The Kidney: From Normal Development to Congenital Disease

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This can occur because of a constricting band of fibrous scar-like tissue or from a crossing blood vessel. Though it is generally present at birth, sometimes it is so mild as to not cause a problem, but the obstruction may worsen with time later in infancy or in childhood as a result of changing relationships of the structures of the urinary tract and of surrounding tissues as growth occurs. A band of tissue or blood vessel near the ureter may impinge on the ureter with time and growth. This is usually a unilateral finding, but it can be bilateral, and it also is associated with problems with the other kidney.

Multicystic dysplastic kidneys are associated with a UPJ obstruction of the other kidney. Twenty percent of babies with a multicystic dysplastic kidney have a UPJ obstruction of the other kidney. UPJ obstruction usually must be fixed surgically. The surgery is safe and successful, requiring only days in the hospital.

Generally if it is discovered in the newborn period and is unilateral, the surgery is postponed a month or two until the baby is stable and growing and doing well. It is an abnormality in the insertion of the ureter into the bladder, blocking the flow of the urine into the bladder, and is fixed by surgically reinserting the ureter into the bladder, the same as is done for vesicoureteral reflux see hydronephrosis without obstruction.

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A ureterocele is a cystic dilatation of the ureter as it inserts into the bladder. The cystic area "pouches" into the bladder and causes the opening for urine flow from the ureter into the bladder to be very constricted, resulting in obstruction and hydronephrosis. It can often be handled at cystoscopy, with excision of the abnormal tissue with a small knife passed through the cystoscope.

There can be associated dysplasia on the side of the ureterocele, but not always. A neurogenic bladder is usually associated with a problem with the spinal cord, the most common being a myelomeningocele often referred to by the lay public as spina bifida. This is a defect where the vertebrae in the lumbosacral area are not properly fused all the way around the spinal cord, and so a sac protrudes from the spinal cord with a mass of nerves all tangled together. In these nerves are those to the bladder, so that it has no functional nerve supply.

The bladder then tends to fill and fill and only empty by overflow when it is quite full. There is often backpressure to the kidneys. This can lead to vesicoureteral reflux explained later in this treatise and chronic infection and scarring of the kidneys, with eventual renal failure. Hydronephrosis without obstruction, including vesicoureteral reflux.

What causes urinary tract abnormalities to occur before birth?

Hydronephrosis without obstruction means dilatation of the ureter and collecting system of the kidney but not because of backup above an area of narrowing and obstruction, rather primary dilatation of the collecting system and ureter. The conditions causing this that I will discuss are:. Prune belly syndrome occurs almost exclusively in boys; there is a controversy whether there is a variant in girls. Frequently the dilated ureters come with vesicoureteral reflux VUR; see below.

The boys are sterile and unable to make sperm, even if the testicles are surgically brought down, but they function normally sexually. The bladder may be large and floppy and not contract and empty like it should, or it may be fine. High-grade reflux is common, but reimplantation of the ureters surgically into the large, floppy bladder may be difficult. With the renal dysplasia, renal failure may be a problem.

The renal dysplasia may be of any degree of severity. Some of these boys progress to needing transplantation; others do not. In boys with prune belly syndrome the abdomen is protuberant because of the decreased or absent abdominal muscles and the lower part of the rib cage in front may flare out a little, causing an abnormal profile. The absence of the muscles of the abdomen causes surprisingly little functional problem.

Some boys as they get older wear an elastic support garment around the abdomen to hold it in so that it looks better and clothes fit better. It also offers some protection to the abdominal organs.

The Kidney: From Normal Development to Congenital Disease by Peter D. Vize

Vesicoureteral reflux VUR involves the two-way flow of the urine up and down the ureters, and they should be a one-way down system. The ureter inserts into the bladder down near its base such that the muscle of the bladder wall does not allow the urine to flow out of the bladder and back up the ureter towards the kidney. This insertion can be abnormal and allow different degrees of back flow, which are graded I to V, depending on severity. When grades I to III happen in an infant or young child, the likelihood is that with time and increasing maturity the problem will resolve itself.

Grade IV reflux occasionally spontaneously resolves, and grade V reflux virtually never resolves spontaneously. Grades IV and V usually need surgical correction. Megaureter and megacystis are two problems that often occur together. Megaureter is the congenital dilatation of all or a portion of the ureter without obstruction.

Megacystis means enlargement of the bladder, which may affect function in that the bladder may be slow to empty, and in some cases this may mean that intermittent catheterization is needed to empty the bladder. Both megaureter and megacystis are frequently associated with renal dysplasia.

Most of the cystic diseases occur in both kidneys, but in the great majority of cases a multicystic dysplastic kidney is confined to one kidney. In this disorder, the kidney is worthless and without any functioning filters and the ureter is atretic, meaning that it stops part way down to the bladder, so is incomplete. The "kidney", and I put it in parentheses since it is not really a kidney, as we know it, since something went awry very early in its development and it became a group of cysts rather than a kidney.

A cyst is a fluid filled round bag-like structure. In most cases the other kidney is normal and grows larger than a normal kidney to make up for the fact that there is only one functioning kidney.

What is Congenital Kidney Disease: Symptoms, Causes, Diagnosis, and Treatment

Two defects do occur in the functioning kidney with enough frequency to be of concern. The first is vesicoureteral reflux discussed previously in this article so every infant found to have a multicystic dysplastic kidney should have a VCUG, a voiding cystourethrogram to look for reflux.

The second is ureteropelvic junction obstruction, also discussed previously, which, if present, will have been noted on the ultrasound examination that was done to pick up the multicystic dysplastic kidney. A multicystic dysplastic kidney is often picked up on prenatal ultrasound, then confirmed on ultrasound after the baby is born.

Since the bags of cysts can be confused with severe dilatation of the kidney related to a severe ureteropelvic junction obstruction, the doctor may want to look at the kidneys with a nuclear renal scan to differentiate between the two. To do this scan, the infant is given an IV injection of a radioactive substance that shows blood flow into the kidney and is filtered by the kidney. The multicystic dysplastic kidney has no blood flow and no function, but the kidney with obstruction has blood flowing through it.

The amount of radioactivity given is no more than one receives getting a standard X-ray. So the infant felt to have a multicystic dysplastic kidney generally has two tests done, a VCUG and a nuclear renal scan. If an associated problem such as reflux or UPJ obstruction is found, they are treated as noted above, with the need for treatment being related to the severity of the problem.

The multicystic dysplastic kidney itself is followed with serial ultrasound examinations at intervals over time. Most of the time when cystic kidney disease is mentioned, polycystic kidney disease PKD comes to mind. Let me give you a mini-genetics lesson to help explain the two diseases. Each of us has a set of genes from each parent, so we have a double of every gene. In diseases where the trait is dominant, getting an abnormal gene from either parent gives you the disease.

So in the typical case of a parent with a dominant disease, the parent got one normal gene from one parent who was healthy and an abnormal gene from a parent with the disease. So the child of that parent has a chance of getting the disease, depending on whether he got the diseased gene or the healthy one. Having one gene with the disease means you have the disease. In the case of parents of a child with a recessive disease, both parents have one normal and one abnormal gene, and the normal, rather than the abnormal gene, predominates.

A child of such parents has a one out of two chance of getting the abnormal gene from each parent, or a one out of four chance of getting an abnormal gene from both parents. Having both genes of the set abnormal gives the disease. So parents of the child with ARPKD are healthy and do not know that they carry the disease until they have a child with the disease.

The kidneys