Causes of development and treatment options for developmental anomalies of the genitourinary system. Anomalies in the development of the urinary system. Treatment of congenital malformations of the urinary system.

The clinical significance of kidney development anomalies is determined by the fact that in 43-80% of cases they create conditions for the addition of secondary diseases that are more severe than in kidneys of normal structure. With anomalies in the development of the kidneys, chronic pyelonephritis develops in 72-81% of cases, and it has a persistent course, with it blood pressure often rises and renal failure quickly progresses [Trapeznikova M. F., Bukharkin B. V., 1979] The cause of the frequent occurrence of pyelonephritis with Most authors believe that renal anomalies are either congenital inferiority of the kidneys themselves, or a violation of uro- and hemodynamics, a combination of various types of renal anomalies with malformations of the lower urinary tract, in particular with vesicoureteral reflux.

Often, kidney abnormalities first appear during pregnancy, and the main disease for which patients are examined is pyelonephritis. We, together with M. S. Bazhirova, identified anomalies in the development of the urinary tract in 115 women. Most of them were admitted to the hospital for pyelonephritis that existed before pregnancy or occurred during it. Anomalies in the development of the kidneys were detected in 85 pregnant women, anomalies in the development of the ureters and bladder - in 20, anomalies in the development of the renal vessels - in 10. A double kidney was diagnosed in 30, a congenital single kidney - in 12, hypoplasia of the kidney - in 4, hydronephrosis - in 17, polycystic kidney disease - in 9, solitary kidney cyst in 4, spongy kidneys - in 2, fused kidneys - in 4, lumbar dystonia of the kidney - in 2, kidney rotation - in 1 patient.

Among the anomalies of the ureters and bladder, stricture of the ureter was most often observed (in 12), kinking of the ureter was in 2, duplication of the ureter in 1, megaloureter in 2, vesicoureteral reflux in 1 and malformations of the bladder (aplasia, atony, underdevelopment) - in 3 pregnant women. Anomalies in the development of the renal vessels in all 10 women consisted of unilateral or bilateral stenosis of the renal arteries. During pregnancy, 57 of 115 women experienced exacerbation of pyelonephritis, 12 had nephrogenic hypertension, and 9 patients had renal failure. An employee of the All-Russian Research Center for Healthcare and Healthcare, D. K. Kurbanov, who performed an ultrasound examination of pregnant women suffering from pyelonephritis, diagnosed 20 out of 161 women (12.4%) with anomalies in the development of the kidneys and renal vessels (anomalies of the lower urinary tract are not detected by this method).

Anomalies of kidney development are divided into 4 groups: anomalies of number, position, relationship and structure. According to A. Ya. Abrahamyan et al. (1980), the most common types of developmental anomalies are duplication of the kidneys, pelvis and ureters (23%), polycystic kidney disease (16.5%), lumbar dystopia (14.2%), horseshoe kidney (13.7%). Other types of anomalies are less common and range from 0.2 to 8.1% each. A combination of renal developmental anomalies with malformations of the musculoskeletal, cardiovascular and digestive systems was noted in 3.7% of patients, a combination with developmental anomalies of the genital organs - in 0.7%.

Abnormalities of quantity include renal aplasia, renal hypoplasia, duplication of kidneys, and additional third kidney.

About aplasia kidneys has already been discussed in the section “Pregnancy in women with one kidney.” It should be added that renal aplasia is usually accompanied by hypertrophy of the contralateral organ. When its function is normal, renal failure does not develop. A single kidney is more susceptible to various diseases than each of the normal kidneys. Infection of this single kidney is manifested by pain in the lumbar region, fever, pyuria, hematuria, and anuria. Renal failure develops in 25-63% of patients with a single congenital kidney. Of the 12 women with aplasia we observed, 1 had an abortion due to renal failure, 5 had a cesarean section for obstetric indications, 6 women had timely births.

Hypoplasia- congenital reduction in kidney size (Fig. 7). The kidney may be vestigial and dwarf.

Vestigial kidney- This is a sclerotic, small structurally and functionally underdeveloped organ.

Dwarf bud- normal kidney reduced in size.

The dysplastic form of the dwarf kidney is characterized by excessive development of fibrous tissue to the detriment of parenchymal tissue; such an anomaly is often accompanied by nephrogenic hypertension, often malignant. In 2 of 6 pregnant women with kidney hypoplasia observed by us, blood pressure was increased, 2 patients underwent nephrectomy of a non-functioning hypoplastic kidney; all 6 pregnant women had pyelonephritis, which worsened in 4 of them. 1 patient developed chronic renal failure. There were 5 timely births and 1 birth occurred prematurely, a stillborn child was born.

Kidney duplication- a common anomaly. An enlarged kidney could have doubled pelvis, vessels or ureters; there could be simultaneous doubling of all these elements - complete doubling of the kidney (Fig. 8, 9, 10). However, each of the halves of the kidney is, as it were, an independent organ, and the pathological process affects usually one of them.

These may be hydronephrosis, pyelonephritis, urolithiasis, tuberculosis. The cause of these double kidney diseases is most often vesicoureteral reflux. With ectopia of the accessory ureter into the vagina or cervix, involuntary urination is observed.

It is generally accepted that a double kidney is the least serious variant of developmental anomalies from the point of view of the course of pregnancy and childbirth. Our research shows that this is not the case. A double kidney during pregnancy is prone to the development of pyelonephritis (in 14 out of 30 women), and a persistent course of the disease is observed. Quite often (in 3 out of 30), a double kidney is accompanied by nephrogenic hypertension, which adversely affects pregnancy and fetal development. Many women with a double kidney develop late toxicosis of pregnancy (17 out of 30), which is often severe and difficult to treat. Therefore, pregnant women with a double kidney need clinical observation from a antenatal clinic physician and a urologist. Pregnancy is contraindicated in cases where the disease is accompanied by chronic renal failure.

Positional anomaly or dystopia can be pelvic, iliac, lumbar, thoracic and cross, unilateral or bilateral. Pelvic dystopia is the location of the kidney deep in the pelvis between the uterus and rectum. A bimanual examination in the vicinity of the posterior vaginal fornix reveals a dense, smooth formation. With dystopia of the kidney in the iliac fossa, pain may occur, often during menstruation. Upon palpation, the kidney may be mistaken for an ovarian cyst. The lumbar dystopic kidney can be palpated in the hypochondrium. Thoracic dystopia is a very rare anomaly and is an incidental finding during fluoroscopy. With cross dystopia, the kidney is displaced to the opposite side.

Dystopiakidney accounts for 1/5 of all anomalies of kidney development, with 2/3 of cases accounting for lumbar dystopia, which was also diagnosed in the patients we observed. Dystopic kidneys during pregnancy can manifest themselves as abdominal pain if they are affected by hydronephrosis, pyelonephritis, nephrolithiasis, and the lower the dystopia, the more often secondary kidney disease. In patients with kidney dystopia, intestinal activity may be impaired.

Pregnancy and childbirth are not special in all types of kidney dystopia, except pelvic. The location of the kidney in the pelvis can become an obstacle to natural delivery; in this case, a planned cesarean section is indicated. In the 4 patients with lumbar dystopia we observed, pregnancy was accompanied by the threat of termination in 3 women, the birth proceeded safely. With anomalies in the position of the kidneys, pregnancy is not contraindicated

Abnormalities of the relationship of the kidneys- this is the fusion of the kidneys with each other.

Various types of bud fusion give this conglomerate the shape of a biscuit-shaped, S-shaped, L-shaped and horseshoe-shaped kidney. Such kidneys are very susceptible to inflammation, hydronephrosis, and can be a source of renal hypertension. The causes of arterial hypertension in this case are chronic pyelonephritis, hydronephrosis, abnormal blood supply, and high intrarenal hypertension.

If there are abnormalities in the relationship, pregnancy is permissible if there is no secondary kidney damage. In 1 of 4 patients with an L-shaped kidney we observed, there were indications for termination of pregnancy, since often recurrent pyelonephritis occurred with chronic renal failure.

Abnormalities of the kidney structure include polycystic and multicystic kidneys, dermoid and solitary cysts, spongy kidney, pelvic diverticulum and peripelvic renal cyst.

Polycystic kidney disease- severe bilateral developmental anomaly.

The disease has a dominant type of inheritance. The kidney is an organ in which the parenchyma is almost completely replaced by multiple cysts of various sizes (Fig. 11). About 70% of babies with polycystic kidney disease are stillborn. With a small number of affected nephrons, children are viable, but renal failure develops when infection occurs and pyelonephritis develops. Polycystic kidney disease can be combined with polycystic disease of the lungs, ovaries, liver, and pancreas.

There are 3 stages of the clinical course of polycystic kidney disease:

  • Stage I- compensated, manifested by dull pain in the kidney area, general malaise, minor functional disorders;
  • Stage II- subcompensated, which is characterized by lower back pain, dry mouth, thirst, headache, nausea associated with renal failure and arterial hypertension;
  • Stage III- decompensated, in which signs of chronic renal failure are expressed, the functional state of the kidneys is sharply depressed. This is confirmed by a decrease in the filtration and concentration capacity of the kidneys, retention of nitrogenous waste in the body, and anemia.

The kidneys are usually palpated as large, tuberous formations, always bilateral, unlike renal tumors. Patients begin to complain of lower back pain early. Hematuria appears in half of the patients.

The question of the advisability of continuing pregnancy with polycystic kidney disease is still debated. There is an opinion that pregnancy is contraindicated for this group of patients, since it worsens chronic pyelonephritis. D.V. Kahn (1978) argues against this view, arguing that in the absence of renal failure, pregnancy is acceptable. He pays attention to the age of patients, believing that it is better for them to give birth before the age of 25, since the symptoms of polycystic disease mainly appear at the end of the third or beginning of the fourth decade of life. All 6 observed D.V. Kan, patients with polycystic kidney disease gave birth safely the first time, but during the second pregnancy they developed arterial hypertension and eclampsia. N A Lopatkin and A L. Shabad (1985) consider pregnancy and childbirth extremely undesirable for women suffering from polycystic disease.

Due to the high probability of transmitting this defect to offspring, pregnancy should not be recommended for women suffering from polycystic kidney disease, since such patients develop early chronic renal failure, which is aggravated by the state of pregnancy and chronic nyelonephritis, which often complicates the course of polycystic disease. Half of the 9 patients we observed had an exacerbation of pyelonephritis, and half had chronic renal failure. In addition, polycystic kidney disease leads to the development of symptomatic arterial hypertension (in 5 out of 9 women), which also worsens the course of pregnancy and fetal development. 5 out of 9 pregnant women developed nephropathy, 1 had preeclampsia. Given these data and the hereditary nature of the disease, polycystic kidney disease should be considered a contraindication for pregnancy.

Solitary renal cyst- single cystic formation. The cyst can be congenital or acquired.

This anomaly is not hereditary and is unilateral. An increase in the size of the cyst leads to atrophy of the kidney parenchyma, impaired hemodynamics in the kidney and the development of arterial hypertension. Patients complain of dull pain in the lower back. The enlarged kidney is palpable. Pyuria or hematuria is observed. In the absence of renal hypertension, pregnancy is not contraindicated. All 4 patients we observed with this form of kidney anomaly gave birth safely

Sponge bud- an anomaly in which numerous cysts form in the renal pyramids.

The disease is bilateral, manifested by hematuria, pyuria, and pain in the lumbar region. Kidney failure usually does not develop. Pregnancy with this kidney anomaly is not contraindicated. We observed 2 patients whose pregnancy and childbirth proceeded well, despite the exacerbation of pyelonephritis during pregnancy.

Multicystic kidney, dermoid cyst of the kidney, diverticulum of the pelvis and peripelvic cyst- very rare developmental anomalies.

Congenital hydronephrosis in the 17 pregnant women we observed was caused by stricture of the ureteropelvic segment (in 10), kinking of the ureter (in 3), reflux in (1) and anomalies of the renal vessels. A special section of this chapter is devoted to hydronephrosis.

Abnormalities of the ureters and bladder are as varied as abnormalities of the kidneys. Aplasia of the pelvis and ureter is observed as a component of renal aplasia, doubling of the pelvis and ureter, sometimes combined with complete doubling of the kidney.

Ureterocele- intravesical hernia-like protrusion of the intramural part of the ureter.

Ureterocele can cause dilatation of the upper urinary tract, pyelonephritis, and urolithiasis.

Ectopic ureteral orifice- abnormal location of the ureteric orifice in the posterior part of the urethra, vaginal vault, in the area of ​​the external genitalia or rectum.

This anomaly is characterized by constant incontinence of urine from one ureter and entry into the bladder from the second ureter with periodic natural emptying of the bladder. Neuromuscular dysplasia of the ureters (megaloureter) is a combination of congenital narrowing of the ureteric orifice with neuromuscular dysplasia of lower cystosis. The overlying parts of the ureter expand and lengthen, forming the megaloureter. The kinetics of the ureter is sharply disrupted, contractility is slow or absent.

All variants of developmental anomalies of the ureters contribute to impaired urodynamics, the development of pyelonephritis, hydronephrosis, and renal failure. Among the 17 patients we observed with ureteral anomalies, 12 had ureteral stricture, which resulted in hydronephrosis in 6 pregnant women with renal failure in 1 of them. Vesicoureteral reflux was detected in 2 patients: in one it was combined with hydronephrosis, in the other with narrowing and kinking of the ureter. 2 patients had megaloureter, 1 had duplication of the ureter. All women suffered from chronic pyelonephritis, and 12 out of 16 had an exacerbation of pyelonephritis during pregnancy, and 1 had renal failure. All women were able to achieve pregnancy except the patient with renal failure. 2 patients underwent cesarean section for obstetric indications.

Anomalies in the development of the bladder.

Meet bladder duplication, bladder diverticulum- saccular protrusion of the wall, bladder exstrophy- absence of the anterior wall of the bladder, etc. We observed 3 pregnant women with aplasia of the bladder, its atony and underdevelopment.

All of the women we observed with anomalies in the development of the bladder and some of the patients with anomalies in the development of the ureter underwent corrective urological operations, which improved their condition and allowed them to endure pregnancy and childbirth. It should be noted that their condition worsened during pregnancy and the difficulty of resolving the issue of the method and timing of delivery in case of bladder anomalies. One patient with aplasia of the bladder and transplantation of the ureters into the rectum had to undergo a minor cesarean section at 30 weeks of pregnancy; another, who underwent bladder surgery, had a cesarean section during a full-term pregnancy. Observations of the course of labor in women with developmental anomalies of the ureters and bladder are few, and tactics for managing pregnancy and childbirth have not been developed. In most cases, it is necessary to decide individually for each patient the question of the possibility of continuing pregnancy, the timing and method of delivery.

Diagnosis of anomalies in kidney development is based on data from chromocystoscopy, ultrasound, excretory urography, pneumo-retroperitoneum, radioisotope scanning of the kidneys. During pregnancy, only the first two methods are permissible. With anomalies in the development of the ureters, the echocardiographic method is not informative, so it is almost impossible to diagnose them during pregnancy; the diagnosis is made before pregnancy or retrospectively during examination after childbirth.

In addition to kidney and urinary tract abnormalities, there are abnormalities in the development of renal vessels, which affect kidney function and the woman’s health, and therefore can affect the gestational process. Accessory, double or multiple renal arteries, arteries with an atypical direction, as well as additional or atypically directed veins, compress the ureters, disrupting urodynamics and contributing to the formation of hydronephrosis, urolithiasis, pyelonephritis and nephrogenic hypertension. A. A. Spiridonov (1971) believes that in the development of arterial hypertension with multiple renal arteries, 3 factors that cause activation of the renin-angiotensin system can play a role:

  1. damping of the pulse wave as it passes through several small arteries;
  2. discrepancy between blood flow and venous outflow;
  3. urodynamic disorders.

Anomalies in the development of renal vessels can be diagnosed by angiography and aortography, which are unacceptable during pregnancy. Therefore, the diagnosis is usually made before pregnancy. In pregnant women, only ultrasound examination in some cases helps in diagnosis. As a rule, the diagnosis must be confirmed after childbirth or termination of pregnancy using x-ray methods.

We observed 10 women with renal artery stenosis due to fibromuscular hyperplasia. The first 4 patients were described in the book by M. M. Shekhtman, I. Z. Zakirov, G. A. Glezer “Arterial hypertension in pregnant women” (1982; Renal artery stenosis causes a consistently high (200-250/120-140 mm Hg Art., or 26.7-33.3/16.0-18.7 kPa) blood pressure, not corrected by drug therapy. Pregnancy usually ends in intrauterine fetal death or spontaneous abortion. Therefore, the only correct solution is termination of pregnancy and surgical treatment renovascular hypertension. Resection or plastic surgery of the renal artery (sometimes bougienage) leads to normalization of blood pressure and a successful pregnancy and childbirth. All the women we observed after the operation gave birth to live children, one of them three times.

The commonality of embryogenesis of the urinary and genital organs creates the prerequisites for the development of anomalies in both systems.

N. A. Lopatkin and A. L. Shabad (1985) believe that the combination of developmental anomalies in both systems reaches 25-40%, and point to the following patterns: the existence of an internal dependence of the organogenesis of the urinary and genital organs in women; the side of the kidney development anomalies coincides with the side of the genital anomalies. This combination of anomalies of the two systems is explained by unilateral or bilateral disruption of the development of the mesonephric and paramesonephric ducts in ontogenesis. E. S. Tumanova (1960) found kidney anomalies in every 5th woman with anomalies of genital development.

Among the women we observed with anomalies in the development of the urinary organs, 6 (8%) had malformations of the genital organs. 2 women with aplasia of the kidney had a saddle-shaped uterus, 1 had a septum in the vagina, 1 woman with megaloureter had a bicornuate uterus, a woman with underdevelopment of the bladder had a saddle-shaped uterus, and a patient with aplasia of the bladder had a septum in the vagina.

Combined pathology poses new tasks for the obstetrician-gynecologist observing a pregnant woman, makes changes in the tactics of pregnancy and childbirth, and affects the prognosis of pregnancy outcome, so clarifying the situation is of great importance. Since it is impossible in most cases during pregnancy, it should be performed before pregnancy, during the observation of women in the antenatal clinic for some other reason.

Treatment of developmental anomalies of the urinary tract is surgical. It is not carried out during pregnancy. In pregnant women, treatment is carried out for complications such as pyelonephritis, arterial hypertension and renal failure.

Frequency: found in approximately 1% of the population, diagnosed prenatally in approximately 0.2% of children.

Attention: 30% of prenatal diagnoses are not confirmed, so monitoring after birth is necessary.

Forms:

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

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

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

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

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

First aid with bladder exstrophy:

  • Immediately place on a sterile diaper.
  • Moisturizing the eventrated parts.
  • Constant assessment of the eventrated bladder: color, hemorrhage, swelling.
  • Wrapping up the eventured tea! in a sterile plastic bag or moisturize with sterile Vaseline.
  • Don't forget to weigh your baby.
  • Emergency (up to 24-48 hours) surgery.
  • Examination of the proximal urinary tract.

Hypospadias

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

Tactics depend on the location of the external opening:

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

Combined with vices:

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

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

Treatment:

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

Hydrocele (dropsy)

Hydrocele (dropsy): Persistence of a patent processus vaginalis can lead to the accumulation of peritoneal fluid within the processus vaginalis.

Definition: a fluid-filled cyst in the area of ​​the testicular membranes or spermatic cord.

Shapes:

  • Hydrocele of the cord.
  • Hydrocele of the testicle: the testicle is most often not palpable.
  • Inguinoscrotal hydrocele: the upper pole of the cyst reaches the abdominal cavity. A dense formation is palpated medial to the inguinal ligament in the small pelvis. When pressing on the scrotum, its volume increases.
  • With communicating dropsy (the size changes), there is a connection with the abdominal cavity. The clinic and treatment correspond to an inguinal hernia.
  • Nuco canal cysts (in girls): localized accumulations of fluid in the area of ​​the round ligament outside the inguinal canal.

Clinic:

  • Various sized asymptomatic unilateral or bilateral tumor-like formations in the testicular area.
  • Often found in newborns. Spontaneously regresses during the first 3-4 months of life.
  • They can appear spontaneously without an obvious reason outside the neonatal period, and they can also regress.
  • After the first year of life, dropsy occurs in less than 1% of boys.

Diagnostics:

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

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

Important: Testicular torsion can also be congenital; this is an indication for immediate surgery.

Treatment:

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

Inguinal hernia

Formed as a result of persistence of the vaginal process of the peritoneum. A sufficiently large size of the hole allows intestinal loops to penetrate into the appendix.

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

Clinic:

  • Most often, there are asymptomatic, soft, reducible tumors in the groin medial to the inguinal ligament (inguinal hernia), which may reach the scrotum (scrotal hernia).
  • When strangulated, the hernia becomes painful and irreducible.

Diagnostics:

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

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

Complications:

  • Potentially life-threatening condition.
  • Intestinal strangulation or peritonitis.
  • Loss of a testicle/ovary or section of intestine.

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

  • Clinic: sudden onset of illness, severe pain, anxiety, symptoms of peritoneal irritation.
  • Findings: elastic-elastic, tense, sedentary inguinal or inguinoscrotal tumor.

Treatment and indications for surgery:

  • Reduction of an inguinal hernia often occurs independently.
  • Active reduction (under sedation) is possible no later than 6-8 hours from the onset in the absence of symptoms of shock
  • If reduction is unsuccessful, urgent surgery is indicated.
  • Elective surgery after successful reduction.
  • In all other cases, early hernia repair is optimal so that the risk of anesthesia does not increase.

Umbilical hernia

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

CONTENTS:

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

Kidney anatomy

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

Embryogenesis:

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

Main features of embryogenesis of the kidneys and ureters

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

Merged Müllerian ducts (9 weeks)

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

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

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

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

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

Developmental anomalies

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

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

13. Absence of gonads –
AGENESIS,
incomplete

HYPOPLASIA;
14. Impaired descent of the testicles
scrotum

ONEor
BILATERAL CYPTORCHISM;
15.
Lowering
testicles
along
guides
fibers
the guide ligament causes
ECTOPY OF THE TESTIS;
16.
AZOOSPERMIA
(infertility)
arises
V
result
nonunion
networks
testicles
With
efferent tubules;
17.
MICROPENIS
at
pseudohermaphroditism
18. No front wall
Bladder - EXTROPHY
BLADDER.

Kidney anatomy

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

Kidney anatomy

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

Kidney anatomy

The kidneys consist
from the brain and
cortical
substances

Kidney anatomy

Renal vascular abnormalities

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

84,6%

Additional
artery

Accessory and multiple renal veins:

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

Double renal artery

Multiple arteries

O.11%

Renal artery aneurysm
O.11%

Fibromuscular stenosis:

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

Fibromuscular stenosis
arteries

Arteriovenous fistula and renal aplasia

Kidney abnormalities

Occurs in 3-5.5% of patients
Make up 10% of all anomalies
Ministry of Railways
In recent years they have not
downward trend

Kidney abnormalities are divided into 5 groups:

quantity anomalies
magnitude anomalies
location anomalies
relationship anomalies
structural anomalies

Classification (N.A. Lopatkina)

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

Aplasia:

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

0,083% 1:1200

Kidney aplasia
0,083%
1:1200

Kidney number abnormalities

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

Kidney duplication
10,4%
Accessory kidney

Kidney number abnormalities

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

Accessory (third) kidney:

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

Accessory kidney

Complete bud duplication

Duplication and hydronephrosis of the lower half of the kidney

Incomplete bud duplication

Magnitude anomalies

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

Renal hypoplasia

Classification

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

2,8%

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

Thoracic dystopia of the kidney

Anomalies in the location of the kidneys (dystopia)

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

Lumbar kidney dystopia

Edge

Anomalies in the location of the kidneys (dystopia)

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

Ilial dystopia

Anomalies in the location of the kidneys (dystopia)

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

Pelvic dystopia

Anomalies in the location of the kidneys (dystopia)

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

Pelvic dystopia of the cup (medially).

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

Cross dystopia.

Cross Dystopia

ANOMALIES OF KIDNEY RELATIONSHIP:

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

Relationship anomalies

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

Biscuit bud

S-shaped and L-shaped kidneys

Relationship anomalies

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

J-bud

0,25%

Horseshoe kidney:

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

Horseshoe kidney

Classification

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

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

Anomalies of structure

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

Kidney dysplasia

Anomalies of structure

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

Multicystic kidney:

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

Multicystic kidney disease

Anomalies of structure

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

Solitary renal cyst:

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

Solitary renal cysts

Anomalies of structure

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

Spongy bud:

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

Spongy kidney (overview photo)

Polycystic kidney disease:

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

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

Polycystic kidney disease

0,17%

Anatomy of the ureters

Histological structure of the ureter.

URETER ANOMALIES:

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

Duplication of the pelvis and ureter:

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

Tripling

13,4%

Full doubling
13,4%

Incomplete doubling

Retrocaval ureter:

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

0,21%

Retrocaval ureter:
0,21%

Retrocaval ureter:

Corkscrew ureter:

Ureterocele:

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

Ureterocele:

Ureterocele:

Incomplete dysplasia
7:1000
Ureteral plication

Neuromuscular dysplasia

Ectopic ureteral orifice:

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

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

Abnormalities of the urethra:

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

Hypospadias

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

Abnormalities of the urethra

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

Hypospadias:

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

1:450-500

"hypospadias without hypospadias"

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

4 types of "hypospadias without hypospadias"

Diagnosis of hypospadias

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

Treatment

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

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

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

Abnormalities of the urethra

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

Epispadias

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

Epispadias:

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

Epispadias

Epispadias of the glans penis

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

Stem form of epispadias

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

Total (complete) epispadias

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

Treatment

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

Abnormalities of the urethra

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

Congenital urethral valves

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

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

Congenital obliteration of the urethra

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

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

Bladder exstrophy:

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

Pathology of the urachus

Testicular abnormalities (5-7%)

Anorchism
Monorchism
Polyorchidism
Hypoplasia
Synorchism
Cryptorchidism
Ectopic testicle

Cryptorchidism:

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

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

Anorchism

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

Polyorchidism

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

Testicular hypoplasia

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

Penile abnormalities

Congenital phimosis
Hidden penis
Ectopia of the penis
Double penis

Congenital phimosis:

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

Phimosis

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

Paraphimosis

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

Hidden penis

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

Short frenulum of the penis

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

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

Congenital malformations (anomalies) of the urinary tract

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

Congenital malformations (anomalies) of the bladder

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

Congenital anomalies of the urethra

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

Symptoms of kidney abnormalities:

    urinary disturbance;

    growth disorder;

    pain in the side and abdomen;

    frequent urinary tract infections.

Causes of urinary tract abnormalities

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

Treatment of anomalies of the genitourinary system

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

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

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

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

Course of the disease

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

Modern therapy for anomalies of the urinary system is possible only if the pathology is diagnosed early, so every child must undergo all mandatory preventive examinations.

Introduction


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

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

The greatest number of complications occurs when there is a mechanical obstruction to the outflow of urine; urine stagnates or returns (returns) to the upper parts of the urinary system. The tissue in the area of ​​the mechanical obstruction swells and as a result the tissue is damaged. The most serious damage is to the kidney tissue, leading to impaired renal function.

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

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

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

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

The urinary tract is formed in the early stages of fetal formation (the first 6 weeks after fertilization); the resulting defects can arise from a number of factors affecting the fetus, although the exact mechanism of their occurrence remains unclear. It has been established that the percentage of defects is higher in children born to mothers who abused drugs or alcohol.

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


Quality anomalies

genitourinary agenesis kidney dystopia

Anomalies of the urinary system are disorders in the development of organs of the genitourinary system that do not allow the body to function normally.

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

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

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

Ø Anomalies in the position of the kidneys - momolateral dystopia (the drooping kidney is on its side); heterolateral crossed dystopia (movement of the kidney to the opposite side);

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

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

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

Many of these anomalies cause the development of kidney stones, inflammation (pyelonephritis), and arterial hypertension.

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

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

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

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

Cloaca exstrophy

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

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

Bladder exstrophy

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

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

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

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

Progress in surgical technology allows, in most cases, to reliably correct this type of developmental defect.

Epispadias

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

Pyeelectasis

Pyeelectasia is an enlargement of the renal pelvis. The explanation of the origin of the term “pyelectasia” is very simple. Like most complex medical names, it is derived from Greek roots: pyelos - “trough”, “tub”, and ektasis - “stretch”, “stretch”. The stretching is understandable, but what is called the “tub” needs to be dealt with.

Pyeelectasia is one of the most common structural anomalies that are detected during ultrasound examination of the urinary system. An ultrasound diagnostic doctor usually does not have specialized information in the field of nephrology, he works in his area of ​​expertise, so his report contains the phrase: “Consultation with a nephrologist is recommended,” and you get an appointment with a nephrologist. Most often, pyeloectasis is found during ultrasound during pregnancy, before the baby is born, or in the first year of life. Therefore, it would not be a big mistake to attribute the expansion of the pelvis to congenital structural features.

But the expansion of the pelvis may appear later. For example, at 7 years of age, during a period of intensive child growth, when the location of organs relative to each other changes, the ureter may be compressed by an abnormally located or accessory vessel. In adults, dilation of the pelvis may be a consequence of blocking the lumen of the ureter with a stone.

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

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

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

· with the presence of an obstruction in the passage of urine into the bladder from the ureter or in its excretion through the urethra;

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

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

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

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

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

· with general weakness of the muscular system in case of prematurity of the baby (muscle cells are part of the ureters and pelvis);

· with neurological problems.

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

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

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

You should be wary:

· pelvis size 7 mm or more;

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

· changes in its size throughout the year.

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

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

Pyeelectasia is primarily associated with increased pressure in the pelvis, which cannot but affect the kidney tissue adjacent to it.

Over time, part of the kidney tissue is damaged under constant pressure, which, in turn, leads to disruption of its functions. In addition, due to the high pressure in the pelvis, the kidney requires additional effort to remove urine, which “distracts” it from its immediate work. How long can the kidneys work in such an enhanced mode?

For low-grade pyeloectasia (5-7 mm), control ultrasounds of the kidneys and bladder are performed once every 1-3 months. (frequency is determined by a nephrologist) in the first year of life, and in older children - once every 6 months.

When an infection occurs and (or) when the size of the pelvis increases, an X-ray urological examination is performed in a hospital setting. Usually this is excretory urography, cystography. These examinations allow us to determine the cause of pyelectasis. Of course, they are not absolutely harmless and are carried out strictly when indicated and according to the decision of the supervising doctor - a nephrologist or urologist.

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

In the absence of a sharp deterioration and visible disorders (according to ultrasound, urine tests, etc.), another tactic may be proposed: observation and conservative treatment. It usually includes physiotherapeutic procedures, taking (if necessary) herbal medications, and ultrasound monitoring.

Let's summarize:

· Pyeelectasia is not an independent disease, but can only serve as an indirect sign of a violation of the outflow of urine from the pelvis as a result of some structural anomaly, infection, reverse reflux of urine, etc.

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

· Since the kidney is a paired organ, pyeloectasia can be unilateral or bilateral.

· Pyeelectasia can be a consequence of a urinary tract infection and, conversely, can itself contribute to the development of inflammation.

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

· Pyeelectasis requires mandatory observation by a nephrologist and ultrasound monitoring.

· In most cases, pyeelectasis is transient, i.e., a temporary condition.

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

Increased kidney mobility and nephroptosis

Abnormalities in kidney development may not make themselves felt, but may manifest as persistent abdominal pain. It can be difficult even for a specialist to detect the source of the problem, since in many cases nothing can be detected during examination, and all tests are normal. But thanks to ultrasound, it has become possible to quickly and painlessly detect abnormalities in the functioning of the kidneys, although they are often discovered by chance.

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

Normally, the kidneys can perform a certain amount of movement during breathing; moreover, the absence of such kidney mobility can be a sign of a serious disease.

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

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

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

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

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

If an ultrasound scan shows the presence of nephroptosis, an x-ray urological examination is required to determine the degree of nephroptosis and possible abnormalities in the structure of the urinary tract.

Quantity anomalies


Renal agenesis

Mention of kidney agenesis is found in Aristotle: he wrote that an animal without a heart cannot exist in nature, but without a spleen or with one kidney they are found. The first attempt to describe aplasia in humans belongs to Andreas Vesalius in 1543. In 1928, N. N. Sokolov identified the frequency of aplasia in humans. As a result of his research, he analyzed 50,198 autopsies and found renal agenesis in 0.1% of cases. According to his data, the frequency of occurrence does not depend on the gender of the person. Modern scientists, based on a fairly large sample, give slightly different numbers. According to their data: the incidence of agenesis is 0.05%, and it occurs three times more often in males.

general information

Agenesis (aplasia) of the kidneys is a malformation of the organ during embryogenesis, as a result of which one or both kidneys are completely absent. There are also no rudimentary kidney structures. The ureter at the same time can be developed almost normally or be absent altogether. Agenesis is a common developmental defect and occurs not only in humans, but also in those animals that normally have two kidneys.

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

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

Types of renal agenesis:

Bilateral renal agenesis

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

Today, progress does not stand still, and it is technically possible to transplant a kidney into a newborn and perform hemodialysis. It is very important to timely differentiate bilateral renal agenesis from other defects of the urinary tract and kidneys.

Unilateral renal agenesis

Unilateral renal agenesis with preservation of the ureter

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

Unilateral renal agenesis with absence of the ureter

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

Treatment of renal agenesis

The method of kidney treatment depends on the degree of kidney dysfunction. The most commonly used surgical intervention is kidney transplantation. Along with surgical methods, there is also antibacterial therapy.

Kidney duplication

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

Causes of kidney duplication:

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

Symptoms of kidney duplication

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

Diagnosis of kidney duplication

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

Dystopia is the location of an organ, tissue or individual cells in an unusual place for them, caused by dysembryogenesis, trauma or surgery.

Heterolateral cross kidney dystopia (d. renis heterolateralis cruciata) is a congenital kidney dystopia with its location on the opposite side, next to the second kidney.

Homolateral kidney dystopia (d.renis homolateralis) is a congenital kidney dystopia with its location above or below normal.

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

Dystopia of the ileal kidney (d. renis iliaca) is a homolateral D. of the kidney with its location in the large pelvis.

Lumbar kidney dystopia (d.renis lumbalis) is a homolateral kidney dystopia with its location in the lumbar region below normal.

Pelvic kidney dystopia (d. renis pelvina) is a homolateral kidney with its location in the small pelvis.


Literature


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

Sapin M.R. - HUMAN ANATOMY AND PHYSIOLOGY with age-related characteristics of the child’s body. - publishing center "Academy" 2005

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

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

Guide to urology, edited by N. A. Lopatkin, Medicine Publishing House, Moscow, 1998. Download the essay "Human Urinary System" in full for free

A. G. Khripkova, V. S. Mironov, I. N. Shepilo Human Physiology, Prosveshchenie Publishing House, Moscow, 1971.

R. D. Sinelnikov Atlas of Human Anatomy, Medicine Publishing House, Moscow, 1973.

A. V. Kraev Human Anatomy, Medicine Publishing House, Moscow, 1978.


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