Congenital anomalies of the genitourinary system. Anomalies of the urinary tract

Anomalies in the development of the 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 to each other by their anatomical location and functions. 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 external factors may pose a threat to the proper development of the internal organs of the unborn child:

• 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 of medications;
• bad habits – alcohol abuse, smoking, drug addiction.

Genetic predisposition plays an important role in the occurrence of abnormalities in the development of a child. 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 the development and functioning of the 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 abnormalities of the renal vessels are not accompanied by painful symptoms and are detected during the 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 the lumen of the 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 negative symptoms appear. Kidney dystopia may be accompanied by periodic abdominal pain.

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

Congenital pathologies of the bladder

This organ plays a 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-chambered” 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 of inflammatory processes and the formation of stones. 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. A severe congenital defect in which the bladder has no anterior muscular wall, and there is a hole in the lower abdomen with a diameter of several centimeters. 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.

Anomalies of the ureters, as a rule, are accompanied by congenital pathologies of 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.

Congenital defects of this organ include the following conditions:

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 male reproductive 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.

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,
take on an oval shape and turn into
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
In recent years they have not
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 groove of varying degrees of severity. Upper
half of the doubled bud is 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).
Indications for surgical treatment - implementation
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.
An 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

Complete bud duplication

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 there is a 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
Cystic kidney diseases include 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 flow 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 to the upstream 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
located on the back of the 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
protrusion of the posterior wall of the 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. Incomplete duplication of the urethra is more common. 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 the development of 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
hormone replacement 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 of the foreskin, preventing
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

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

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

Embryogenesis of the genitourinary system

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

5.1. KIDNEY ANOMALIES Classification

Renal vascular abnormalities

■ Quantity abnormalities: solitary renal artery;

segmental renal arteries (double, multiple).

■ Positional anomalies: lumbar; ileal;

pelvic dystopia of the renal arteries.

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

■ Congenital arteriovenous fistulas.

■ Congenital changes in the renal veins:

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

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

Kidney number abnormalities

■ Aplasia.

■ Kidney duplication (complete and incomplete).

■ Accessory, third kidney.

Abnormalities in kidney size

■ Renal hypoplasia.

Anomalies in the location and shape of the kidneys

■ Kidney dystopia:

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

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

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

Abnormalities of kidney structure

■ Kidney dysplasia.

■ Multicystic kidney disease.

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

■ Solitary renal cysts: simple; dermoid.

■ Parapelvic cyst.

■ Diverticulum of the calyx or pelvis.

■ Calyceal-medullary anomalies: spongy kidney;

megacalyx, polymegacalyx.

Combined kidney anomalies

■ With vesicoureteral reflux.

■ With bladder outlet obstruction.

■ With vesicoureteral reflux and bladder outlet obstruction.

■ With anomalies of other organs and systems.

Renal vascular abnormalities

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

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

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

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

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

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

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

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

or plastic surgery of the renal artery with synthetic materials.

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

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

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

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

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

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

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

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

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

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

Kidney number abnormalities

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

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

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

Rice. 5.4. Aplasia of the left kidney and ureter

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

Rice. 5.5. Sonogram. Kidney duplication

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

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

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

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

Rice. 5.6. Excretory urograms:

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

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

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

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

Accessory kidney- an extremely rare abnormality in the number of kidneys. The third kidney has its own blood supply, fibrous and fatty capsules and a ureter. The latter flows into the ureter of the main kidney or opens with an independent mouth in the bladder, and in some cases it can be ectopic. The size of the accessory bud is significantly reduced.

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

Abnormality in kidney size

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

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

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

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

Rice. 5.8. Scintigram. Hypoplasia of the left kidney

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

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

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

Anomaly of the location and shape of the kidneys

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Abnormalities of kidney structure

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

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

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

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

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

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

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

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

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

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

Rice. 5.19. Sonogram. Polycystic kidney disease

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

Surgical treatment consists of nephrectomy.

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

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

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

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

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

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

Rice. 5.20. Excretory urogram. Polycystic kidney disease

Rice. 5.21. CT. Polycystic kidney disease

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

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

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

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

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

Solitary cysts can be simple And dermoid. A solitary simple cyst can be not only congenital, but also acquired. A congenital simple cyst develops from germinal collecting ducts that have lost connection with the urinary tract. The pathogenesis of its formation includes a violation of the drainage activity of the tubules with the subsequent development of the retention process and ischemia of the renal tissue. The inner layer of the cyst is represented by single-layer squamous epithelium. Its contents are most often serous, in 5% of cases hemorrhagic. Hemorrhage into a cyst is one of the signs of its malignancy.

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

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

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

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

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

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

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

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

(Fig. 5.24).

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Rice. 5.27. Excretory urogram. Megapolycalicosis on the left

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

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

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

5.2. URETER ANOMALIES

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

■ agenesis (aplasia);

■ doubling (full and incomplete);

■ tripling.

■ retrocaval;

■ retroiliac;

■ ectopia of the ureteric orifice.

Abnormalities in the shape of the ureters

■ spiral (ring-shaped) ureter.

■ hypoplasia;

■ neuromuscular dysplasia (achalasia, megaureter, megadolihoureter);

■ congenital narrowing (stenosis) of the ureter;

■ ureteral valve;

■ ureteral diverticulum;

■ ureterocele;

■ vesicoureteropelvic reflux. Abnormalities in the number of ureters

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

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

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

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

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

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

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

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

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

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

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

Triplication of the pelvis and ureters is casuistry.

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

Abnormalities of the position of the ureters

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

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

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

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

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

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

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

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

Abnormalities in the shape of the ureters

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

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

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

Abnormalities of the ureter structure

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Vesicoureteropelvic reflux (VUR)- the process of retrograde reflux of urine from the bladder into the upper urinary tract. It is the most common pathology of the urinary system in children and is divided into primary And secondary. Primary VUR occurs as a result of congenital incompetence (incomplete maturation) of the vesicoureteral junction. Secondary - is a complication of bladder outlet obstruction, developing due to increased pressure in the bladder.

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

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

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

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

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

5.3. BLADDER ANOMALIES

The following malformations of the bladder are distinguished:

■ abnormalities of the urinary duct (urachus);

■ agenesis of the bladder;

■ bladder duplication;

■ congenital bladder diverticulum;

■ exstrophy of the bladder;

■ congenital contracture of the bladder neck.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Rice. 5.36. Descending cystogram. Bladder diverticula

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

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

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

■ reconstructive plastic surgery aimed at closing the defect of the bladder and abdominal wall with one’s own tissues;

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

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

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

5.4. ANOMALIES OF THE URETHER CHANNEL

Malformations of the urethra include:

■ hypospadias;

■ epispadias;

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

■ hypertrophy of the seminal tubercle;

■ duplication of the urethra;

■ urethro-rectal fistulas;

■ prolapse of the urethral mucosa.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

5.5. ANOMALIES OF THE MALE GENITAL ORGANS

Testicular abnormalities

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

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

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

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

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

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

Abnormalities of the position of the testicles include:

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

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

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

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

Rice. 5.37. Forms of cryptorchidism and testicular ectopia:

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

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

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

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

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

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

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

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

Penile abnormalities

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

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

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

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

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

Control questions

1. Give a classification of kidney anomalies.

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

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

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

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

6. What is the clinical significance of ureterocele?

7. What anomalies of the urachus are found?

8. List the types of hypospadias.

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

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

Clinical task 1

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

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

Clinical task 2

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

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

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

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

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

Clinical task 3

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

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

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

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

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

Types of pathologies

Disorders in the development of the urinary tract

Urinary tract pathology refers to a disorder in the development of the kidneys, ureters, renal vessels and bladder. This type of pathology is divided into two groups - anomalies of structure and position. If the proper development of the kidneys is disrupted, its underdevelopment (aplasia), hypoplasia of the ureters and pelvis, polycystic kidney disease, lumbar dystopia occurs. Developmental disorders of the ureters include:

• doubling;
• aplasia;
• congenital narrowing;
• pathology of ureteral valves;
• retrocaval location;
• ectopia (incorrect position of the mouth).

Bladder pathologies

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

Aplasia, duplication, diverticulum and exstrophy are common. A diverticulum occurs when the wall of the bladder bulges. When the urinary duct does not clog, a cyst is formed. In this case, no symptoms of illness are observed. But the most severe and dangerous defect is considered to be exstrophy - a pathology not only of the bladder, but also of the abdominal cavity, pelvic bones, and also the urinary canal. This deviation implies mandatory treatment, since if ignored, death is possible.

Pathologies of the urethra in women, men and children

Malformations of the urinary system (urethra) are a congenital pathology of the structure of the urethra and, as a result, dysfunction. In this case, there is difficulty passing urine, which negatively affects the functioning of the kidneys, and therefore requires prompt treatment. It is worth noting that pathologies of the urinary system are more common than other organs. These include doubling, hypospadias, and epispadias. Hypospadias is an incorrect location of the external opening of the urethra (located below where it should be). Hypospadias occurs only in men. In women, clitoral development is often disrupted. Epispadias is a defect of the genital organ when the external opening of the urethra in women and men is located above normal on the upper surface of the genital organ.

Causes of development and occurrence of anomalies of the urinary system

During pregnancy, the woman and the condition of the fetus are affected by various harmful factors.

Anomalies of the genitourinary system are congenital malformations of the fetus (CDF). Some problems are inherited from parents. The pathology may lie in a gene mutation. The occurrence of deviations is influenced by external factors. Congenital malformation begins to manifest itself in the fetus in the first three months of pregnancy. During this period, the formation of internal organs occurs, and any external impact of unfavorable factors directly on the fetal body can provoke a delay or, conversely, excessively rapid development. Unfavorable factors include viral infections that a pregnant woman suffers from, the influence of harmful chemical factors (dyes, resins), consumption of alcoholic beverages during pregnancy, as well as antibiotics, hormones and other medications, and the influence of radiation.

PRE-AND PERINATAL PATHOLOGY.

PATHOLOGY OF THE PLACENTA.

Prenatal pathology includes all pathological processes and conditions of the fetus from the moment of fertilization to birth. The founder of the doctrine of prenatal pathology is German. scientist Schwalbe, whose works date back to the beginning of the twentieth century. The period from the 196th day of development and the first 7 days after birth is called pernatal (“around childbirth”) and in turn is divided into antenatal, intra- and postnatal or prenatal (the prenatal period is wider, includes everything that happens before the 196th day), during childbirth and postpartum, neonatal. All fetal development can be divided into 2 periods: progenesis (the time of maturation of gametes and germ cells) and cymatogenesis (the period of fetal development from the moment of fertilization to birth). Cymatogenesis is divided into:

Blastogenesis – up to 15 days

Embryogenesis – up to 75 days

Fetogenesis – early – up to 180 days and late – up to 280 days

During the period of progenesis, the maturation of germ cells - eggs and sperm - they can be damaged, associated both with exogenous influences (radiation, chemical substances) and with hereditary changes in chromosomes or genomes. This is accompanied by mutations and hereditary diseases, including congenital defects and enzymopathies. Congenital defects are observed more often in children whose parents are over 40-45 years old

Cymatopathies are pathological processes occurring during cymatogenesis. The main pathology of this period is developmental defects, amounting to 20% or more.

Congenital malformations are persistent changes in an organ or the entire organism that go beyond variations in their structure and, as a rule, are accompanied by dysfunction. The causes of congenital malformations can be divided into: endogenous and exogenous.

Endogenous causes include mutations (it is believed that 40%)

Mutations may be: genetic - a persistent change in the molecular

gene structure

chromosomal - changes in the structure of chromosomes

genomic – change in the number of chromosomes, more often

trisomy.

With genomic mutations, the fetus most often dies. The role of natural mutagenesis is small. Induced mutations play an important role.

Mutagenic factors: ionizing radiation

chem. substances (cytostatics)

viruses (rubella)

over-ripening of germ cells (extension of time from the moment of complete maturation of germ cells to the formation of a zygote causes a complex of pathological changes in them.)

Exogenous causes include:

Radiation when exposed to critical periods of embryonic development. Irradiation in the first 3 months of pregnancy leads to congenital malformations, mainly of the nervous system.

Mechanical effects are the result of amniotic fusions, which is a mechanism for the formation of the defect, and not its cause;

Chemical factors, medicinal substances (anticonvulsant thalidomide);

Alcohol – leads to alcoholic embryofetopathy, subsequently there is a lag in weight, height, mental development, there is microcephaly, strabismus, a thin upper lip, frequent heart defects in the form of septal defects

Maternal diabetes - diabetic embryopathy with the formation of congenital malformations, skeletal malformations, heart defects, nervous system, high fetal weight, Cushingoid syndrome, hyperplasia of the islets of Langerhans. There are signs of immaturity, cardio-hepato-splenomegaly, microangiopathy, pneumonia;.

Viruses (cytomegaly)

The pattern in this period is dysontogenesis with any impact on the fetus. The time of exposure to a teratogenic agent is important: different agents at the same period of fetal development give the same congenital malformations, and the same agent at different times gives different malformations

CONGENAL DEVELOPMENTAL DEFECTS.

Congenital malformations of the central nervous system.

Take 1st place. The etiology is varied. From exo, the influence of rubella virus, cytomegaly, Coxsackie, polio, etc. has been precisely established. Drugs (quinine, cytostatics), radiation energy, hypoxia, gene mutations, chromosomal b-ni.

Anencephaly is agenesis of the brain, the anterior, middle and posterior parts are absent. The medulla oblongata and spinal cord are preserved. At the site of the brain, the connection. tissue rich in blood vessels.

Acrania is the absence of the bones of the cranial vault.

Microcephaly – hypoplasia of the g.m. combined with a decrease in the volume of the bones of the cranial vault and thickening.

Microgyria is an increase in the number of cerebral convolutions with a decrease in their size.

Porencephaly is the appearance of cysts communicating with the lateral ventricles.

Congenital hydrocephalus - excessive accumulation of cerebrospinal fluid in the ventricles (internal) or in the subarachnoid spaces (external) - atrophy of the brain due to impaired outflow of cerebrospinal fluid.

Cyclopia – one or two eyeballs in one socket

Hernias of the brain and spinal cord - meningocele - the presence of only membranes in the hernial sac, meninoencephale - and the brain, myelocele - herniation of the spinal cord.

Rasischis is a complete defect of the posterior wall of the spinal canal, soft tissues of the skin and meninges, and the spinal cord.

Congenital malformations of the digestive organs.

They are found in 3-4% of autopsies of the deceased and account for 21% of all congenital malformations.

Atresias and stenoses are observed in the esophagus, duodenum, proximal segment of the jejunum and distal ileum, in the rectum and anus. There may be tracheoesophageal fistulas in the esophagus, leading to severe aspiration pneumonia. Atresias can be single or multiple. In the area of ​​atresia, the intestine has the appearance of a dense connected tissue cord, which, under the influence of peristalsis, can stretch and break.

Duplication of individual sections of the intestine - most often affects only the mucous membrane, the muscular layer is common. The duplicated area may take the form of a cyst, diverticulum, or tube. The defect is complicated by bleeding, inflammation, necrosis with perforation.

Hirschsprung's disease - segmental aganglionosis megaoclonus - absence of neurons of the intermuscular plexus of the lower part of the sigmoid and rectum. Due to the preservation of the submucosal (Meissner's) plexus, the aganglionic section of the intestine is spastically contracted; above it, distension of the intestine occurs with meconium or feces, followed by compensatory hypertrophy of the muscular layer. Patients suffer from constipation, coprostasis and obstruction develop.

Hypertrophic pyloric stenosis is a congenital hypertrophy of the muscles of the pyloric region with a narrowing of its lumen. Persistent vomiting is observed, from 3-4 weeks until the development of coma from loss of chlorides.

Defects of the digestive tract associated with the preservation of certain embryonic structures. These include a umbilical hernia - a defect of the anterior abdominal wall with protrusion of a translucent hernial sac formed by the umbilical cord and amnion, containing loops of the small intestine.

Eventarization of the abdominal organs with its hypoplasia - the abdominal wall is open, the hernial sac is absent.

Cysts and fistulas in the area of ​​the umbilical ring are due to persistence of the vitelline duct.

Meckel's diverticulum is a finger-like protrusion of the wall of the ileum.

Congenital defects of the liver and biliary tract - polycystic liver, atresia and stenosis of the extrahepatic bile ducts, agenesis and hypoplasia of the intrahepatic bile ducts in the portal tract in the triad area, leading to the development of congenital biliary cirrhosis, congenital hyperplasia of the intrahepatic ducts.

Congenital malformations of the genitourinary system.

The etiology is not associated with certain exogenous factors, but with heredity and family, and occurs with chromosomal aberrations

Renal agenesis is the congenital absence of one or both kidneys, hypoplasia is a congenital decrease in the mass and volume of the kidneys, either unilateral or bilateral, renal dysplasia is hypoplasia with the simultaneous presence of embryonic tissue in the kidneys, large cystic kidneys is an enlargement of the kidneys with the formation of numerous small cysts, fusion of the kidneys (horseshoe kidney), and dystopia. – are not clinically manifested.

Congenital defects of the urinary tract:

Duplication of the pelvis and ureter

Agenesis, atresia, ureteral stenosis

Megaureter - sudden dilatation of the ureters

Exstrophy - as a result of his aplasia of his lane. walls, peritoneum and skin in the pubic area.

Bladder agenesis

Atresia, stenosis of the urethra, hypospadias - a defect of the lower wall, epispadias - the upper wall of the urethra in boys.

All developmental defects lead to impaired urine outflow and, without timely surgical treatment, lead to renal failure.

Congenital malformations of the respiratory system:

Aplasia and hypoplasia of the bronchi and lungs

Lung cysts, multiple and single, subsequently lead to the development of bronchiectasis

Tracheobronchomalacia - hypoplasia of the elastic and muscular tissue of the trachea and main bronchi, leading to the formation of diverticula or diffuse expansion of the trachea and bronchi

Congenital emphysema due to cartilage hypoplasia. Elastic and muscular tissue of the bronchi.

All congenital lung defects, if they are compatible with life, are easily complicated by secondary infection with the development of chronic disease. bronchitis and pneumonia with the development of cor pulmonale.

Congenital malformations of the osteoarticular system:

Systemic defects:

Fetal chondrodystrophy and achondroplasia (impaired development of bones of cartilaginous origin, bones of connective tissue origin develop normally - shortening and thickening of the limbs

Osteogenesis imperfecta – congenital bone fragility

Congenital marbled b-n – severe osteosclerosis with simultaneous disruption of the development of hematopoietic tissue

Congenital dislocation and dysplasia of the hip joint

Congenital amputation or amelia of the limbs

Phocomelia – underdevelopment of the proximal limbs

Polydactyly - an increase in the number of fingers

Syndactyly is the fusion of the fingers.