeletal, cardiovascular, digestive.

Anomalies of structure

Kidney dysplasia - with this anomaly
there is a congenital decrease
kidneys the size of vicious
development of parenchyma and decrease
renal function. There are 2
forms
dysplasia
kidneys
vestigial and dwarf bud.

Kidney dysplasia

Anomalies of structure

Multicystic kidney disease - characterized by
complete replacement of kidney tissue
cysts and obliteration of the ureter
V
near the pelvis
department
or
the absence of its distal part. More often
The entire process is one-sided.
Diagnosed by aortography.

Multicystic kidney:

Multicystic kidney:
a rare anomaly characterized by
multiple cysts of different shapes
And
quantities,
occupying
all
parenchyma, with the absence of its normal
tissue and underdevelopment of the ureter
one-way process.
Before
accession
infections
unilateral multicystic kidney
not clinically manifested.
Diagnosis is made using
sonography and X-ray radionuclide
research methods
Surgical treatment consists of
nephrectomy.

Multicystic kidney disease

Anomalies of structure

Solitary renal cysts
TO cystic diseases kidneys belong to simple
solitary cysts, which can be congenital and
acquired. The origin of the latter is associated with
compression of the renal hilum by enlarged lymphatic
nodes or other formations.
The cyst usually originates from the renal cortex,
localized in any part of the renal parenchyma and can
contain up to several liters of interstitial
liquids. The walls of cysts consist of fibrous
connective tissue and are lined with flat, and sometimes
multilayered epithelium. The cyst does not communicate with
calyces and renal pelvis. Its contents are mostly
some cases are serous, less often (12-15%) - hemorrhagic.

Solitary renal cyst:

characterized by the formation of one or more
cysts localized in the cortical layer of the kidney.
develops
tubules
from
germinal
collective
Its contents are often serous, in 5% of cases
hemorrhagic.
ranges from 2 cm in diameter to gigantic
formations with a volume of more than 1 liter.
Kidney dermoid cysts are extremely rare.
They may contain fat, hair, teeth and bones.
squeezing
pyelocaliceal
ureter,
vessels
kidneys,
hemorrhage and malignancy.
presence of hypoechoic homogeneous with clear
contours, rounded fluid medium in the cortical
kidney area.
low contrast
education.
more than 3 cm and the presence of its complications, percutaneous
puncture of the cyst, injecting sclerosing agents
(ethanol).
avascular
shadow
systems,
suppuration,
rounded

Solitary renal cysts

Anomalies of structure

Spongy
bud
characterized
the presence of congenital multiple
small cysts in the renal pyramids.
The main symptoms are hematuria, pain in
lumbar region, pyuria. Diagnostics
- X-ray examination (shadows
small
petrification
V
projections
medullary
substances
kidneys),
excretory urography (in the area
papillae
visible
group
little ones
cavities
V
brain
substance).

Spongy bud:

Characterized by
availability
congenital
multiple small cysts in the kidneys
pyramids.
Typically this pathology occurs between two
sides
It occurs more often in men.
Manifestations of a spongy kidney may include pain
in the lower back and hematuria.
based on X-ray data.
The overview image shows multiple
small shadows of stones located in
area of ​​the renal medulla.
Treatment for spongy kidney is required only in
in case of complications.

Spongy kidney (overview photo)

Polycystic kidney disease:

heavy
bilateral
kidney abnormality,
characterized by substitution
renal
parenchyma
multiple
cysts
of various sizes.
The kidneys look like
bunches of grapes.
This
hereditary
disease transmitted by
autosomal recessive
type in children and autosomal dominant in adults.

Clinical manifestations: abdominal pain,
weakness, increased blood pressure.
In urine: gross hematuria.
In the blood: anemia, increased levels are noted
creatinine and urea.
Diagnosis
is installed
on
basis
ultrasonic and x-ray radionuclide
research methods.
Conservative
treatment
polycystic disease
is
V
symptomatic
And
antihypertensive therapy.
Surgical treatment is indicated for development
complications: suppuration of cysts or malignancy.
hemodialysis and kidney transplantation.

Polycystic kidney disease

0,17%

Anatomy of the ureters

Histological structure of the ureter.

URETER ANOMALIES:

Abnormalities in the number of ureters
◦ agenesis (aplasia);
◦ doubling (full and incomplete);
◦ tripling.
Abnormalities of the position of the ureters
◦ retrocaval;
◦ retroiliac;
◦ ectopia of the ureteric orifice.
Abnormalities in the shape of the ureters
◦ spiral (ring-shaped) ureter.
Abnormalities of the ureter structure
◦ hypoplasia;
◦ neuromuscular dysplasia (achalasia, megaureter,
megadolihoureter);
◦ congenital narrowing (stenosis) of the ureter;
◦ ureteral valve;
◦ ureteral diverticulum;
◦ ureterocele;
◦ vesicoureteropelvic reflux.

Duplication of the pelvis and ureter:

1 in 150 births
it happens 5 times more often in girls
can be one- or two-sided, full (ureter
duplex) and incomplete (ureter fissus)
the mouth of the superior is located below and medially, and
lower - higher and lateral. one mouth.
Complaints arise when complications develop.
hydroureteronephrosis.
vesicoureteropelvic reflux.
on
basis
excretory
urography,
multislice CT with contrast, MRI and
cystoscopy.
ureterocystoanastomosis, antireflux operations, heminefroureterectomy, nephroureterectomy.

Tripling

13,4%

Full doubling
13,4%

Incomplete doubling

Retrocaval ureter:

Retrocaval ureter:
a rare anomaly, with
which the ureter is in the lumbar
department goes under the vena cava.
leads to disruption of urine passage
with the development of hydroureteronephrosis.
The diagnosis is confirmed using
multislice CT and MRI.
execution
ureteroureteroanastomosis
With
location
organ
V
his
normal position to the right of
vena cava.

0,21%

Retrocaval ureter:
0,21%

Retrocaval ureter:

Corkscrew ureter:

Ureterocele:

cyst-like expansion of the intramural region
ureter with its protrusion into the lumen of the urinary
bubble
in 1-2% of patients, unilateral and bilateral.
Its outer wall is the mucous membrane
bladder, and the inner mucosa of the ureter.
At the apex of the ureterocele there is a narrowed orifice
ureter.
There are two types of this ureteral anomaly: orthotopic
And
heterotopic
(ectopic) ureterocele.
Ureterocele causes urinary passage disturbance, which
gradually leads to the development of hydroureteronephrosis.
A common complication of ureterocele is formation
there is a stone in it.
Cystoscopy is the main diagnostic method
ureterocele.
transurethral
endoscopic
resection
ureterocele or open resection with
ureterocystoanastomosis.

Ureterocele:

Ureterocele:

Incomplete dysplasia
7:1000
Ureteral plication

Neuromuscular dysplasia

Ectopic ureteral orifice:

Ectopic ureteral orifice:
Intravesical types include its downward displacement and
medially into the neck.
at
their
extravesical
ectopia
open
V
urethra, paraurethral, ​​into the uterus,
vagina, vas deferens, seminal vesicle,
rectum.
manifests itself as urinary incontinence with persistent
normal urination.
excretory urography, CT, vaginography, urethro- and
cystoscopy, catheterization of the ectopic orifice and
retrograde urethro- and ureterography.
consists of transplanting an ectopic ureter into
bladder (ureterocystoanastomosis).

Ureterocystoanastomosis for ureterocele or
high or low (according to intravesical ectopia of the orifice)

Abnormalities of the urethra:

hypospadias
epispadias
congenital valves, obliterations,
strictures, diverticula and cysts
urethra
hypertrophy of the spermatic tubercle
duplication of the urethra
urethro-rectal fistulas
mucosal prolapse
urethra.

Hypospadias

congenital underdevelopment of the spongy part
urethra with replacement of the missing section
connective tissue and curvature
penis towards the scrotum. Hypospadias
is
one
from
most
often
common urinary abnormalities
canal (in 1 out of 150-300 newborns). IN
depending on the location of the external
The openings of the urethra are distinguished:
capitate hypospadias,
truncal hypospadias,
scrotal hypospadias,
perineal hypospadias.

Abnormalities of the urethra

1.
2.
3.
4.
5.
Hypospadias of the penis (crown
heads, pericapital, distal-,
middle, proximal third of the genital
member)
Scrotal hypospadias (distal,
middle third of the scrotum)
Scroperineal hypospadias
Perineal hypospadias
Hypospadias without hypospadias

Hypospadias:

Hypospadias:
1: 250-300 newborns,
Testicular failure.
Hypospadias of the crown of the penis.
Pericapital
(pericoronal) hypospadias.
Hypospadias distal, middle and
proximal third of the penis.
Scroperineal
And
perineal forms of hypospadias
The diagnosis of hypospadias is established when
objective research, determine
genetic sex of the child.
the operation is performed with significant
curvature of the glans penis and/
or meatostenosis.

1:450-500

"hypospadias without hypospadias"

hypospadias, in which external
the opening of the urethra is in the usual
place on the head of the penis, but
it itself has been significantly shortened.
Between the shortened urethra and
normal length penis
located dense
connective tissue cord (chord),
which makes the penis sharp
curved in the dorsal
direction.

4 types of “hypospadias without hypospadias”

Diagnosis of hypospadias

established with objective
research. In some cases
can be difficult to distinguish
scrotal and perineal
hypospadias from female false
hermaphroditism. In such cases
needs to be determined
genetic sex of the child.

Treatment

Surgical treatment is indicated for all forms of this
anomalies and is performed in the first years of a child’s life.
For capitate and coronal hypospadias surgery
carried out with significant curvature of the head
penis and/or meatostenosis.
Treatment methods are aimed at achieving two
main goals: creation of the missing part of the urethra with
the formation of its external opening in a normal
anatomical position and straightening of the penis
due to excision of connective tissue scars (notochords).
Prognosis for timely plastic surgery
operation favorable.

Tactics for managing patients with Hypospadias.
With “hypospadias without hypospadias” urination
is violated slightly, so the main
criterion determining the need
surgical correction is the degree
curvature of the penis.
Since when straightening you have to cross
a short, although normally opening, urethra and
create an artificial dystopia for some time
external hole, then the decision on the need
intervention is difficult and
responsible task. It is necessary to consider how
the patient’s persistence and the medical experience
institutions in the treatment of hypospadias.

Indications for surgery for pericapital
Hypospadias.
They are a narrowing of the external opening of the urethra,
disrupting the outflow of urine, and (or) significant curvature
penis and its glans. If the narrowing of the external
the urethral opening in these cases is absolute
indication for surgical treatment(meatotomy) due to
its dangers for superiors urinary tract and health
patient, then the curvature of the penis is relative
and should be taken into account depending on the degree of its influence
on sexual function usually in adulthood
patient. In the absence of these signs, lengthening of the urethra
by 1 - 2 cm and moving the distopped hole by
head is not advisable due to possible serious
complications (formation of strictures, curvature and
desolation of the vessels of the head, etc.).

Abnormalities of the urethra

1.
2.
3.
Epispadias glans
Epispadias of the penis
Complete (total) epispadias

Epispadias

malformation of the urethra, for
which is characterized by underdevelopment or absence of
greater or lesser extent of its upper
walls. Frequency of occurrence is less frequent than that of
hypospadias occurs in approximately 1 in 50,000 newborns.
The urethra with this pathology
situated on back side penis between
split cavernous bodies.
There are:
epispadias of the head,
epispadias of the penis,
total epispadias.

Epispadias:

Congenital clefting of all or part of the anterior wall of the urethra,
The urethral opening is found on the dorsal surface of the penis.
Epispadias of the glans penis is extremely rare and does not require surgery.
corrections.
Epispadias of the penis. The external opening of the urethra is located in the area of ​​the crown on
dorsum of the penis.
Complete (total) epispadias is the most severe form in which the external opening
The urethra is located at the root of the penis. The hole resembles a wide funnel.
The clitoral form of epispadias in girls is a slight splitting of the terminal
urethral department. Most often this form goes unnoticed.
Subpubic epispadias is characterized by splitting of the urethra to
bladder neck and clitoral cleft.
Complete (retropubic) epispadias: anterior wall of the urethra and wall
the anterior segment of the bladder neck is absent.
Surgical treatment of epispadias is carried out in the first years of life. It lies in
reconstruction of the urethra and elimination of penile curvature.

Epispadias

Epispadias of the glans penis

characterized by the fact that
anterior wall of the urethra
split to coronoid
grooves. Penis
slightly bent and
raised up.
Urination and erection during
This form of epispadias is usually
not violated.

Stem form of epispadias

characterized by the fact that the anterior wall of the urethra
split throughout the entire penis to the area where the skin transitions to the pubic area.
With this form of epispadias it is noted
clefting of the pubic symphysis, and sometimes
separation of the abdominal muscles.
The penis is shortened and curved to the side
anterior abdominal wall. Urethral opening
has the shape of a funnel. When urinating, the stream
directed upwards, urine sprays out, which
leads to wet clothes.
Sexual life is impossible because the penis
small in size and strong during erection
twisted.

Total (complete) epispadias

except for splitting the anterior wall of the urethra
characterized by splitting of the sphincter
Bladder. The urethra has the shape of a funnel and
located immediately under the womb.
This form is characterized by urinary incontinence
due to underdevelopment of the urinary sphincter
bubble Constant leakage of urine leads to
to skin irritation in the scrotal area and
perineum, dermatitis develops,
normal social adaptation is disrupted
child in a society of peers. Noted
underdevelopment of the penis and scrotum.

Treatment

Surgical treatment
epispadias is performed in
first years of life.
It lies in
urethral reconstruction and
eliminating curvature
penis.

Abnormalities of the urethra

1.
2.
3.
Clitoral form of epispadias
Subpubic epispadias
Complete (retropubic) epispadias

Congenital urethral valves

presence in its proximal part
pronounced folds of the mucous membrane protruding into
the lumen of the urethra in the form
jumpers.
Occurs in 1 in 50 thousand newborns.

Urethral valves disrupt normal
urination is difficult
emptying the bladder,
lead to the appearance of residual
urine, development of hydroureteronephrosis
and chronic pyelonephritis.
Surgical treatment –
endourethral mucosal resection
membranes of the urethra
along with the valve.

Congenital obliteration of the urethra

Congenital urethral stricture is a rare anomaly in which
there is a cicatricial narrowing of its lumen, leading to
urinary disorders.
Congenital urethral diverticulum is also a rare defect
development, consisting in the presence of a sac-shaped
protrusions back wall urethra. More often
localized in the anterior urethra. Manifested by dysuria
and the release of drops of urine after the end of the act
urination. The diagnosis is made based on
urethrography and urethroscopy, voiding
cystoureterography. Treatment consists of excision
diverticulum.
Congenital urethral cysts develop as a result of
obliteration of the outlet openings of the bulbourethral glands.
Predominantly localized in the bulb area
urethra. Allows you to make a diagnosis
voiding cystourethrography. They are removed surgically
way.

Duplication of the urethra is a rare developmental defect. It happens
complete and incomplete. Full doubling is combined with doubling
penis. More common incomplete doubling urethra. IN
in most cases, additional urination
the channel ends blindly. Accessory urethra always
has an underdeveloped corpus cavernosum. Treatment consists of
complete excision of the accessory urethra and
paraurethral passages.
Urethro-rectal fistulas - a defect
development, which is almost always combined with posterior atresia
passage. Occurs as a result of underdevelopment
urinary rectal septum.
Prolapse of the urinary mucosa
canal is a rare anomaly. Lost mucous membrane due to
microcirculation disorders have a bluish tint, sometimes of the bladder near the mouth,
slightly higher and lateral to it.
Persistent stagnation of urine in the diverticulum
promotes the formation of stones in it
and the development of chronic inflammation.
difficulty urinating and
emptying the bladder in two
stage.
based on ultrasound, cystography and
cystoscopy.
Surgical treatment consists of
diverticulum excision and suturing
formed wall defect
Bladder.

Bladder exstrophy:

severe developmental defect, consisting of
absence of the anterior wall of the bladder and
the corresponding part of the anterior abdominal wall.
in 1 out of 30-50 thousand, it is often combined with defects
development of the upper and lower urinary tract,
Bladder exstrophy is always accompanied by
total epispadias and cleft pubis
bones
With such an anomaly, urine is constantly pouring out
out.
promotes development chronic cystitis And
pyelonephritis.
reconstructive plastic
operations,
formation of artificial
orthotopic
urinary reservoir from the ileum.

Pathology of the urachus

Testicular abnormalities (5-7%)

Anorchism
Monorchism
Polyorchidism
Hypoplasia
Synorchism
Cryptorchidism
Ectopic testicle

Cryptorchidism:

malformation (from the Greek kriptos - hidden and orchis testicle), in which there is undescended
the scrotum of one or both testicles.
is 3%,
Abnormal position of the testicle leads to its
anatomical and functional insufficiency up to
to atrophy
risk of malignancy
The
vice
development
Maybe
be
one-sided
And
bilateral,
true and false.
The diagnosis is made based on the data
physical examination, sonography, CT,
testicular scintigraphy and laparoscopy.
Use hormonal therapy with human chorionic
gonadotropin.
Surgical treatment is performed in the first years
child's life
if ineffective (orchiopexy).

Ectopic testis is a congenital malformation
in which it is located in different
anatomical areas, but not along the course of its
embryonic path to the scrotum. This
the anomaly is different from cryptorchidism. IN
Depending on the location of the testicle, they are distinguished
inguinal, femoral, perineal and
cross ectopia.
Surgical treatment - relegation of the testicle to
the corresponding half of the scrotum.
Prognosis for testicular development with cryptorchidism and
ectopia is favorable if surgery
performed in the first years of a child’s life.

Anorchism

This is the absence of both testicles. Usually
accompanied by
simultaneous
underdevelopment
appendages
testicles
And
vas deferens. With this
abnormalities in the child are sharply reduced
the amount of male sex hormones,
there are no secondary male reproductive organs
signs (eunuchoidism).

Polyorchidism

there are three at the same time or, what happens
very rarely, more than testicles. Underdeveloped
the accessory testicle is located next to
normal testicle. Sometimes additional
the testicle is found in the pelvis.
The accessory testicle is removed because it
susceptible to frequent malignant
rebirth.

Testicular hypoplasia

abnormality of the testicular structure. At the same time, one thing
or both testicles are underdeveloped, reduced in size
sizes up to 5-7 mm. Two-way
underdevelopment
testicles
accompanied by
hormonal deficiency and requires
replacement hormone therapy.

Penile abnormalities

Congenital phimosis
Hidden penis
Ectopia of the penis
Double penis

Congenital phimosis:

congenital narrowing of the foramen
flesh that does not allow the head to be exposed
penis.
Mostly boys under 3 years of age
cases, physiological
phimosis
In case of pronounced narrowing of the extreme
flesh resort to her circular
excision (circumcision).

Phimosis

narrowing foreskin, obstructing
liberation
heads
from
preputial sac. With phimosis often
balanoposthitis occurs. Phimosis is
predisposing factor for development
penile tumors.

Paraphimosis

pinching of the glans penis
foreskin. Paraphimosis occurs
swelling of the head, severe pain, difficulty
urination, sudden swelling of the genital skin
member. In case of untimely reduction
necrosis of the strangulating tissue may develop
rings.

Hidden penis

an extremely rare anomaly, with
which are normally developed
corpora cavernosa are hidden
surrounding tissues of the scrotum and
skin of the pubic area.
The penis is usually
reduced in size, cavernous
bodies are determined only when
palpation in the folds of the surrounding
skin.

Short frenulum of the penis

prevents
liberation
heads
penis from the preputial sac,
causes curvature of the penis
erections and pain during sexual intercourse
intercourse.

One of the main ones in the body. Each pathology of the urinary tract makes life difficult and leads to diseases. This anomaly occurs in many people. Sometimes they live with these abnormalities without unnecessary problems, and sometimes they have to be treated or operated on to avoid worsening the disease. Often, developmental anomalies occur in young children, then parents, together with the doctor, decide how best to help the child and what measures to take to save him from the difficulties associated with the disease.

What is pathology of the urinary system?

During the development of the genitourinary system, sometimes deviations occur and one or more organs that belong to this system begin to develop differently from the rest and mutate. Sometimes this manifests itself in the underdevelopment of an organ, and sometimes they develop too actively and are ahead of other organs. Because of this, an imbalance occurs in the entire genitourinary system, and other organs begin to hurt and work incorrectly. There are developmental defects that do not affect other organs in the system and do not negatively affect their functioning. Duplication of the ureters is considered such a simple deviation. Developmental anomalies occur in all organs of the genitourinary system:

• kidneys;
• bladder;
• ureters;
• urethra;
• genitals.

What are the anomalies in the development of the urinary system?

All malformations of the urinary system are divided into types depending on the organ or organ system in which the anomaly was found. If an abnormality is found in the ureters or renal vessels talk about abnormalities in the urinary tract. If a problem is found in the bladder, they talk about abnormalities in this organ, which, in turn, are divided into several types.

What are the types of urinary tract abnormalities?

Often there is a deformed and enlarged urethra in women, which provokes abnormal development of the clitoris.

Kidney duplication is one of the deviations in the development of the organ.

Urinary tract defects imply abnormalities in the functioning or development of one of the organs responsible for the production and excretion of urine. This includes the kidneys, their vessels, ureters and bladder. These organs have deviations in tissue structure and location. Depending on which organ is affected, corresponding pathologies develop, the relationship of which is presented in the table:

Organ	Pathology
Bud	Aplasia
	Hypoplasia
	Polycystic
	Lumbar dystopia
	Horseshoe kidney
Ureter	Doubling
	Underdevelopment
	Congenital narrowing
	Valve pathology
	Retrocaval arrangement
	Ectopia

Bladder abnormalities in women, children and men

Most often, when examined, the presence of aplasia, duplication, diverticulum or exstrophy is noted. Diverticulum refers to a condition where a fragment of the wall of an organ protrudes above the rest. A cyst is formed when the urinary tubule becomes overgrown. This condition does not cause changes in sensations. Exstrophy is a pathology affecting the bladder, abdominal cavity, pelvic bones and urinary canal. It is considered the most difficult to treat and life-threatening.

Urethral disorders

Duplication of the urethra is one of the deviations in the development of the urethra.

Pathologies of the urinary system are deviations in the structure and structure of the channel through which urination occurs. Such congenital defects violate correct work the entire urinary system. Conditions in which urine is difficult to pass out negatively affects the functioning of the kidneys, so doctors recommend starting treatment as soon as such a problem is discovered. Doctors note that the urinary system suffers from abnormalities more often than other organs, and this happens due to malformations of the organs of the genitourinary system during the period of intrauterine development.

Name of pathology	Description	Sex predisposition
Doubling	Multiple structure of the urethra	Occurs in children, men and women
Hypospadias	The external opening of the urethra is located lower than it should be	Diagnosed in males
Epispadias	Deviation in the development of the genital organs, in which the external opening of the urinary canal is located higher than necessary on the genital organ	Observed in representatives of both articles

For what reasons do deviations occur?

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

Deviations in the structure and development of this organ system occur during intrauterine development and are called birth defects fetal development (FDP). They are inherited or due to gene mutation. The urinary system of the fetus is very sensitive to the effects of negative external factors and influences. The presence of developmental pathologies becomes visible already by the third month of pregnancy. A pregnant woman needs to be very careful, as during this period internal organs child, and any adverse influence provokes abnormal development. Any colds chemical substances, alcohol, nicotine, drugs and radiation will provoke deviations not only of the genitourinary system, but also of other vital organs.