Congenital defects of the urinary system
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Congenital developmental defects of the kidneys
- relatively frequent congenital developmental defects;
- hypoplasia, dysplasia, cystic kidney diseases;
- often asymptomatic, sometimes prone to urinary tract infection;
- secondarily they can lead to the formation of a wrinkled kidney.
- The Potter sequence
- malformation characterized by hypertelorism, broad nasal root, epicantha, low-set auricles, hypognathia and lung hypoplasia due to oligo- to anhydramnios in developmental defects of the kidneys.Cite error: Closing
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Atresia and stenosis of the ureter
- Congenital mega(lo)ureter
- significant enlargement of the ureter;
- primary: based on congenital obstruction at the transition point of the ureter into the bladder;
- enlargement of the ureter and the hollow system of the kidney = ureterohydronephrosis;
- secondary: due to vesicoureteral reflux (VUR) or obstruction of the outflow part of the bladder (eg posterior urethral valve in boys);[1]
- Congenital ureterocele
Agenesis of the ureter
- Duplication of the ureter
- Position of the ureter
- Congenital vesico-uretero-renal reflux (VUR)
- backflow of urine from the bladder into the ureter or the hollow space of the kidney in case of failure of the valve (antireflux) mechanism at the level of the ureteral ostium in the bladder (shortened intramural section, ectopic ureteral ostium);
- occurs quite often in children; genetic background;
- consequences: dilatation of the hollow system, risk of urinary tract infection and post-infectious scars in the kidney parenchyma (deterioration of renal functions, development of hypertension), functional disorders of bladder emptying;[1]
- VUR grades according to MCUG:
- I. = VUR to ureter;
- II. = VUR to the ureter and to the calicopelvic system;
- III. = II + dilatation of the calicopelvic system;
- IV. = III + pressure atrophy of the parenchyma;
- V. = massive VUR with extensive destruction of renal parenchyma.[2]
- conservative solution (VUR I-III): sterile VUR is not too risky for the kidney → long-term chemoprophylaxis with the aim of preventing the formation of post-infectious scars of the kidney parenchyma; postnatally, the antireflux mechanism matures and VUR tends to resolve;
- operative solution (VUR IV-V): extension of the intramural section of the ureter and ureterocystoneostomy (reimplantation of the ureter);
- endoscopy: cystoscopically, an inert material is applied to the ostium to create an antireflux mechanism.[1]
Other congenital defects of the urinary system
- Epispadias
- Hypospadia
- Bladder exstrophy
- Posterior urethral valve
- the most severe congenital disorder of urine outflow – already prenatally leads to damage to the kidney parenchyma and glomerular filtration disorder;
- cystostomy is necessary immediately after delivery; later, the valve is surgically removed;
- only in boys; the long-term prognosis is not favorable - chronic renal failure develops with the need for dialysis or a kidney transplant.[1]
- Other atresia and stenosis of the urethra and bladder neck
Defects of the brain
- urachu cyst, persistence, hernia
- Congenital absence of bladder and urethra
- Congenital bladder diverticulum
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References
- ↑ Jump up to: a b c d Cite error: Invalid
<ref>tag; no text was provided for refs namedKlinPed2012 - ↑ MUNTAU, Ania Carolina. Pediatrics. 4. edition. Grada, 2009. pp. 442. ISBN 978-80-247-2525-3.
