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

Anomalies in the development of the genitourinary system are not uncommon, and may have a different severity of manifestations. Most often, these are congenital malformations associated with failures in the processes of fetal formation during fetal development. Some of these pathologies are incompatible with life, and the baby dies while still in the womb or immediately after birth. Others are treated conservatively or surgically. Still others do not cause 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 existence of the embryo, and at this time the fetus is especially vulnerable.

For the proper development of the internal organs of the unborn child, a number of external factors may pose a threat:

• unfavorable ecological situation (increased radiation background, emissions of toxic substances into the atmosphere and water, etc.);
• 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 drugs;
• bad habits - alcohol abuse, smoking, drug addiction.

Not the last role in the occurrence of anomalies in the development of the baby is played by a genetic predisposition. Mutations of genes or other errors in the genetic apparatus can cause improper formation and development of the internal organs of the future person.

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

Abnormal changes may be subject to:

• kidneys - pathology can be unilateral or bilateral;
• one of the ureters (rarely 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, in a certain way affect the operation of all its systems.

Deviations in the structure and function of the kidneys

Congenital pathologies of the kidneys can be associated with their placement inside the body, the number of organs and their structure, as well as the atypical structure of their circulatory system.

Congenital pathologies of vessels that provide blood supply to the kidneys:

1. Number and placement of renal arteries. In this case, there may be an additional, double or multiple renal artery.
2. Anomalies in the structure and shape of 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 veins of the kidneys - by number: additional and multiple, by shape and position - annular, retroaortic, extracaval.

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

However, they can become a "delayed-action bomb", since an aneurysm rupture 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:

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

Kidney defects:

1. Aplasia - the absence of a kidney, as well as its vessels. The bilateral form of this pathology is incompatible with life. With unilateral aplasia, one kidney functions, and it has an enlarged size.
2. Doubling of the kidney. The organ consists of two vertically fused parts - upper and lower. It is much longer than normal. The half of such a double kidney, located on top, is often underdeveloped. Each of the parts of the doubled organ has its own blood supply system. Doubling is complete or incomplete, unilateral or bilateral.
3. Additional (third) kidney - has its own blood supply system. The sizes are smaller than usual, and the location is in the pelvis or iliac region (below normal). The third kidney itself is often abnormal. It has its own ureter.
4. Hypoplasia is a kidney reduced in size, which has a normal structure and functionality. "Dwarf kidney" is unilateral or bilateral. With one-sided - 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 chest or pelvic cavity, in the iliac or lumbar region.
6. Grown kidney. It can be bilaterally symmetrical (“biscuit-shaped” - both kidneys are completely fused, “horseshoe-shaped” - fusion occurs with the upper or lower poles), bilateral asymmetrical L, S - shaped, unilateral - L-shaped.
7. Dysplasia is an anomaly of the structure, in which the kidney has a reduced size, an abnormal structure of the parenchyma (dwarf, rudimentary).
8. Polycystic kidney disease - normal parenchymal tissue is modified in the form of cysts. Only insignificant healthy parts of the parenchyma of the organ function, not replaced by cysts. The pathology is bilateral.
9. Multicystic kidney - the tissues of the organ are replaced by multiple cysts containing fluid. This kidney is not functioning.
10. Megacalicosis - expansion of the cups 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 the addition of an infection, when negative symptoms appear. Kidney dystopia may be accompanied by recurrent abdominal pain.

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

Congenital pathologies of the bladder

This organ plays an important 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 failures in the course of the intrauterine formation of the unborn child under the influence of a number of adverse external or internal factors:

1. Agenesia. The bladder and urethra are absent in the body of the fetus, which is incompatible with life.
2. Doubling. The organ is divided into two parts by a longitudinal septum. With complete bifurcation - each part of the bladder has its own urethra and one ureter. Incomplete duplication, called a "two-chamber" bladder, is characterized by the presence of one common urethra and a single neck.
3. Diverticulum. This disease is characterized by the presence of sac-like "protrusions" of the bladder walls. In these formations, urine accumulates and stagnates, which contributes to the development of inflammatory processes and the formation of stones. Such anomalies of the bladder can be both congenital and acquired. The most characteristic symptoms are urinary retention, its absence or urination in two doses.
4. Exstrophy. A severe congenital defect in which the bladder does not have an anterior muscular wall, and in the lower abdomen there is a hole several centimeters in diameter. The posterior half of the bladder with the ureters protrudes into this open cavity, from which urine is excreted. This anomaly is combined with defects of other organs and splitting of the pubic bones. It is treated only by surgery.
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, urinary diverticula.
6. Narrowing of the lumen of the bladder neck. A significant proliferation of fibrous tissues in the neck of the organ, which prevents the outflow of urine from the bladder.

Congenital pathologies of the ureters

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

Deviations can be as follows:

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

Anomalies of the ureters, as a rule, are accompanied by congenital pathologies of other elements of the urinary system - the kidneys, bladder, urethra and reproductive organs:

1. Agenesia. The urination organ is absent on the right or left side. Unilateral - accompanied by the absence of a kidney.
2. Doubling. Tripling. It is characterized by a double (triple) pelvis. It happens complete and incomplete, unilateral and bilateral.
3. Retrocaval, retroiliacal ureter - rare anomalies of the position when the ureter intersects with the vessels, which lead to compression and obstruction of the ureter.
4. Ectopic mouth. Displacement of the mouth of the ureter inside the neck of the bladder (intravesical). Extravesical ectopia - displacement of the mouth of the ureter into the urethra, rectum, vas deferens, uterus.
5. Spiral annular, the 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 a change in their structure - hypoplasia (the lumen of the ureter is narrowed, the wall is thinned), neuromuscular dysplasia (absence 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 severe. Treatment is most often performed surgically.

Anomalies in the development of the urethra lead to both obstruction of urine output and reproductive dysfunction in men.

Congenital malformations of this organ include the following conditions:

1. Hypospadias. Atypical location of the urinary opening of the urethra due to the replacement of the anterior part of the urethra with a chord. This phenomenon is accompanied by deformation of the reproductive male organ.
2. Epispadias. It is 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, total. In girls - clitoral or subsymphyseal.
3. congenital valves. Folded formations of the mucous membrane inside the urethra, having the form of jumpers. They make it difficult to urinate, cause stagnation of urine, infection, the development of pyelonephritis and hydroureteronephrosis.

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

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

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

Treatment of this kind of congenital malformations is carried out surgically in the first months and years of life of infants.

CONTENT:

INTRODUCTION
Anomalies of the renal vessels
Kidney anomalies
Anomalies of the ureters
Bladder anomalies
Anomalies of the male genital organs

Kidney Anatomy

Kidneys:
paired organ
located along
both sides of
spine in
lumbar
areas

Embryogenesis:

The basis for the development of the kidney are three
structures:
Pronephros - ontogenetic residue 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. At 12-14 weeks.
its atrophy.
Metanephros - consists of secretory and collecting systems.
The secretory system of the kidney consists of the mesonephrogenic blastema,
and excretory - from the remainder of the Wolffian duct.
The renal cortex is from a metanephrogenic blastema.

The main features of the embryogenesis of the kidneys and ureters

nephrotomes
mesonephritic duct
urogenital ridge
1.
2.
3.
4.
5.
6.
7.
8.
Develop from the mesoderm;
The pronephros and primary kidney are rudimentary;
The pronephric ducts give rise to the Wolffian ducts and hence
ureters;
The Wolffian duct does not disappear and is involved in the development of the reproductive system in
male fetus;
From the aorta, vessels approach the tubules of the primary kidney, forming
capillary coil. As a result, a renal corpuscle is formed, consisting of
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;
A glomerular capsule is formed from the metanephrogenic tissue, convoluted and straight
tubules of the nephron.

Merged Müllerian ducts (9 weeks)

By 7 weeks Embryogenesis Wolff
ducts and ureters open into
urinary
sinus
separate
holes. Appears between them
accumulation of mesoderm (triangle
Bladder).
Volfian (vas deferens) ducts
move down, and the ureters
up.
Merged Müllerian ducts (9 weeks)
The genitourinary sinus is divided into 2
segment:
The ureters flow into the first,
from it are formed: urinary
bubble, female and part of male
urethra;
In the second - Volfov and
confluent Mullerian ducts,
from
him
formed:
Part
male
urinary
canal, distal part and
vestibule
Müllerian tubercle (9 weeks)

The muscular coat is formed from the surrounding mesenchyme.
The prostate is formed from epithelial outgrowths. At the 3rd month
embryogenesis ventral urogenital sinus
expands to form the bladder.
The bladder moves to the navel, connects with the allantois,
which for 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 region of the urogenital sinus is formed
part of the male urethra

DEVELOPMENT OF THE REGENERAL SYSTEM In the development of the male reproductive system, the Wolf channel takes part, and the female - the Müller channel

MULLER (PARAMESONEPHRAL) CHANNEL
at the 3rd week of embryogenesis along Volfov
channel, a cell cord is formed, gradually
it separates itself 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 one
through the common duct they empty into the urogenital
sinus

development of the gonads in both sexes
the early stages are the same
(indifferent stage)
the surface of the primary kidney is covered
coelomic epithelium (splanchnot)
on the medial surfaces of the primary
kidney
going on
thickening
coelomic
epithelium,
which
gets the name of the genital ridges
in the region of the genital ridges of their endoderm
yolk sac migrate primary
germ cells - gonoblasts
in the future, the genital ridges significantly
develop, begin to protrude into the cavity
bodies, separate from the primary kidney,
acquire an oval shape and become
into the gonad
V
process
development
genital
glands
coelomic cells and gonoblasts of germ cells
rollers grows 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 male
sex), where are the primary sexual
cells, which will be further
form
gametes,
And
cells
coelomic epithelium, of which there will be
take shape
follicular
And
interstitial cells of the ovary
Leydig and testicular Sertoli cells

Anomalies of development

1. With a disturbed cranial displacement, KIDNEY DYSTOPIA occurs;
2. Fusion of metanephrogenic tissue results in KIDNEY FUNCTION
(formation of a horseshoe kidney);
3. Splitting of the ureteral outgrowth leads to INCOMPLETE DOUBLE
URETER, and with an additional ureteral outgrowth - COMPLETE
DOUBLE URETER;
4. ADDITIONAL KIDNEY is formed as a result of the presence of an additional
area of ​​metanephrogenic tissue;
5. If an additional ureteral outgrowth is laid far from the main one,
then, accordingly, the mouth of the ureter will open either in the neck
bladder or urethra (ectopia)
URETER);
6. In the absence of a ureteral outgrowth on one side, it develops
unilateral KIDNEY AGENESIA and only half is formed
triangle;

7. If the urinary rectal septum for 5 weeks.
does not separate the cloaca - CONGENITAL CLOACA;
8. Due to incomplete separation - CONGENITAL
FISTULAS in combination with posterior atresia;
9.
Non-occlusion of the urinary tract leads to
formation of the vesico-umbilical fistula
(cyst);
10. Violation of the displacement of the bladder down leads to the formation
DIVERTICULUM OF THE URINARY BLADDER;
11. Violation of the anlage of the genital tubercle leads to the fact that the urogenital
the groove is partially or completely open (on the dorsal surface
cavernous bodies) - EPISPADIUM;
12. Violation of the fusion of the urogenital folds causes - HYPOSPADIA;

13. Absence of gonads -
AGENESIA,
incomplete
–
HYPOPLASIA;
14. Violation of the lowering of the testicles in
scrotum
–
ONEor
BILATERAL CYPTORCHISM;
15.
lowering
testicles
along
guides
fibers
guide ligament causes
ECTOPIA OF THE SCHOOL;
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
back
surface,
lateral
And
medial edge,
upper and lower
ends (poles).

Kidney Anatomy

Veins in the sinus of the kidney
lie ahead,
arteries and nerves
behind the veins
renal pelvis and
ureter posterior to
arteries.

Kidney Anatomy

The kidneys are
from the brain and
cortical
substances

Kidney Anatomy

Anomalies of the renal vessels

1.
Anomalies in the number and position of the renal vessels:
a) Accessory renal artery
b) Double renal artery
c) Multiple arteries
2.
Anomalies in 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
testicles into the right renal vein
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 veins of the kidneys:

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

Double renal artery

Multiple arteries

Oh, 11%

Renal artery aneurysm
Oh, 11%

Fibromuscular stenosis:

Fibromuscular stenosis:
More common in women.
The disease leads to
lumen of the renal artery
Is high diastolic and
low pulse pressure, and
refractoriness to hypotensive
therapy.
On
basis
renal
angiography.
Surgical balloon treatment
dilatation
placement of an arterial stent
perform reconstructive
operation

Fibromuscular stenosis
arteries

Arteriovenous fistula and renal aplasia

Kidney anomalies

Occur in 3-5.5% of patients
Make up 10% of all anomalies
MPS
In recent years they have not
decreasing trend

Kidney anomalies are divided into 5 groups:

quantity anomalies
magnitude anomalies
location anomalies
relationship anomalies
structure anomalies

Classification (N.A. Lopatkina)

1 Anomalies in the number of kidneys:
a) aplasia
b) Doubling of the kidney
c) Accessory kidney
2 Anomalies in the size of the kidneys: hypoplasia

Aplasia:

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

0,083% 1:1200

Aplasia of the kidney
0,083%
1:1200

Abnormalities in the number of kidneys

Doubling of the kidney
a common anomaly. Double kidney in length
much more than normal, it is often expressed
embryonic lobulation. Between the upper and lower kidneys
there is a furrow of varying severity. Upper
half of the doubled kidney is often smaller than the lower one.
The blood supply of the double kidney is carried out by 2 renal
arteries. Lymphatic circulation in each half of the doubled kidney
also separate. With complete doubling of the kidney, in each of the halves
there is a separate pyelocaliceal system, and in the lower
it is developed normally, and in the upper one it is underdeveloped. From every pelvis
passes through the ureter. Doubling of the parenchyma and vessels of the kidney without
doubling of the pelvis should be considered an incomplete doubling 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.

Doubling of the kidney
10,4%
Accessory kidney

Abnormalities in the number of kidneys

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 permanent
leakage of urine. The accessory kidney is located
below normal and is at the level of the lower
lumbar vertebrae or in the iliac region, less often
in the pelvis. Its size is variable, but most often
significantly reduced.
Diagnostics: excretory urography, scanning
kidneys, renal arteriography (aortography).
Indication for surgical treatment - implementation
nephrectomy - hydronephrosis, nephrolithiasis, pyelonephritis,
as well as tumor.

Additional (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 and ureter and is often located
below the normal kidney (in the iliac region, in the pelvis, in front of the pubic
symphysis).
Dimensions are usually significantly reduced except in cases
hydronephrotic transformation.
The accessory kidney has its own capsule, sometimes has a loose
connective tissue fusion with a normal kidney.
The accessory kidney is often abnormal (hypoplasia, doubling
pelvis and ureters, dystopia) and is combined with various defects
development of the main kidneys.
The ureter of the accessory kidney can open on its own
the bladder is lateral to and above the orifices of the main ureters, or
open outwardly.
The accessory kidney usually has clinical manifestations only when
the development of pyelonephritis, hydronephrosis, stones, tumors in it, or with
ectopic ureter.
Chronic pyelonephritis, urolithiasis
indication for nephrectomy
disease
And
others
serves

Accessory kidney

Complete doubling of the kidney

Doubling and hydronephrosis of the lower half of the kidney

Incomplete duplication of the kidney

Magnitude anomalies

Hypoplasia of the kidney is characterized by a normal histological
structure and the absence of impaired renal function. hypoplasia
it is more often unilateral, but it can also occur on both sides.
Diagnosis - excretory urography, radioisotope and
ultrasound scan of the kidneys.
Renal arteriography differentiates hypoplasia
from a reduced kidney size due to pathological
process (nephrosclerosis).
With hypoplasia, the lumen of the vessels both in the renal pedicle and inside
kidneys are evenly reduced, and with secondary atrophy there is
sharp
decrease
lumen
intrarenal
vessels,
their incorrect distribution in the kidney, a significant decrease
their quantity, especially in the cortex of the kidney, with a normal caliber
vessels
renal
legs.
With unilateral hypoplasia of the kidney, the patient needs treatment
only if there is a pathological process in it. Usually this
pyelonephritis, which is often complicated by wrinkling of the kidney and
arterial
hypertension.
IN
this
case
perform
nephrectomy.

Hypoplasia of the kidney

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 kidneys;
asymmetric - L and S shaped kidneys)

2,8%

Thoracic dystopia of the kidney
rare, may be vague
pain behind the sternum, often after eating.
Diagnosis - chest X-ray,
fluorography - detect a shadow in the chest
cavities
above
aperture.
WITH
help
excretory urography and kidney scan
a correct diagnosis can be made. At
thoracic
dystopian
kidneys
the ureter is longer than usual and is noted
high
discharge
vessels
kidneys.

Thoracic dystopia of the kidney

Anomalies in the location of the kidneys (dystopia)

Lumbar dystopia of the kidney
artery of the dystopic kidney 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 dystopia of the kidney

Edge

Anomalies in the location of the kidneys (dystopia)

Iliac dystopia
the kidney is located in the iliac fossa,
renal arteries are usually multiple,
arise from the common iliac artery.
Manifests
myself
pains
V
belly,
driven by pressure dystopian
kidneys to neighboring organs and nerve plexuses,
as well as signs of impaired urodynamics.

Iliac dystopia

Anomalies in the location of the kidneys (dystopia)

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

Pelvic dystopia

Anomalies in the location of the kidneys (dystopia)

Cross dystopia
characterized by displacement of one kidney behind
midline, whereby both kidneys
are located on one
sides. With kidney dystopia, blood vessels
short, extending lower than usual, kidney
deprived of mobility. The operation is carried out
only in the presence of a pathological process
in a dystopic kidney.

Pelvic cup dystopia (medially).

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

Cross dystopia.

Cross dystopia

ANOMALIES OF KIDNEY RELATIONSHIPS:

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

Relationship anomalies

Adhesions between both kidneys are considered
as relationship anomalies. Fusion of the kidneys
on their medial surface is called
gallet-shaped kidney. When connecting the top
poles of one kidney with the lower pole of the other
an S-shaped or L-shaped kidney is formed. At
1st form pelvic-ureteral segment
one kidney faces medially, and the other laterally; in the 2nd form, the long axes of the kidneys
perpendicular
Friend
friend.

biscuit kidney

S-shaped and L-shaped kidneys

Relationship anomalies

horseshoe
bud
characterized
connection
kidney
eponymous
poles.
The horseshoe kidney is almost immobile. More
durable
fixation
is
result
her
numerous vascular connections and a peculiar
forms. 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-shaped kidney

0,25%

Horseshoe kidney:

With this anomaly of the kidney
spliced ​​with each other
with their upper or, more often,
bottom poles.
This
promotes
more
frequent
traumatic
kidney damage
kidney pressure on neighboring
bodies
urolithiasis disease,
developmental hydronephrosis,
in it the tumor process,
more often in the isthmus.
when occurring in the kidney
diseases
requiring
surgical treatment.

horseshoe kidney

Classification

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

Anomalies in the structure of the kidneys:
Dysplastic kidney (rudimentary, dwarf kidney).
Multicystic kidney.
Polycystic kidney:
adult polycystic.
polycystic childhood.
Parapelvic cyst, calyx and pelvic cysts.
Cup-medullary anomalies:
a) megacalyx, polymegacalyx.
b) spongy kidney.
Associated anomalies of the kidneys:
a) with vesicoureteral reflux.
b) with infravesical obstruction.
c) with
vesicoureteral
reflux
And
infravesical obstruction.
d) with anomalies of other organs and systems - sexual, musculoskeletal, cardiovascular, digestive.

Structural anomalies

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

kidney dysplasia

Structural anomalies

Multicystic kidney - characterized
complete replacement of renal tissue
cysts and obliteration of the ureter
V
prilokhanochny
department
or
the absence of its distal part. More often
The entire process is one-sided.
Diagnose with aortography.

Multicystic kidney:

Multicystic kidney:
a rare anomaly characterized by
multiple cysts of various 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
does not appear clinically.
Diagnosis is established using
sonography and X-ray radionuclide
research methods
Surgical treatment, which is
nephrectomy.

Multicystic kidney disease

Structural anomalies

Solitary kidney cysts
Cystic kidney diseases are simple
solitary cysts, which may be congenital and
acquired. The origin of the latter is related to
compression of the hilum of the kidney by enlarged lymphatic
knots 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 are composed of fibrous
connective tissue and are lined with flat, and sometimes
stratified epithelium. The cyst does not communicate with
calyces and renal pelvis. Its content is more
some cases are serous, less often (12-15%) - hemorrhagic.

Solitary cyst of the kidney:

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 giant
formations with a volume of more than 1 liter.
Dermoid cysts of the kidneys are extremely rare.
They may contain fat, hair, teeth, and bones.
squeezing
pelvicalyceal
ureter,
vessels
kidney,
hemorrhage and malignancy.
the presence of hypoechoic homogeneous with clear
contours, a rounded liquid medium in the cortical
kidney zone.
low contrast
education.
more than 3 cm and the presence of its complications, percutaneous
cyst puncture, sclerosing agents are injected
(ethanol).
avascular
shadow
systems,
suppuration,
rounded

Solitary kidney cysts

Structural anomalies

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
petrificates
V
projections
medullary
substances
kidneys),
excretory urography (in the area
papillae
visible
group
small
cavities
V
cerebral
substance).

Spongy kidney:

Characterized
presence
congenital
multiple small cysts in the kidneys
pyramids.
Usually this pathology occurs with two
sides.
It is more common in men.
Spongy kidney symptoms can be pain
in the lower back and hematuria.
on x-ray data.
The overview image shows multiple
small shadows of calculi located in
zone of the renal medulla.
Treatment for spongy kidney is required only in
case of complications.

Spongy kidney (general view)

Polycystic kidney:

heavy
bilateral
kidney anomaly,
characterized by substitution
renal
parenchyma
multiple
cysts
of various sizes.
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 the urine: gross hematuria.
In the blood: marked anemia, increased levels
creatinine and urea.
Diagnosis
installed
on
basis
ultrasonic and X-ray radionuclide
research methods.
conservative
treatment
polycystic
is
V
symptomatic
And
antihypertensive therapy.
Surgical treatment is indicated for the 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.

URETERAL ANOMALIES:

Abnormalities in the number of ureters
◦ agenesis (aplasia);
◦ doubling (complete and incomplete);
◦ tripling.
Anomalies in the position of the ureters
◦ retrocaval;
◦ retroiliacal;
◦ ectopia of the mouth of the ureter.
Anomalies in the shape of the ureters
◦ spiral (annular) ureter.
Anomalies in the structure of the ureters
◦ hypoplasia;
◦ neuromuscular dysplasia (achalasia, megaureter,
megadolichoureter);
◦ congenital narrowing (stenosis) of the ureter;
◦ ureter valve;
◦ diverticulum of the ureter;
◦ ureterocele;
◦ vesicoureteral pelvic reflux.

Doubling of the pelvis and ureter:

1 in 150 newborns
girls are 5 times more likely
can be one- or two-sided, full (ureter
duplex) and incomplete (ureter fissus)
the mouth of the upper is located lower and more medially, and
the lower one is higher and more lateral. one mouth.
Complaints arise with the development of complications.
hydroureteronephrosis.
vesicoureteral pelvic reflux.
on
basis
excretory
urography,
multislice CT with contrast, MRI and
cystoscopy.
ureterocystoanastomosis, antireflux surgeries, heminephroureterectomy, nephroureterectomy.

tripling

13,4%

Full doubling
13,4%

incomplete doubling

Retrocaval ureter:

Retrocaval ureter:
a rare anomaly
which the ureter in the lumbar
department goes under the vena cava.
leads to obstruction of the passage of urine
with the development of hydroureteronephrosis.
Diagnosis is confirmed with
multislice CT and MRI.
implementation
ureteroureteroanastomosis
With
location
body
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 compartment
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 internal - ureteral mucosa.
At the apex of the ureterocele is a narrowed orifice
ureter.
There are two types of this anomaly of the ureters orthotopic
And
heterotopic
(ectopic) ureterocele.
A ureterocele causes a violation of the passage of urine, which
gradually leads to the development of hydroureteronephrosis.
A common complication of ureterocele is
stone in it.
Cystoscopy is the main diagnostic method
ureterocele.
transurethral
endoscopic
resection
ureterocele or its open resection with
ureterocystoanastomosis.

Ureterocele:

Ureterocele:

Incomplete dysplasia
7:1000
Plication of the ureter

Neuromuscular dysplasia

Ectopia of the mouth of the ureter:

Ectopia of the mouth of the ureter:
To intravesical species, include its displacement down and
medially to the neck.
at
their
extravesical
ectopia
open
V
urethra, paraurethral, ​​into the uterus,
vagina, vas deferens, seminal vesicle,
rectum.
manifested by urinary incontinence
normal urination.
excretory urography, CT, vaginography, urethro- and
cystoscopy, catheterization of the ectopic mouth and
retrograde urethro- and ureterography.
is to transplant the ectopic ureter into
bladder (ureterocystoanastomosis).

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

Anomalies of the urethra:

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

hypospadias

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

Anomalies of the urethra

1.
2.
3.
4.
5.
Hypospadias of the penis
head, paracapitate, distal-,
middle-, proximal third of the genital
member)
Scrotal hypospadias (distal,
middle third of the scrotum)
Scroto-perineal hypospadias
perineal hypospadias
Hypospadias without hypospadias

Hypospadias:

Hypospadias:
1: 250-300 newborns,
testicular insufficiency.
Hypospadias of the crown of the penis.
Paracapitate
(perivenous) hypospadias.
Hypospadias distal, middle and
proximal third of the penis.
Scroto-perineal
And
perineal forms of hypospadias
Hypospadias is diagnosed with
objective research, determine
the genetic sex of the child.
the operation is carried out with a significant
curvature of the glans penis and/
or metastenosis.

1:450-500

"hypospadias without hypospadias"

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

4 types of "hypospadias without hypospadias"

Diagnosis of hypospadias

set at an objective
research. In some cases
can be difficult to distinguish
scrotum and perineum
hypospadias from female false
hermaphroditism. In such cases
it is necessary to determine
the 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.
Surgery for capitate and coronal hypospadias
carried out with a significant curvature of the head
penis and/or meatostenosis.
Treatment methods aim to achieve two
main goals: creation of the missing part of the urethra with
the formation of its external opening in the normal
anatomical position and straightening of the penis
due to excision of connective tissue scars (chords).
Prognosis for timely plastic surgery
favorable operation.

Tactics of managing patients with hypospadias.
In "hypospadias without hypospadias" urination
only slightly disturbed, so the main
criterion for determining the need
surgical correction, is the degree
curvature of the penis.
Since when straightening it is necessary to cross
short, though normally open, urethra and
create for some time an artificial dystopia of her
outer hole, then the decision on the need
intervention is difficult and
responsible task. It must be taken into account how
persistence of the patient, and the experience of medical
institutions in the treatment of hypospadias.

Indications for surgery for paracapitate
Hypospadias.
Are narrowing of the external opening of the urethra,
obstructing the outflow of urine, and (or) a significant curvature
the penis and its glans. If the narrowing of the outer
urethral opening in these cases is an absolute
indication for surgical treatment (meatotomy) due to
its dangers to the overlying 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 dystopic hole by
head is impractical due to possible serious
complications (formation of strictures, curvature and
desolation of the vessels of the head, etc.).

Anomalies of the urethra

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

epispadias

malformation of the urethra
which is characterized by underdevelopment or lack of
more or less over the top of it
walls. The frequency of occurrence is less than that of
hypospadias - in about 1 in 50,000 newborns.
The urethra in this pathology
located on the back of the penis between
split cavernous bodies.
Distinguish:
head epispadias,
epispadias of the penis,
total epispadias.

Epispadias:

Congenital splitting 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 region of the crown on
dorsal surface 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
section of the urethra. Most often this form goes unnoticed.
Subpubic epispadias is characterized by a splitting of the urethra to
bladder neck and cleft clitoris.
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 consists in
reconstruction of the urethra and elimination of curvature of the penis.

epispadias

Epispadias of the glans penis

characterized by the fact that
anterior wall of the urethra
split to the coronary
grooves. Penis
slightly twisted and
lifted up.
Urination and erection
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 penis to the transition area of ​​the skin in the pubic region.
With this form of epispadias, there is
splitting of the pubic symphysis, and sometimes
divergence of the abdominal muscles.
The penis is shortened and curved to the side
anterior abdominal wall. urethral opening
has the shape of a funnel. Stream when urinating
directed upwards, urine is sprayed, which
causes clothes to get wet.
Sexual life is not possible because the penis
small size and during erection strongly
twisted.

Total (complete) epispadias

except for splitting the anterior wall of the urethra
characterized by splitting of the sphincter
Bladder. The urethra is funnel-shaped and
located immediately below the bosom.
This form is characterized by urinary incontinence.
due to underdevelopment of the urinary sphincter
bubble. Constant leakage of urine
to irritation of the skin in the scrotum and
perineum, dermatitis develops,
normal social adaptation is disturbed
child in the community of peers. noted
underdevelopment of the penis and scrotum.

Treatment

Surgical treatment
epispadias is carried out in
first years of life.
It consists in
urethral reconstruction and
elimination of curvature
penis.

Anomalies of the urethra

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

Congenital valves of the urethra

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

The valves of the urethra disturb the normal
urination, difficulty
bladder emptying,
lead to residual
urine, the development of hydroureteronephrosis
and chronic pyelonephritis.
Surgical treatment -
endourethral mucosal resection
lining of the urethra
along with the valve.

Congenital obliteration of the urethra

Congenital stricture of the urethra is a rare anomaly in which
there is a cicatricial narrowing of its lumen, leading to
urination disorders.
Congenital urethral diverticulum is also a rare malformation.
development, consisting in the presence of a saccular
protrusion of the posterior wall of the urethra. More often
localized in the anterior urethra. Manifested by dysuria
and excretion of drops of urine after the end of the act
urination. The diagnosis is established on the basis
urethrography and ureteroscopy, voiding
cystoureterography. Treatment is vissection
diverticulum.
Congenital urethral cysts develop as a result of
obliteration of the excretory openings of the bulbourethral glands.
Predominantly localized in the area of ​​the bulb
urethra. Allows diagnosis to be made
voiding cystourethrography. They are removed surgically.
way.

Doubling of the urethra is a rare malformation. It happens
complete and incomplete. Full doubling combined with doubling
penis. Incomplete duplication of the urethra is more common. IN
in most cases an extra urethra
the channel ends blindly. Extra urethra always
has an underdeveloped cavernous body. Treatment consists of
complete excision of the accessory urethra and
paraurethral passages.
Urethral-rectal fistulas - defect
development, which is almost always combined with atresia of the posterior
passage. Occurs as a result of underdevelopment
urinary septum.
Prolapse of the urinary tract
channel is a rare anomaly. Prolapsed mucous due to
microcirculation disorders have a bluish tinge, sometimes the bladder near the mouth,
slightly above and lateral to it.
Persistent stasis of urine in a diverticulum
promotes the formation of stones in it
and development of chronic inflammation.
difficulty urinating and
bladder emptying in two
stage.
based on ultrasound, cystography and
cystoscopy.
Treatment is operative,
diverticulum excision and suturing
resulting wall defect
Bladder.

Bladder exstrophy:

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

Urachus pathology

Testicular anomalies (5-7%)

Anorchism
Monorchism
Polyorchism
hypoplasia
Synorchism
cryptorchidism
Ectopic testis

Cryptorchidism:

malformation (from the Greek. kriptos - hidden and orchis testicle), in which there is an undescended
scrotum of one or both testicles.
is 3%,
Abnormal position of the testicle leads to its
anatomical and functional insufficiency up to
to atrophy
malignancy risk
The
vice
development
Maybe
be
unilateral
And
bilateral,
true and false.
Diagnosis is based on data
physical examination, sonography, CT,
testicular scintigraphy and laparoscopy.
Use hormone therapy with chorionic
go-nadotropin.
Surgical treatment is carried out in the first years
child's life
with inefficiency (orchidopexy).

Ectopic testis is a congenital malformation,
in which it is located in different
anatomical areas, but not in the course of its
embryonic pathway to the scrotum. This
the anomaly is distinct from cryptorchidism. IN
depending on the localization of the testicle distinguish
inguinal, femoral, perineal and
cross ectopia.
Surgical treatment - lowering the testicle into
corresponding half of the scrotum.
Prognosis for testicular development in cryptorchidism and
ectopia favorable if operation
performed in the first years of a child's life.

Anorchism

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

Polyorchism

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

testicular hypoplasia

testicular anomaly. At the same time, one
or both testicles are underdeveloped, reduced in
sizes up to 5-7 mm. double sided
underdevelopment
testicles
accompanied
hormonal deficiency and requires
hormone replacement therapy.

Anomalies of the penis

congenital phimosis
hidden penis
Ectopic penis
double penis

congenital phimosis:

congenital narrowing of the foramen
flesh, not allowing to expose the head
penis.
Up to 3 years in boys in the majority
cases recorded physiological
phimosis
In the case of a pronounced narrowing of the extreme
flesh resort to her circular
excision (circumcision).

phimosis

constriction of the foreskin that prevents
release
heads
from
preputial sac. Often with phimosis
balanoposthitis occurs. Phimosis is
predisposing factor in the development
penile tumors.

paraphimosis

infringement of the glans penis narrow
foreskin. When paraphimosis occurs
swelling of the head, severe pain, difficulty
urination, sharp swelling of the skin of the genital
member. In case of untimely administration
may develop necrosis of the infringing
rings.

hidden penis

extremely rare anomaly
which are normally developed
cavernous bodies are hidden
surrounding tissues of the scrotum and
skin of the pubic region.
The penis is usually
reduced in size, cavernous
bodies are defined only when
palpation in the folds of the surrounding
skin.

Short frenulum of the penis

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

Anomaly(from Greek. anomaly- deviation, unevenness) - a structural and / or functional deviation due to a violation of embryonic development. Anomalies of the genitourinary apparatus are widespread and account for about 40% of all congenital malformations. According to autopsy data, about 10% of people have various anomalies in the development of the genitourinary system. To understand the causes of 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 pronephros (pronephros). In humans and higher vertebrates, it quickly disappears, being replaced by a more important primary kidney.

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

3. Paramesonephric duct. 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. gonads arise relatively later in the form of an accumulation of germinal epithelium on the medial side mesonephros. The seminiferous tubules of the testis and the ovarian follicles containing eggs develop from germinal epithelial cells. From the lower pole of the gonad, a connective tissue cord stretches down the wall of the abdominal cavity. (gubernaculum testis)- conductor of the testicle, which with its lower end goes into the inguinal canal.

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

bud, permanent buds are formed (metanephros), The parenchyma of permanent kidneys (urinary tubules) develops from the nephrogenic cord. Starting from the 3rd month, the permanent kidneys, as functioning excretory organs, replace the primary ones. With the growth of the trunk, 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 the diverticulum of 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 where the urinary, genital tract and hindgut initially open. It looks like a blind sac, closed from the outside by a cloacal membrane. Later, a frontal partition appears inside the cloaca, which divides it into two parts: ventral (sinus urogenitalis) And dorsal (rectum). After the rupture of the cloacal membrane, both of these parts open outwards with two openings: sinus urogenitalis- anterior, opening of the genitourinary system, and rectum- anus (anus).

Associated with the urogenital sinus urinary sac(allantois), which in lower vertebrates serves as a reservoir for the excretion products of the kidneys, and in humans part of it turns into the bladder. Allantois consists of three departments: lower- sinus urogenitalis, from which the triangle of the bladder is formed; middle extended department, which turns into the rest of the bladder, and upper narrowed section, representing the urinary tract (urachus) going from the bladder to the navel. In lower vertebrates, it serves to divert the contents of the allantois, and in humans, by the time of birth, it becomes 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 from the merged lower parts. In men ductus paramesonephrici are reduced, and only the appendage of the testis remains from them (appendix testis) and prostatic uterus (utriculus prostaticus). Thus, in men, reduction and transformation into rudimentary formations undergo ductus paramesonephrici, and in women ductus mesonephrici.

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

In men, these rudiments undergo the following changes: the genital tubercle develops strongly in length, it forms 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 labioscrotal tubercles grow intensively and turn into the scrotum, growing together along the midline.

In women, the genital tubercle turns into a clitoris. Growing genital folds form the labia minora, but complete connection

folds do not occur sinus urogenitalis remains open, forming the vestibule of the vagina (vestibulum vaginae). The labioscrotal tubercles do not grow together, which then turn into large labia.

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

5.1. KIDNEY ANOMALIES Classification

Anomalies of the renal vessels

■ Number anomalies: solitary renal artery;

segmental renal arteries (double, multiple).

■ Position anomalies: lumbar; iliac;

pelvic dystopia of the renal arteries.

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

■ Congenital arteriovenous fistulas.

■ Congenital changes in the renal veins:

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

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

Abnormalities in the number of kidneys

■ Aplasia.

■ Kidney duplication (complete and incomplete).

■ Additional, third kidney.

Anomalies in the size of the kidneys

■ Kidney hypoplasia.

Anomalies in the location and shape of the kidneys

■ Kidney dystopia:

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

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

bilateral (horseshoe-shaped, biscuit-shaped, asymmetric - L- and S-shaped kidneys).

Anomalies in the structure of the kidneys

■ Kidney dysplasia.

■ Multicystic kidney.

■ Polycystic kidney disease: adult polycystic; polycystic childhood.

■ Solitary kidney cysts: simple; dermoid.

■ Parapelvic cyst.

■ Diverticulum of the calyx or pelvis.

■ Cup-medullary anomalies: spongy kidney;

megacalyx, polymegacalyx.

Associated anomalies of the kidneys

■ With vesicoureteral reflux.

■ With infravesical obstruction.

■ With vesicoureteral reflux and infravesical obstruction.

■ With anomalies of other organs and systems.

Anomalies of the renal vessels

quantity anomalies. These include the blood supply to the kidney by solitary and segmental arteries.

Solitary renal artery- this is a single arterial trunk extending from the aorta, and then dividing 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 extending from the aorta. An increase in their number should be attributed to the segmental loose type of the structure of the renal arteries. In the literature, including educational literature, often one of the two arteries supplying the kidney, especially if it is of a smaller diameter, is called additional. However, in anatomy, an accessory, or aberrant, artery is one that supplies blood to a specific part of the organ in addition to the main artery. Both of these arteries form a wide network of anastomoses between themselves in their common vascular pool. Two or more arteries of the kidney supply each one of its certain segments and do not form anastomoses between themselves in the process of dividing the anastomoses.

Thus, in the presence of 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 lower polar vessels of the kidney, if its resection is not planned.

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

From these positions, the number of renal arteries more 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 (doubled) renal artery, and with more of them - multiple type of structure of the arteries of the kidney(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 it can also be observed with a normal structure of the organ.

Anomalies in the position of the renal vessels - a malformation characterized by an atypical discharge of the renal artery from the aorta and determining the type of kidney dystopia. Allocate lumbar(with a low discharge of the renal artery from the aorta), iliac(when departing from the common iliac artery) and pelvic(when leaving the internal iliac artery) dystopia.

Anomalies of form and structure. Renal artery aneurysm- local expansion of the artery, due to the absence of muscle fibers in its wall. This anomaly is usually unilateral. A renal artery aneurysm can manifest itself as arterial hypertension, thromboembolism with the development of a kidney infarction, and if it ruptures, massive internal bleeding. With an aneurysm of the renal artery, surgical treatment is indicated. Resection of the aneurysm, suturing of the defect of the vascular wall

or plasty of the renal artery with synthetic materials.

Fibromuscular stenosis- anomaly of the renal arteries, due to the excessive content of fibrous and muscle tissue in the vascular wall (Fig. 5.2).

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

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)

lar stenosis is high diastolic and low pulse pressure, as well as refractoriness to antihypertensive therapy. The diagnosis is established on the basis of renal angiography, multislice computed angiography and radioisotope examination of the kidneys. Perform selective blood sampling from the renal vessels to determine the concentration of renin. Treatment is operative. Balloon dilatation (expansion) of renal artery stenosis and / or installation of an arterial stent is carried out. If angioplasty or stenting is impossible or ineffective, a reconstructive operation is performed - renal artery prosthesis.

Congenital arteriovenous fistulas - a malformation of the renal vessels, in which there are pathological fistulas between the vessels of the arterial and venous circulatory systems. Arteriovenous fistulas, as a rule, are localized in the arcuate and lobular arteries of the kidney. The disease is often asymptomatic. Possible clinical manifestations of it 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 in endovascular occlusion (embolization) of pathological anastomoses with special emboli.

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

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

Anomalies in shape and location include annular(passes in two trunks around the aorta), retroaortic(passes behind the aorta and flows into the inferior vena cava at the level of II-IV lumbar vertebrae) extracaval(flows not into the inferior vena cava, but more often into the left common iliac vein) renal veins. The diagnosis is based on the data of venocavography, selective renal venography. In cases of severe venous hypertension, they resort to surgery - the imposition of 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, however, information about them is extremely important when planning surgical interventions. Clinically, malformations of the renal vessels are manifested in those cases when they cause a violation of the outflow of urine from the kidneys. The diagnosis is established on the basis of Doppler ultrasound scanning, aorto- and veno-cavography, multislice CT and MRI.

Abnormalities in the number of kidneys

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 the underdevelopment of metanephrogenic tissue. In half of the cases, the corresponding ureter is absent on the side of the aplasia of the kidney, in other cases its distal end ends blindly (Fig. 5.4).

Aplasia of the kidney 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 (vicar) 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 feature 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, multislice computed and magnetic resonance angiography). The cystoscopic picture is characterized by the absence of the corresponding half of the interureteral fold and the orifice of the ureter. With a blindly ending ureter, its mouth is hypotrophic, there is no contraction and excretion of urine. This type of defect is confirmed by catheterization of the ureter with the performance of retrograde ureterography.

Rice. 5.5. Sonogram. Doubling of the kidney

Doubling of the kidney- the most common anomaly in the number of kidneys, occurs in one case in 150 autopsies. In women, this malformation is observed 2 times more often.

As a rule, each of the halves of the doubled kidney has its own blood supply. Characteristic of such an anomaly is the anatomical and functional asymmetry. The upper half is often less developed. The symmetry of the organ or the 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 doubling implies the presence of two pyelocaliceal systems, two ureters, opening with two mouths in the bladder (ureter duplex). With incomplete duplication, the ureters eventually merge into one and open at one mouth in the bladder (ureter fissus).

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

Rice. 5.6. Excretory urograms:

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

(opening into the urethra or vagina), the formation of an ureterocele, or the failure of the vesicoureteral fistula 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 found in patients during a random examination. However, it is more often than normal, prone to various diseases, such as pyelonephritis, urolithiasis, hydronephrosis, nephroptosis, neoplasms.

The diagnosis is not difficult and consists in 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 violations of urodynamics associated with an abnormal course of the ureters, as well as other diseases of the double kidney.

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

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

Anomaly in the size of the kidneys

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

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

One-sided renal hypoplasia may not be clinically manifested, however, in an abnormal kidney, pathological processes develop much more often. bilateral hypoplasia is accompanied by symptoms of arterial hypertension and renal insufficiency, the severity of which depends on the degree of congenital defect and complications arising mainly from 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 established on the basis of data from ultrasound diagnostics (Fig. 5.7), excretory urography, CT and radioisotope scanning (Fig. 5.8).

Of particular difficulty is the differential diagnosis of hypoplasia from dysplasia And wrinkled as a result of nephrosclerosis of the kidney. Unlike dysplasia, this anomaly is characterized by the normal structure of the renal vessels, pelvicalyceal system and ureter. Nephrosclerosis is more 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 cups.

Treatment of patients with a hypoplastic kidney is carried out with the development of pathological processes in it.

Anomaly in the location and shape of the kidneys

Anomaly of the location of the kidney - dystopia- finding the kidney in an anatomical region 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 fetal development. Dystopia is caused by fixation of the kidney in the early stages of embryonic development by an 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).

Anomalies in the location of the kidneys can be unilateral And bilateral. Kidney dystopia without 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 malformation detected with a frequency of 1: 10,000 autopsies. It is characterized by the 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 in the normal location of the kidneys. The bladder triangle is preserved.

A dystopian kidney can cause constant or recurrent pain in the corresponding half of the abdomen, lumbar region, and 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 (cross) dystopia of the right kidney

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

This anomaly is in the first place among the causes of erroneously performed surgical interventions, since the kidney is often mistaken for a tumor, appendicular infiltrate, pathology of the female genital organs, etc. Pyelonephritis, hydronephrosis, and urolithiasis often develop in dystopic kidneys.

The greatest difficulty in making a diagnosis is pelvic dystopia. Such an arrangement of the kidney can be manifested by pain in the lower abdomen and simulate an acute surgical pathology. Lumbar and iliac dystopias, even if not complicated by any disease, can be manifested by 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 anomalies in the position 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 location and, as a result of rotation, looks flattened (Fig. 5.11).

With 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 may originate from the abdominal aorta, aortic bifurcation, common iliac and hypogastric arteries (Fig. 5.13). The presence of multiple vessels feeding the kidney is characteristic. This anomaly is most clearly detected on multislice CT with

rastering (Fig. 39, see color insert). Incomplete rotation of the kidney and a short ureter are important differential diagnostic features to distinguish kidney dystopia from nephroptosis. The dystopic kidney, in contrast to the early stages of nephroptosis, is devoid of mobility.

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

Form anomalies include various types fusion of the kidneys between themselves. Fusion kidneys occur in 16.5% of cases among all their anomalies.

Fusion involves joining two kidneys into one organ. Cro-

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

its supply is always carried out by abnormal multiple renal vessels. In such a kidney, there are two pelvicalyceal 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 may also be associated with vesicoureteral pelvic reflux.

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

Kidney fusion may be symmetrical And asymmetrical. In the first case, the kidneys grow together with the same poles, as a rule, the lower and extremely rarely the upper (horseshoe-shaped kidney) or middle sections (biscuit-shaped kidney). In the second, fusion occurs with opposite poles (S-, L-shaped kidneys).

horseshoe kidney is the most common anomaly of union. In more than 90% of cases, fusion of the kidneys with the lower poles is observed. More often, such a kidney consists of symmetrical, identical kidneys in size and is dystopic. The size 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 with it at a right angle, across the spine, the kidney is called L-shaped.

In those cases when in an fused kidney lying on one side of the spine, the gate is directed in different directions, it is called S-shaped.

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

Clinically fused kidneys may present with pain in the para-umbilical region. 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. Multispiral CT (axial projection). horseshoe kidney

symptoms. With such a kidney, the appearance or intensification of pain in the umbilical region during bending the body back (Rovsing's symptom) is typical. There may be disorders of the digestive system - pain in the epigastric region, nausea, bloating, constipation.

Ultrasound, excretory urography (Fig. 5.14) and multispiral 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 with the development of diseases of the abnormal kidney (urolithiasis, pyelonephritis, hydronephrosis). When identifying hydronephrosis of the horseshoe kidney, it should be determined whether it is a consequence of the obstruction of the pyeloureteral segment characteristic of this disease (stricture, intersection of the ureter with the lower polar vascular bundle) or was formed due to pressure on it of the isthmus of the horseshoe kidney. In the first case, you need

perform plastic surgery of the pyeloureteral segment, and in the second - resection (rather than dissection) of the isthmus or ureterocalicoanastomosis (Neivert operation).

Anomalies in the structure of the kidneys

Kidney dysplasia is characterized by a decrease in its size with a simultaneous violation of the development of blood vessels, parenchyma, pyelocaliceal system and a decrease in renal function. This anomaly results from

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

insufficient induction of the metanephros duct on the differentiation of the metanephrogenic blastema after their fusion. Very rarely, such an anomaly is bilateral and is accompanied by severe renal failure.

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

The most common malformations of the structure of the kidney parenchyma are cortical cystic lesions (multicistosis, polycystosis and solitary cyst of the kidney). These anomalies are united by the mechanism of violation 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 terms of violation of such a 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 with multicystic kidney disease.

Multicystic kidney- a rare anomaly characterized by multiple cysts of various 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 violation of the connection of the metanephros duct with a metanephrogenic blastema and the absence of an excretory bookmark while maintaining the secretory apparatus of the permanent kidney at the early stages of its embryogenesis. Urine, formed, accumulates in the tubules and, having no way out, stretches them, turning them into cysts. The contents of the cysts are usually a clear liquid, vaguely reminiscent of

urinating. 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-cystosis is incompatible with life.

Prior to infection, a unilateral multicystic kidney is not clinically manifested and may be an accidental finding during a dispensary examination. The diagnosis is established using sonography and X-ray radionuclide research methods with a separate determination of kidney function. In contrast

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

Rice. 5.19. Sonogram. Polycystic kidney disease

from polycystic, multicystic is always a unilateral process with a lack of function of the affected organ.

Treatment is surgical, consisting in nephrectomy.

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

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

In pathogenetic and clinical terms, this anomaly is divided into polycystic kidney disease in children and adults. For polycystic childhood, an autosomal recessive single type of transmission of the disease is characteristic, for polycystic adults - autosomal dominant. This anomaly in children is severe, most of them do not survive to adulthood.

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

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

Patients complain of pain in the abdomen and lumbar region, weakness, fatigue, thirst, dry mouth, headache, which is associated with chronic renal failure and high blood pressure.

Rice. 5.20. Excretory urogram. Polycystic kidney disease

Rice. 5.21. CT. Polycystic kidney disease

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

In blood tests, anemia, increased levels of creatinine and urea are noted. The diagnosis is established on the basis of ultrasound and X-ray radionuclide research methods. Characteristic features are increased in times

measures of the kidney, entirely represented by cysts of various sizes, compression of the pelvis and calyces, the necks of which are elongated, the 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 dispensary observation at the urologist and the nephrologist. Surgical treatment is indicated for the development of complications: suppuration of cysts or malignancy. Given the bilateral process, it should be of an organ-preserving nature. In a planned manner, percutaneous puncture of cysts can be performed, as well as their excision by laparoscopic or open access. In severe chronic renal failure, hemodialysis and kidney transplantation are indicated.

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

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

A simple cyst is usually single (solitary), although there are 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.

Dermoid cysts of the kidneys are extremely rare. They may contain fat, hair, teeth, and bones that can be seen on x-rays.

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

Rice. 5.22. Sonogram. Cyst (1) kidney (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. There may be a rupture of a large kidney cyst.

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

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

On excretory urograms, multislice CT with contrast and MRI, the kidney is enlarged in size due to a rounded thin-walled homogeneous liquid formation, to some extent deforming the pelvicalyceal system and causing deviation of the ureter (Fig. 5.23). The pelvis is compressed, the calyces are pushed aside, moved apart, with obstruction of the neck of the calyx, hydrocalyx occurs. These studies also make it possible to identify anomalies 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 the accumulation of the radiopharmaceutical.

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, polycystic, hydronephrosis and, especially, neoplasms of the kidney.

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 is subject to cytological examination. If necessary, perform a cystography. After evacuation of the contents, sclerosing agents (ethyl alcohol) are injected into the cyst cavity. The method gives a high percentage of relapses, since the cyst membranes that can produce fluid are preserved.

Currently, the main method of treatment 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 a huge size, has a multifocal character 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 the 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 the parapelvic cyst are due to its location, that is, pressure on the pelvis and vascular pedicle of the kidney. Patients experience pain. Hematuria and arterial hypertension may be observed.

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

The need for treatment arises with a significant increase in the size of the cyst and the development of complications. Technical difficulties in its excision are associated with the proximity of the pelvis and renal vessels.

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

Rice. 5.26. Multislice CT with contrast. Diverticulum of the calyx of the left kidney (arrow)

necks or pelvises. 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 pelvicalyceal system of the kidney is clearly established.

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

Cup-medullary anomalies.spongy kidney- a very rare malformation characterized by cystic expansion of the distal

parts of the collecting ducts. The lesion 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, with little to no impairment of kidney function.

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

Spongy kidney is diagnosed by X-ray methods. On review and excretory urograms, nephrocalcinosis is often detected - 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 revealed. Some of them protrude into the lumen of the cups, resembling a bunch of grapes.

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

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

Megacalix (megacalicosis)- congenital non-obstructive expansion of the calyx, resulting from medullary dysplasia. The expansion of all groups of calyces is called polymegacalyx (megapolycalicosis).

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, poorly differentiated. Extended

Rice. 5.27. Excretory urogram. Megapolycalicosis on the left

the calyces can pass directly into the pelvis, which, unlike the case of hydronephrosis, retains normal dimensions. The pyeloureteral segment is usually formed, the ureter is not narrowed. In an uncomplicated course, the kidney function is not impaired. Expansion of the calyxes is not due to obstruction of their necks, as is the case with the presence of a stone in this area or Frehley's syndrome (compression of the neck of the calyx by a segmental arterial trunk), but is congenital non-obstructive in nature.

For diagnosis, ultrasound, radiological methods with contrasting of the urinary tract are used. On excretory urograms, the expansion of all groups of cups is determined with the absence of ectasia of the pelvis (Fig. 5.27).

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

5.2. URETERAL ANOMALIES

Malformations ureters account for 22% of all anomalies of the urinary system. In some cases, they are combined with anomalies in the development of the kidneys. As a rule, anomalies of the ureters lead to a violation of urodynamics. The following classification of malformations of the ureters has been adopted.

■ agenesia (aplasia);

■ doubling (complete and incomplete);

■ tripling.

■ retrocaval;

■ retroiliacal;

■ ectopia of the mouth of the ureter.

Anomalies in the shape of the ureters

■ spiral (annular) ureter.

■ hypoplasia;

■ neuromuscular dysplasia (achalasia, megaureter, megadolichoureter);

■ congenital narrowing (stenosis) of the ureter;

■ ureter valve;

■ diverticulum of the ureter;

■ ureterocele;

■ vesicoureteral pelvic reflux. Abnormalities in the number of ureters

Agenesia (aplasia)- congenital absence of the ureter, due to underdevelopment of the ureteral 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 multicystosis. bilateral is extremely rare and incompatible with life.

Diagnosis is based on the data of X-ray methods of examination with contrast and nephroscintigraphy, which reveal the absence of one kidney. Characteristic cystoscopic signs are underdevelopment or absence of half of the bladder triangle and the mouth of the ureter on the corresponding side. With the preserved distal ureter, 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 with the development of a purulent-inflammatory process and the formation of stones in the blindly ending ureter. Perform surgical removal of the affected organ.

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

As a rule, during caudal migration, the ureter of the lower half of the kidney is the first to connect to the bladder and, therefore, occupies a higher and lateral position than the ureter of its upper half. The ureters in the pelvic regions mutually cross and flow into the bladder in such a way that the mouth of the upper one is located lower and more medially, and the lower one is higher and more lateral (Weigert-Meyer law) (Fig. 5.29).

Doubling of the upper urinary tract can be one- or bilateral, full (ureter duplex) And incomplete (ureter fissus)(Fig. 41, 42, see color insert). In case of full 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 doubling of the urinary tract

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

The described features of the topography of the ureters in doubling the upper urinary tract are predisposing to the occurrence of 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 in this ureter. On the contrary, the orifice of the ureter of the upper half of the kidney is often ectopic and predisposed to stenosis, which is the cause of hydroureteronephrosis.

Doubling of the ureter in the absence of a violation of urodynamics is not clinically manifested. This anomaly can be suspected by sonography, in which the doubling of the kidney is determined, and the ureters, in the presence of expansion, can be seen 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.

Tripling of the pelvis and ureters is casuistry.

Treatment is operative in the development of complications. 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 one of its halves - heminephroureterectomy.

Anomalies in 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 circled around it in an annular shape, returns to its previous position when it passes into the pelvic region (Fig. 43, see color insert). Compression of the ureter by the inferior vena cava leads to a violation of the passage of urine 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 cavitary system of the kidney and ureter to its middle third, a loop-shaped bend and a normal structure of the ureter in the pelvic region. The diagnosis is confirmed by multislice CT and MRI.

Surgical treatment consists in crossing, as a rule, with resection of the altered sections of the ureter and performing an ureteroureteroanastomosis with the location 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 in crossing the ureter, releasing it from under the vessels and performing an antevasal ureteroureteroanastomosis.

Ectopia of the mouth of the ureter- an anomaly characterized by an atypical intra or extravesical location of the orifices of one or both ureters. This malformation is more common in girls and is usually associated with duplication of the ureter and/or ureterocele. The cause of this anomaly is a delay or violation of the separation of the ureteric germ from the Wolffian duct during embryogenesis.

TO intravesical types of ectopia of the mouth of the ureter include its displacement down and medially to the neck of the bladder. Such a change in the location of the mouth, as a rule, is asymptomatic. The mouths of the ureters with their extravesical ectopias open into the urethra, paraurethral, ​​into the uterus, vagina, vas deferens, seminal vesicle, rectum.

The clinical picture of extravesical ectopia of the mouth of the ureter is determined by its localization and depends on gender. In girls, this malformation is manifested by urinary incontinence while maintaining normal urination. In boys, during the intrauterine development of the wolfs, the duct turns into the vas deferens and seminal vesicles, so the ectopic mouth 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.

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

Anomalies 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 can be 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.

Twisting 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 establish the diagnosis.

Surgical treatment. Resection of the ureter with ureteroureteroanastomosis or ureterocystoanastomosis is performed.

Anomalies in the structure of the ureters

hypoplasia of the ureter is usually combined with hypoplasia of the corresponding kidney or its half when doubling, as well as with a multicystic kidney. The lumen of the ureter with this anomaly is sharply narrowed or obliterated, the wall is thinned, peristalsis is weakened, the mouth 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 the expansion and lengthening of the ureter (similar to the term "megacolon"). This is one of the frequent and severe malformations of the ureters, due to the underdevelopment or complete absence of its muscular 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 its even greater expansion and elongation with the formation of cranked kinks (megadolichoureter). The deterioration of urine transport is facilitated by the normal tone of the bladder detrusor and the combination of this anomaly with other malformations (ectopia of the ureter orifice, ureterocele, vesicoureteropelvic reflux, neurogenic bladder dysfunction). Frequent accession of infection against the background of urostasis contributes to the development of chronic ureteritis, followed by scarring of the ureteral wall and an even greater decrease in the function of the upper urinary tract. The characteristic histological features of the megaureter are a significant underdevelopment of the neuromuscular structures of the ureter with a predominance of scar tissue.

Achalasia ureter is a neuromuscular dysplasia of its pelvic region. The underdevelopment of the ureter in 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 in the development of the megaureter, but one of its varieties. As a rule, the expansion of the ureter in its pelvic region with achalasia remains at the same level throughout life. In some cases, the overlying ureter may be involved

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

into the pathological process secondarily as a result of stagnation of urine in an enlarged 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 a megaureter are due to the addition of chronic pyelonephritis. There are pains in the corresponding lumbar region, fever with chills, dysuria. A severe clinical course is observed with bilateral megaureter. From an early age, symptoms of chronic renal failure are detected.

sufficiency: child's lag in physical development, decreased appetite, polyuria, thirst, weakness, fatigue.

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

With a megaureter, there is an increase in length and a significant expansion of the ureter along its entire length with areas of knee-shaped kinks. Antegrade pyeloureterography makes it possible to establish a diagnosis in the absence of kidney 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 neuromuscular dysplasia of the ureter largely depends on the stage of the disease. More than 100 methods of operative 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 in resection of the dilated ureter in 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 prilokhanochny, less often - prevesical departments, as a result of which hydronephrosis or hydroureteronephrosis develops. Due to the frequency, features of the etiology, pathogenesis, clinical course and methods of surgical correction, hydronephrotic transformation is singled out as a separate nosological form and is discussed in Chapter 6.

Ureteral valves- these are local duplications of the mucous and submucosal or less often all layers of the ureter wall. This anomaly is extremely rare. The reason for its formation is a congenital excess of the ureteral mucosa. 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 portion of the ureter with anastomosis between the unchanged parts of the urinary tract.

A diverticulum of the ureter is a rare anomaly that manifests itself as a saccular protrusion of its wall. Most often there are diverticula of the right ureter with predominant localization in the pelvic region. Bilateral diverticula of the ureter have also been described. The wall of the diverticulum consists of the same layers as the ureter itself. Diagnosis is based on excretory urography, retrograde ureterography, helical 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 in resection of the diverticulum and the wall of the ureter 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 refers to frequent anomalies and is diagnosed in 1-2% of patients of all age groups subjected to cystoscopy.

The ureterocele may be one- And bilateral. The reason for its formation is a congenital neuromuscular underdevelopment of the submucosal layer of the intramural ureter in combination with the narrowness of its mouth. Due to such a malformation, a displacement (stretching) of the mucous membrane of this section of the ureter into the cavity of the bladder gradually occurs with the formation of a rounded or pear-shaped cystic formation 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 is a narrowed orifice of the ureter.

There are two types of this anomaly of the ureters - orthotopic And heterotopic (ectopic) ureterocele. The first occurs with the normal location of the mouth of the ureter. It is small, well reduced and, as a rule, does not interfere with the outflow of urine from the kidneys. Such an asymptomatic ureterocele is more often diagnosed in adults. A heterotopic ureterocele occurs when there is low ectopia of the orifice of the ureter towards the outlet of the ureter.

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

A ureterocele causes a violation of the passage of urine, which gradually leads to the development of hydroureteronephrosis. A frequent complication of ureterocele is the formation of a stone in it.

Clinical symptoms depend on the size and location of the ureterocele. The larger the ureterocele and the more pronounced the obstruction of the ureter, the earlier and more clearly the symptoms of this anomaly appear. There are pains in the corresponding lumbar region, with the formation of a stone in it and the attachment of an infection - dysuria. If the ureterocele is large, there may be difficulty urinating due to obstruction of the bladder neck. In women, the ureterocele may protrude beyond the urethra.

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

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

Cystoscopy is the main method for diagnosing ureterocele (Fig. 15, see color inset). 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. Ureterocele is defined as a round formation located in the triangle of the bladder, at the top of which the mouth of the ureter opens, when urine is excreted, the ureterocele contracts and decreases in size (falls off).

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 dilatation 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 is 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 failure (incomplete maturation) of the vesicoureteral fistula. Secondary - is a complication of infravesical obstruction, developing due to increased pressure in the bladder.

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

A characteristic clinical manifestation of VUR is the occurrence of pain in the lumbar region during urination. When the infection is attached, symptoms of chronic pyelonephritis appear.

X-ray radionuclide research methods play a leading role in the diagnosis of VUR. Retrograde cystography at rest and during urination (micting cystography) reveals not only its presence, but also the severity of the anomaly (see Chapter 4, Figure 4.32).

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

of which the simplest is the endoscopic submucosal introduction of shaping bioimplants (silicone, collagen, Teflon paste, etc.) into the mouth area, preventing the reverse flow of urine. Operations for the reconstruction of the ureteral orifice, which are currently performed, including with the use of robotic-assisted technique, have found wide application.

5.3. BLADDER ANOMALIES

There are the following malformations of the bladder:

■ anomalies of the urinary duct (urachus);

■ bladder agenesis;

■ duplication of the bladder;

■ congenital bladder diverticulum;

■ bladder exstrophy;

■ congenital contracture of the bladder neck.

urachus(urachus)- the urinary duct, which connects the emerging bladder through the umbilical cord with the amniotic fluid during the period of intrauterine development of the fetus. Usually, by the time the child is born, it overgrows. With malformations, the urachus may not completely or partially overgrow. Depending on this, anomalies of the urachus are distinguished.

umbilical fistula- non-closure of part of the urachus, opening with 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 non-closure of the urachus. In this case, there is a constant release of urine from the fistula.

Urachus cyst- occlusion of the middle part of the urinary duct. Such an anomaly is asymptomatic and manifests itself only with large sizes or suppuration. In some cases, it can be felt through the anterior abdominal wall.

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

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

Bladder doubling- also a very rare anomaly of this organ. It is characterized by the presence of a septum that divides the cavity of the bladder into two halves. The mouth of the corresponding ureter opens into each of them. This anomaly may be accompanied by a doubling of the urethra and the presence of two bladder necks. Sometimes the septum may be incomplete, and then there is a "two-chamber" bladder (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, somewhat higher and lateral to it.

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

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

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

Diagnosis is based on ultrasound (Fig. 5.35), cystography (Fig. 5.36) and cystoscopy (Fig. 20, see color insert).

Surgical treatment consists in excising the diverticulum and suturing the formed 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. Bladder exstrophy is often combined with malformations of the upper and lower urinary tract, prolapse

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

Rice. 5.36. Descending cystogram. Bladder diverticula

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

Urine with such an anomaly is constantly poured out, which further leads to maceration and ulceration of the skin of the perineum, genitals and thighs. When the child strains (when laughing, screaming, crying), the wall of the bladder protrudes in the form of a ball, and urine output increases. The mucous membrane is hyperemic, bleeds easily. In the lower corners of the defect, the orifices of the ureters are determined. Bladder exstrophy, as a rule, is combined with diastasis of the bones of the pubic joint, 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 a 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 own tissues;

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

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

Contracture of the bladder neck- a malformation characterized by excessive development of connective tissue in a given anatomical region. 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 an instrumental study (uroflowmetry in combination with cystomanometry), urethrography and urethrocystoscopy with biopsy of the bladder neck. Endoscopic treatment consists in dissection or excision of scar tissue.

5.4. URINE ANOMALIES

Malformations of the urethra include:

■ hypospadias;

■ epispadias;

■ congenital valves, obliterations, strictures, diverticula and cysts of the urethra;

■ hypertrophy of the seed tubercle;

■ duplication of the urethra;

■ urethro-rectal fistulas;

■ prolapse of the mucous membrane of the urethra.

hypospadias- congenital absence of a section of the anterior urethra with the replacement of the missing part with 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, strongly curved in the dorsal direction. The bend is especially pronounced during erection. The angle of curvature can be so great that sexual life becomes impossible. Usually the foreskin is split and covers the head in the form of a hood. Meatostenosis may occur.

Allocate capitate(most common) coronal, 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 coronal 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 proximal it is ectopic, the more pronounced the curvature of the organ. Due to bending and meatostenosis emptying of the bladder is difficult, the jet is weak, directed downwards.

The most severe are the scrotal and perineal forms of hypospadias. They are characterized by a sharp underdevelopment and curvature of the penis and a pronounced violation of urination, which is possible only 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 the usual place on the glans penis, but it is itself significantly shortened. Between the shortened urethra and the normal length of the penis is a dense connective tissue cord (chord), which makes the penis sharply curved in the dorsal direction.

Extremely rare female hypospadias, in which the posterior wall of the urethra and the anterior wall of the vagina are split. It can be accompanied by stress urinary incontinence.

The diagnosis of hypospadias is established by an 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 allow you 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. With capitate and coronal hypospadias

the operation is performed with a significant curvature of the glans penis and / or meatostenosis. To correct more severe forms of hypospadias, many different methods of surgical treatment 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 excising connective tissue scars (chords). The prognosis for timely plastic surgery is favorable. Good cosmetic effect, normal urination, preservation of sexual and reproductive function are achieved.

epispadias- congenital splitting along the anterior surface of the entire or part of the urethra. Anteriorly open, this section of the urethra, together with the cavernous bodies, creates a characteristic groove that runs 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.

There are three types of epispadias in boys: capitate, stem And total. Epispadias of the glans penis characterized by the fact that the anterior wall of the urethra is split to the coronal groove. The penis is slightly curved and raised up. Urination and erection in this form of epispadias are usually not disturbed.

stem form characterized by the fact that the anterior wall of the urethra is split throughout the entire penis - up to the area of ​​​​transition of the skin into the pubic region. With this form of epispadias, there is a splitting of the pubic symphysis, and sometimes a divergence of the abdominal muscles. The penis is shortened and curved towards the anterior abdominal wall. The opening of the urethra is funnel-shaped. When urinating, the jet is directed upwards, urine is sprayed, which leads to wet clothes. Sexual life is impossible, as the penis is small and strongly curved during erection.

Total (complete) epispadias in addition to splitting the anterior wall of the urethra, it is characterized by splitting of the sphincter of the bladder. The urethra is funnel-shaped and is located just below the womb. This form is characterized by urinary incontinence due to underdevelopment of the sphincter of the bladder. Constant leakage of urine leads to irritation of the skin in the scrotum and perineum, dermatitis develops, and the normal social adaptation of the child in the society of peers is disturbed. Underdevelopment of the penis and scrotum is noted.

Epispadias in girls is less common than in boys. Three forms are distinguished. clitoral epispadias, characterized only by splitting of the clitoris. The external opening of the urethra is displaced upward and opens above it. Urination is not disturbed.

At subsymphyseal form there is a splitting of the urethra to the neck of the bladder and splitting of the clitoris. The most severe is full epispadia, 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 pubic symphysis and sphincter of the bladder, which is manifested by duck gait and urinary incontinence.

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

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

Congenital valves of the urethra- the presence in its proximal section of pronounced mucosal folds protruding into the lumen of the urethra in the form of bridges. This anomaly is more common in boys and is 1 in 50 thousand newborns. The valves of the urethra disrupt normal urination, make it difficult to empty the bladder, lead to the appearance of residual urine, the development of hydroureteronephrosis and chronic pyelonephritis. Treatment of urethral valves is endoscopic. Their TUR is performed.

Congenital obliteration of the urethra is extremely rare, 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 diverticulum of the urethra- also a rare malformation, consisting in the presence of a saccular protrusion of the posterior wall of the urethra. Most often localized in the anterior urethra. It is manifested by dysuria and the release of drops of urine after the end of the act of urination. The diagnosis is established on the basis of urethrography and ureteroscopy, voiding cystoureterography. Treatment consists of excision of the diverticulum.

Congenital cysts of the urethra develop as a result of obliteration of the excretory openings of the bulbourethral glands. Predominantly localized in the region of the bulb of the urethra. They are removed surgically.

Hypertrophy of the seminal tubercle- congenital hyperplasia of all elements of the seminal tubercle. Causes urethral obstruction and erection during urination. It is diagnosed by ureteroscopy and retrograde urethrography. Treatment consists in TUR of the hypertrophied part of the seminal tubercle.

Doubling the urethra- a rare malformation. It is complete and incomplete. Full doubling combined with a 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 cavernous body.

Urethral rectal fistulas- a rare malformation, which is almost always combined with an atresia of the anus. Occurs as a result of underdevelopment of the urinary rectal septum.

Prolapse of the mucous membrane of the urethra is also a rare anomaly. The mucosa that has fallen out due to a violation of microcirculation has a bluish tint, sometimes it bleeds. Treatment is operative.

5.5. ANOMALIES OF THE MALE GENITAL ORGANS

Testicular anomalies

Malformations of the testicles are divided into anomalies in the number, structure and position. Quantity anomalies include:

Anorchism- congenital absence of both testicles. It is combined with the underdevelopment of other genital organs. Differential diagnosis with bilateral abdominal cryptorchidism is based on testicular scintigraphy, CT, MRI, and laparoscopy. Treatment consists in the appointment of hormone replacement therapy.

Monorchism- congenital absence of one testicle, its epididymis and vas deferens. It should be distinguished from unilateral abdominal cryptotorchism using similar diagnostic methods as for anorchism. For cosmetic purposes, prosthetic testicles are possible.

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

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

Structural anomalies include testicular hypoplasia- his congenital underdevelopment. It is diagnosed during an 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.

Anomalies in the position of the testicles include:

cryptorchidism- a malformation (from the Greek kriptos - hidden and orchis - testicle), in which one or both testicles are not descended into the scrotum. Hour-

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

The abnormal position of the testicle leads to its anatomical and functional failure up to 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 of an undescended testicle, in contrast to a normally located one, increases significantly.

Depending on the degree of failure, there are abdominal And inguinal forms of cryptorchidism

Rice. 5.37. Forms of cryptorchidism and testicular ectopia:

1 - normally located testicle; 2 - testicular delay before entering the scrotum; 3 - inguinal ectopia; 4 - inguinal creep-torchism; 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 malformation may be unilateral And bilateral, true And false. False (pseudocryptorchidism) is noted with excessive testicular mobility, when it is the result of contraction of the muscle that lifts the testicle (t. cremaster), pulls tightly to the outer inguinal ring or even plunges into the inguinal canal. In a relaxed state, it can be brought down into the scrotum with gentle movements, but it often returns.

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

testicular scintigraphy and laparoscopy. Based on the same methods, cryptorchidism is differentiated from anorchism, monorchism, and testicular ectopia.

Conservative treatment is indicated when the testis is located in the distal part of the inguinal canal. Hormone therapy with chorionic gonadotropin is used. Surgical treatment is carried out in the first years of a child's life with the ineffectiveness of hormonal therapy. It consists in opening the inguinal canal, mobilizing the testicle, spermatic cord and bringing it down into the scrotum with fixation in this position (orchidopexy).

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

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

Anomalies of the penis

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

hidden penis- an extremely rare anomaly in which normally developed cavernous bodies are hidden by the surrounding tissues of the scrotum and skin of the pubic region. The penis, as a rule, is reduced in size, the cavernous bodies are determined only by palpation in the folds of the surrounding skin.

Ectopic penis- an extremely rare anomaly in which it is small and located behind the scrotum. Treatment is operative: the penis is moved to its normal position.

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

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 and polycystic kidney disease?

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

4. What are the types of ectopic ureteral orifice?

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

6. What is the clinical significance of ureterocele?

7. What are the anomalies of the urachus?

8. List the types of hypospadias.

9. Give the forms of cryptorchidism and testicular ectopia.

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

Clinical task 1

A 50-year-old patient complained of recurring dull pains in the left flank of the abdomen and lumbar region. In recent months, in the left hypochondrium, he independently began to feel an elastic, rounded, easily displaced formation. At the same time, there was no increase in body temperature, urination disorders, changes in the color of urine and other symptoms. Has addressed to the family doctor. The laboratory tests he ordered were normal, after which a multispiral CT scan of the abdomen was performed (Fig. 5.39).

What would you diagnose? Are other research methods needed to confirm it? What method of treatment to choose?

Clinical task 2

A 25-year-old patient complained of dull pain in the lumbar region on the left, frequent painful urination. Such phenomena are noted for several months. Palpation of the kidneys are not determined. Blood and urine tests were not changed. Ultrasound revealed a rounded 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 with a club-shaped extension

hypoechoic formation in the region of 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 strategy should be chosen?

Clinical task 3

The parents of a boy at the age of 9 months turned to the urologist with a complaint about the absence of the left testicle in the child's scrotum. According to them, the boy was born prematurely and the testicle was absent from the moment of birth. An objective examination revealed that the external opening of the urethra is located typically, the folding of the scrotum is preserved. The right testicle is determined 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 one of the most numerous, since they include malformations of many organs. They can only be diagnosed with medical equipment, such as x-rays or ultrasound.

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

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

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

Varieties of pathologies

Violation in the development of the urinary tract

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

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

Pathology of the bladder

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

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

Pathologies of the urethra in women, men and children

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

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

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

Anomalies of the genitourinary system are congenital malformations of the fetus (CMD). Some problems are inherited from parents. Pathology may lie in the mutation of the gene. The occurrence is affected by deviations from external factors. Congenital malformations begin to appear in the fetus in the first three months of pregnancy. During this period, the formation of internal organs takes place, and any external impact of adverse factors directly on the fetal body can provoke a delay or, conversely, excessively rapid development. Unfavorable factors include viral infections that a pregnant woman suffers from, the influence of harmful chemical factors (dyes, resins), the use of alcoholic beverages during pregnancy, as well as antibiotics, hormones and other medicines, and the effect 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 childbirth. The founder of the doctrine of prenatal pathology is him. scientist Schwalbe, whose works date back to the beginning of the twentieth century. The period from day 196 in development and the first 7 days after childbirth 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 up to day 196), during childbirth and postpartum, neonatal. The entire development of the fetus can be divided into 2 periods: progenesis (the time of maturation of gametes, germ cells) and cymatogenesis (the period of fetal development from the moment of fertilization to childbirth) Cymatogenesis is divided into:

Blastogenesis - up to 15 days

Embryogenesis - up to 75 days

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

During the period of progenesis, the maturation of germ cells - eggs and spermatozoa - their damage can occur, 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, enzymopathies. Congenital malformations are observed more often in children whose parents are older than 40-45 years.

Cymatopathies are pathological processes that occur during cymatogenesis. The main pathology of this period are malformations, accounting for 20% or more.

Congenital malformations are persistent changes in an organ or the whole 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.

Mutations are considered to be endogenous causes (it is believed that 40%)

Mutations can be: gene - a persistent change in the molecular

gene structure

chromosomal - a change in the structure of chromosomes

genomic - a 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. in-va (cytostatics)

viruses (rubella)

overmaturation of germ cells (lengthening the time from the moment of full maturation of germ cells to the formation of a zygote causes a complex of pathological changes in them.)

Exogenous causes include:

Radiation under its action during critical periods of embryo development. Irradiation in the first 3 months of pregnancy leads to congenital malformations of the predominantly nervous system.

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

Chemical factors, medicinal substances (anticonvulsants thalidomide);

Alcohol - leads to alcoholic embryopathy, later there is a lag in weight, height, mental development, there is microcephaly, strabismus, a thin upper lip, heart defects in the form of a septal defect are frequent

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 Langerganas. There are signs of immaturity, cardiohepatosplenomegaly, microangiopathy, pneumonia;

Viruses (cytomegaly)

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

CONGENITAL DEFECTS.

Congenital malformations of the central nervous system.

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

Anencephaly - agenesis of the brain, there are no anterior, middle and posterior sections. The medulla oblongata and spinal cord are preserved. In place of the brain conn. tissue rich in blood vessels.

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

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

Microgyria - 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 eye socket

Hernia of the brain and spinal cord - meningocele - the presence of only membranes in the hernial sac, meningoencephale - and the brain, myelocele - hernia of the spinal cord.

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

Congenital malformations of the digestive system.

They are found in 3-4% of autopsies of the dead 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 region of the rectum and anus. There may be tracheoesophageal fistulas in the esophagus leading to severe aspiration pneumonia. Atresias can be single and multiple. In the area of ​​atresia, the intestine looks like a dense connective tissue cord, which, under the influence of peristalsis, can stretch and break.

Doubling of individual sections of the intestine - more often only concerns the mucosal membrane, the muscular membrane is common. The duplicated area may be in the form of a cyst, diverticulum, or tube. The defect is complicated by bleeding, inflammation, necrosis with perforation.

B-n Hirschsprung - segmental agangliosis megaoclonus - the absence of neurons in the intermuscular plexus of the lower part of the sigmoid and rectum. Due to the preservation of the submucosal (Meissner) plexus, the aganglionic section of the intestine is spastically contracted, above it the intestine is stretched with meconium or feces, followed by compensatory hypertrophy of the muscular membrane. Patients suffer from constipation, coprostasis, obstruction develops.

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 the loss of chlorides.

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

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

Cysts and fistulas in the umbilical ring - due to persistence of the vitelline duct.

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

Congenital malformations 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 area of ​​triads, leading to the development of congenital biliary cirrhosis of the liver, 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 - congenital absence of one or both kidneys, hypoplasia - congenital decrease in the mass and volume of the kidneys, can be unilateral and bilateral, kidney dysplasia - hypoplasia with the simultaneous presence of embryonic tissues in the kidneys, large cystic kidneys - an increase in the kidneys with the formation of numerous small cysts, fusion of the kidneys (horseshoe kidney), and dystopia. - are not clinically manifested.

Congenital malformations of the urinary tract:

Doubling of the pelvis and ureters

Agenesia, atresia, ureteral stenosis

Megaloureter - a sharp expansion 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 in the lower wall, epispadias - the upper wall of the urethra in boys.

All malformations lead to a violation of the outflow of urine 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 are multiple and single, later leading 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 malformations of the lungs, if they are compatible with life, are easily complicated by a secondary infection with the development of xp. bronchitis and pneumonia with the development of cor pulmonale.

Congenital malformations of the musculoskeletal system:

System flaws:

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

Osteogenesis imperfecta - congenital bone fragility

Congenital marble b-n - pronounced osteosclerosis with a simultaneous violation of the development of hematopoietic tissue

Congenital dislocation and dysplasia of the hip joint

Congenital amputation or amelia of limbs

Phocomelia - underdevelopment of the proximal limbs

Polydactyly - an increase in the number of fingers

Syndactyly - fusion of fingers.