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Malformations of the Urinary System with Violation of Urodynamics

Congenital malformations of the urinary system occupy about 50% of the total urological pathology in
children, so it is very important that pediatricians had a clear understanding of this pathology and
current approaches to its treatment.
Hydronephrosis is an extension of the hollow structures of the kidney in organic obstruction at the
level between the bowl and the ureter, which violates the outflow of urine from the kidney. The latter
increases in size, and the parenchyma becomes thinner. This is one of the most common
malformations of the kidneys. The process is more often left-sided.Boys suffer more often.
Forms, depending on the level of obstacles:
- hydrocalicosis (segmental hydronephrosis) is the extension of one or more cups when the obstacle is
localized at the neck of the cup;
- simple hydronephrosis – enlargement of all the cups and when placing obstacles on a level of near
pelvic segment;
- ureterohydronephrosis – expansion of the cups and of the ureter above the obstacle.
Causes:
- the influence of the external structures of the renal pelvis-uretralny segment (aberrant vessels,
retrocaval localization of the ureter, embryonic spikes, periureteritis in patients with recurrent
pyelonephritis, etc.);
- changes of the wall segment (dysplasia of the wall of the ureter, hypertrophy of the muscle layer, a
disruption of the structure of nerve ganglia);
- anomalies of the pelvis-ureteral segment.
The degree of hydronephrosis (for Lopatkin M. A. (1969)):
I – the only extension of the renal pelvis (pyelectasis with minor impairment of renal function;
unmodified parenchyma);
II – the expansion (dilation) of the renal pelvis and calices (hydrocalicosis with thinning of the
parenchyma and dysfunction of the kidneys);
III – atrophy of the renal parenchyma, kidney becomes thin-walled bag with the urine.
According to the Ultrasound study (proposed by the Society for Fetal Urology - SFU):
· HN 0 – no or minimal visualization of the collecting system of the kidney (norm);
· HN I – dilation of the anterior-posterior size of the kidney pelvis less than 5-7 mm (norm variant);
· MN II – anterior-posterior size of the kidney pelvis is from 5 to 7-10 mm, some groups of calices
with unchanged structure are visualized;
· HN III – dilation of the pelvis and calices more than 10 mm with smooth outlines;
· HN IV – significant expansion of the collector system, thinning of the parenchyma;
· HN V – terminal stage of hydronephrosis, complete absence of parenchyma.
Clinical manifestation:
- pain (feeling of heaviness and discomfort or cramping nature abdominal and/or lumbar region;
sometimes with nausea and vomiting, possibly bloating); pain in the groin, thigh or encircling
types of pain); characterized by increased pain in the supine position on the opposite side of the
lesion;
- palpable tumor in the abdomen at the level of the hypochondrium;
- with the development of complications (pyelonephritis) fever, dysuria, pyuria.
Kidney’s function is maintained longer when extrarenal location of the renal pelvis, when intrarenal
location the loss of function occurs earlier. The worst case scenario – intrarenal location of renal pelvis
with obstruction in near renal pelvic segment (cups slightly enlarged, but very high urine pressure).
Diagnosis:
- urinalysis (leukocyturia with the development of complications);
- renal US imaging (calyectasis/pyelectasia, AP renal pelvis diameter > 10 mm, visible dilated renal
pelvis and distal ureter suggests that obstruction is at VUJ, bladder or urethra., dilated renal pelvis
without visible ureter suggests obstruction at the renal pelvis, PUJ obstruction).;
- excretory urography;
- scintigraphic diagnostics;
- angiography;
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- CT.
Treatment:
When hydrocalicosis surgical treatment is not carried out; child needs only urological dispensary
observation.
In other cases, treatment is surgical and consists in the elimination of causes of infringement of
outflow of urine, often in the resection of the narrowed UPJ; however, resection of the displastic parts
must be complete to the level of the normal diameter of the ureter – with pyeloureteroplasty.
Modern treatment option is endoscopic balloon dilatation nephrostomy contractions under pyeloscopic
control (when a short length of constriction in PUJ).
When III hydronephrosis degree occurs– nephrectomy (if the thickness of the parenchyma does not
exceed 3 mm) while maintaining the second kidney.

Vesico-ureteral reflux (VUR) is a congenital anomaly of the upper urinary tract, occurs when a
congenital or acquired deficiency of the ureteric orifices and may be unilateral and bilateral. During
urination the urine goes retrograde into the renal pelvis, which leads to increased pressure in hollow
system of the kidney. In 70-80% of cases of vesicoureteral reflux is complicated by pyelonephritis.
Can be primary (with no increase in intravesical pressure above 50 mm H2O) or secondary.
Reasons:
- dystopia of the ureteric orifices and their doubling;
- hypoplasia of the muscles of the bladder triangle and interureteric folds, short intramural segment
of the ureter;
- obstruction of the bladder;
- developmental abnormalities of the pelvis and spine.
Classification (according cystography, Tereshchenko A.V. (1987):
I – the reflux of contrast into the ureter only without its extension;
II – the reflux of contrast to the level of the renal pelvis, extension of the ureter to 1cm, but without the
extension of the caliculal-pelvical segment;
III - the expansion of the ureter more than 2 cm, its tortuosity, expansion of the caliculal-pelvical
segment, but without the smoothness of the cups;
IV - significant tortuosity of the ureter, a significant expansion of the caliculal-pelvical segment,
obliteration of the angles of the arches of the cups;
V - large curves of the ureter (megadolichoureter), a significant expansion of the caliculal-pelvical
segment, structure of the cups is not specified.
Clinic: for a long time may be asymptomatic. Sometimes, pain in the lumbar region and abdomen
during urination due to the pressure increase. Upon accession of infection – signs of pyelonephritis.
Moreover, the VUR can be both a cause and a consequence of pyelonephritis.
Diagnosis:
- simple and micturating cystography;
- cystoscopy;
- US imaging of kidneys and ureters;
- Excretory urography;
- CT.
Treatment:
1) Conservative therapy (antispasmodics and uroseptic, antibacterial therapy, sanation of
nasopharinx) with I-II stage of reflux.
2) Endoscopic correction with the introduction of implants or Teflon paste under the the ureteric
orifice (high relapse rate, the risk of embolism).
3) Antireflux surgery is a radical method of treatment. As a rule, apply the operation the elongation of
the intramural segment of the ureter.
Complications of urodynamic disorders:
- bacterial and inflammatory lesions of the urinary tract;
- renal failure in bilateral lesions;
- urolithiasis.
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N.B.! There are not specific clinical manifestations of malformations of the upper urinary tract. The
main "alarm" for pediatricians should be leukocyturia against the background of increasing body
temperature or without it and disorders of urination. In each case of detection of the child leukocyturia
it is advisable to take some urinalyses in the dynamics. If several tests have the pyuria, you should
immediately take the child to a urologist to do an ultrasound of the kidneys and bladder already and
depending on results and availability of evidence to perform excretory urography, cystoscopy, and
using other specific diagnostic techniques. The therapy with children with signs of infectious-
inflammatory process and/or violations of urination without prior examination and consultation of the
urologist is the wrong approach.

Independent Study
Megaureter is a developmental anomaly of the ureter, which has four basic forms, on which the treatment tactics
depends:
- obstructive – characterized by the presence of obstruction in the vesicoureteral segment and is subject exclusively to
surgical treatment;
- reflux form – also subject to surgical treatment;
- idiopathic – expansion of the ureter without strictures or reflux;
- bladder-dependent – a combination of obstructive or reflux megaureter with neurogenic hypo- or hyperreflective
dysfunction of the bladder, which is the most severe form and requires the combined use of surgical methods of
treatment and conservative therapy aimed at the normalization of bladder function.
Bladder malformations include abnormalities such as ureterocele, double ureters and diverticula (additional capacity,
which may be even more than the bladder itself).
Ureterocele is a cyst in the area of orifice of the ureter (which is usually doubled), into which the urine gets first, and
then through a small hole is released into the bladder.
Doubling of ureters in the absence of their expansion and vesicoureteral reflux can be considered as a variant of the
norm, which requires only monitoring of the child. But more often it happens that the second ureter departs from the
upper pole of the kidney and is much expanded – in such cases, the function of the upper segment suffers and it is
necessary to carry out heminefrectomy with the removal of the upper ureter.
In boys, a combination of obstructive or reflux bilateral megaureters with knee-shaped fixed curving of the ureters
and valves of the posterior urethra is quite common. The presence of valves of the posterior urethra or its stricture in
the boy, the doctor can clinically detect almost immediately after birth – such a child urinates with drops, not a jet,
straining when urinating. This pathology is almost immediately complicated by severe pyelonephritis with severe
intoxication, and is treated at the present stage mainly endoscopically.

Varicocele – varicose veins of pampiniform plexus of the spermatic cord; is the vascular pathology and is not related
to the violation of urodynamics. Boys usually get sick at the age of 12-17 years.
Forms:
- primary (idiopathic, usually left);
- secondary (symptomatic, more often it is a sign of volume formations in the retroperitoneal space).
The main complication is infertility (30-40% of cases).
Classification:
I-st. - veins are determined in a vertical position and under straining;
II – the veins are visualized during the inspection, do not collapse (disappear) in a horizontal position, the scrotum
hangs, testicle is an unmodified when palpation;
III – the conglomerate of varicose veins reaches the bottom of the scrotum, the scrotum is greatly lowered,
cremasteric reflex weakened, testicle is softened and reduced in size.
Clinic: characterized by discomfort in the scrotum, especially with prolonged exercise. Behind and above the
testicledilated veins are palpated (symptom of ball of earthworms), which are reduced or disappear in the supine
position. Over time, the testicle becomes be soft and decreases in size.
Diagnostics:
- Complaints, inspection, palpation;
- Ultrasound of the testicles, spermatic cords, kidney Dopplerometry of renal and spermatic veins;
- Tensiometry of the inferior Vena cava, renal, left common iliac vein.
- X-ray contrast examination:
- phleborenotesticulography;
- overview and excretory urography.
Treatment:
At I stage – restriction of physical loads (especially static), prevention of constipation, dynamic supervision.
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At II - III st. – surgical:
- Ivanisevich's operation (ligation of internal testicular veins);
- Palomo operation (ligation of the testicular vascular bundle in retroperitoneal space);
- endovascular sclerosing of left iternal spermatic vein;
- the formation of vascular anastomoses (testicular-iliac proximal or distal anastomosis, testicular-saphenal
anastomosis);
- laparoscopic ligation (clipping) of v. spermatica int.sin.