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Birth Defects (Congenital Malformations) of the Organs of the Urinary Tract

Some unfortunate children are born with abnormalities of the structure and function of the urinary tract organs. These children need careful evaluation and planning of surgical correction of such abnormalities. The common birth defects are

Pelvi Ureteric Junction (PUJ) Obstruction

In this condition, there is obstruction to the outflow of urine being produced by the kidneydue to a defect in the junction of the kidney pelvis and the ureter (tube connecting the kidney to the Bladder). As a result, the urine keeps getting accumulating in the Kidney, causing it to swell. This may be detected by an ultrasound examination during antenatal period (before Birth). If it is not diagnosed before birth, then the child may grow up normally. If the obstruction is partial, then it may never be detected. However depending upon the severity of obstruction, this condition may lead to pain, infection, fever and lump in the flank. If Left alone this may progress to failure of the kidney. The treatment is a surgical correction of the defect, which may be performed by an open operation, Laparoscopic operation or endourologically (ENDOPYELOTOMY) The decision of timing and type of surgical intervention would be determined by various factors like the age of the child, the severity of obstruction and the presence or absence of infection.

Posterior Urethral Valves (PUV)

Posterior Urethral Valves is a condition in which there is an abnormal piece of tissue in the exit pipe ( Urethra), causing obstruction to the flow of urine from the bladder to the exterior of the body.This results in accumulation of the urine in the bladder and back pressure in the kidneys. This condition occurs in male children only. 

The child may grow up without having any apparent problem, but in a majority of cases, this condition can be diagnosed in ante natal period( before birth).The child may complain of difficulty in passing urine, the mother may feel that the stream of urination is not adequate, or the child visibly needs to strain to pass urine. Sometimes this condition may come to light because the child is being investigated for urinary infection. Occasionally, the condition may be diagnosed too late when the kidneys have failed. 

Diagnosis is made by a test called MCU and the treatment involves destruction of the abnormal tissue by laser or electric current through a fine telescope passed into the urinary passage.

Vesico Ureteric Reflux (VUR)

Ordinarily when bladder discharges urine the urine flows only in one direction, outwards. The urine does not flow backwards towards the kidneys because of presence of valve like mechanism at the junction of ureters (tubes carrying urine from kidneys to the bladder) and the bladder. Vesico Ureteric Reflux (VUR) is a condition in which these valve mechanisms are defective and cannot prevent the backward flow of urine into the kidneys from the bladder. As a result, the urine in the bladder is allowed to rush into the kidneys as a high pressure jet, resulting in damage of the kidneys. This condition usually presents as urinary tract infection and thus requires frequent antibiotics. If the reflux is severe, then it requires a surgical procedure which may be in the form of an endoscopic procedure (STING) or Re implantation of ureters.

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This is a condition where ther is an obstruction to the movement of urine within the ureter, especially in the lower part. This usually is because of defective muscles of ureter in this portion. This prevents free drainage of urine from the kidneys and therefore may result in swelling of the kidneys causing pain and infection. The treatment involves surgical tailoring and re implantation of ureter.

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Ectopic Insertion Of Ureter

Occasionally, the junction of ureter (The tube carrying urine from the kidney towards the bladder) and the bladder is at an abnormal location. The ureter carrying urine from the kidney may open in the vagina, or prostate or the urethra. This may result in infections, pain and/or continuous flow of urine from the body. 

The treatment depends upon the function of kidney and usually requires surgical re-insertion(re implantation) of ureter into the bladder.

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