Ectopic Ureter

Normally, human beings are born with two ureters – one drains the urine from each of the two kidneys into the bladder. At times, one of these ureters might not be connected properly to the kidney or bladder and even be unable to drain the connected kidney’s urine efficiently. This results in ectopic ureter.

Fortunately, there are effective diagnostic tests and treatment methods that are useful for detecting and treating any abnormality resulting from such a condition.

What causes ectopic ureter?

Normally, there is one ureter each which connects one kidney to the bladder (2 ureters for the 2 kidneys each). Each ureter is responsible for draining the urine from the kidneys into the bladder until the bladder is full and needs urination to empty it.

At times, there might be additional (total 2) ureters connecting a single kidney to the bladder. One of the ureter drains the upper portion of the kidney while the other connects the lower part. This is also known as ‘duplicate collecting system’ and it works normally as long as the kidney is being efficiently drained. In rare cases a child might exhibit ectopic ureter which is not connected properly to the bladder and drains outside of the bladder. In female children, the ectopic ureter drains into the vagina or into the urethra instead of the bladder. In male children, the ectopic ureter may cause the ureter to drain the urine into the urethra near the prostate or into the genital duct system.

Ectopic ureter may be seen in non-duplicated collecting system but is more common in a duplicated collecting system.

What symptoms are seen in ectopic ureter?

Common symptoms of ectopic ureter include urinary incontinence (inability to control urine) or blockage in the ureter. The kidney might become swollen or distended due to poor drainage if it is accompanied by pressure on the back. This is known as hydronephrosis and is easy to distinguish using an ultrasound test. This is the reason that most cases of ectopic ureter are easily detected during the fetal stage with a regular prenatal ultrasound test.

The risk of UTI (urinary tract infection) is increased from poor drainage of the kidney due to ectopic kidney. Apart from hydronephrosis, ectopic ureters in girls can result in incontinence in urination since the ureter drains the urine directly into or near the vagina. This problem is mainly noticed while toilet training the child due to the typical constant moistness it causes, which is different from loss of bladder control episodes. At times, the girl child may be treated using medication or other therapies incorrectly till the correct diagnosis is done many years later. Boys may show signs of hydronephrosis or UTI (urinary tract infection).

Ectopic ureter also results in small flaws in the ureteral connection between kidney and bladder. This can result in VUR (vesicoureteral reflux), which causes blockage of the urine flow from the kidney into the bladder and out of the urethral opening. This causes the urine to flow backwards, from the bladder into the kidney. VUR increases the risk of UTI in children.

How is ectopic ureter diagnosed?

The presence of ectopic ureter is normally detected according to the specific symptoms exhibited by the child. In case hydronephrosis is detected in the fetal stages, then an ultrasound may be repeated after birth as well.

VCUG (voiding cystourethrogram) – x-ray of bladder – is required to rule out the possibility of VUR (vesicoureteral reflux) as the cause of swollen ureter or kidney. This test is also helpful in detecting reflux in the additional ureter connecting the same kidney. A combination of VCUG and ultrasound can help to determine the presence of hydronephrosis.

Other diagnostic tests including formal renal x-ray (IVP) or renal flow scan can help to view the internal working conditions of the affected kidneys efficiently. These test involve injecting contrast dye around the kidney and using x-ray imaging to view the functioning of the kidneys.

The information collected from these scans and tests are most helpful in determining the best-suited treatment method as well.

Last, but not the least, a cystoscopy may also be performed at the time of the treatment using minimally invasive telescopic device to view the internal working and functioning of the kidneys. There are often times when the ectopic ureteral opening cannot be determined. This can be overcome by identifying the locations and the number of ureter openings (when multiple ureters are present).

In case the child exhibits symptoms of urinary incontinence the same diagnostic tests are performed. However in case the ureter is not swollen and no reflux is seen then the results of VCUG and ultrasound are normal. In case symptoms of ectopic ureter are present then renal flow scan or IVP, along with an occasional CT (computerized tomography) scan can help to see the abnormality of the ectopic ureter and the portion of the kidney it is connected to. As diagnosis is often complicated and there are other causes of urinary incontinence as well in children, some children may suffer from the condition for years before correct diagnosis is done.

How is ectopic ureter treated?

Surgical procedure is the best-suited treatment option for ectopic ureter. In order to control the infection, the patient is usually prescribed a low dose of antibiotics before the surgery.

There are mainly three types of surgical procedures used to treat ectopic ureters:

Nephrectomy (upper pole heminephrectomy)

This surgical procedure requires removing the portion of the kidney connected by the ectopic ureter. This helps to stop the flow of urine into the ectopic ureter, which results in treating the urinary incontinence and decreasing the risk of infection. This is comparatively the simplest surgical procedure and has the lowest risk for complications.
This is a better treatment option when the whole kidney or a portion of it is showing improper drainage. It is also useful in case the affected kidney portion and the other kidney is also functioning properly. Today, this procedure is performed using minimally invasive surgical technique (laparoscopy) in most patients, whenever advisable by the urologist/surgeon.
Complications, if present, may include removal of potentially functioning kidney tissue from the bottom portion of the ectopic ureter when it is left intact. The remaining portion of the ectopic ureter may be at slight risk of infection in the future.

Ureteropyelostomy

This surgical procedure aims to divide the ectopic ureter near the bottom and attach it to the bladder so that the urine drains normally and prevents reflux (backward flow).
This procedure has the advantage of leaving the kidney tissue safe along with the lower half of the ectopic ureter intact. However, there is a slightly higher risk of complications in this procedure.

Ureteral reimplantation

This surgery also aims to divide the ectopic ureter in the lower half and connect it properly with the bladder to enable normal flow from the kidney into the bladder, without the risk of reflux.
This is performed using conventional surgical approach with an incision in the lower abdomen, just above the pubic bone.
This procedure is comparatively more prone to complications and is often not advised for infants due to higher complication risk. Unlike ureteropyelostomy, this procedure saves the complete kidney tissue and also
Helps to remove additional ectopic ureter, which is not seen with the other two procedures.

What is recovery like after treatment for ectopic ureter?

Recovery is mainly determined by the type of surgical treatment used. Infants and small children are normally hospitalized for 1 to 5 days following the surgery. A catheter may be attached to prevent fluid accumulation and is removed without discomfort or pain before the child is discharged from the hospital, or in one of the follow-up visits. The catheter incision is stitched close and heals on its own in a few days.

Frequently asked questions:

Are boys or girls more likely to have an ectopic ureter?

This disorder is more common in boys as compared to girls, but develops in both sexes.

What is the optimal age for ectopic ureter surgery?

Nephrectomy and heminephrectomy procedures are normally performed once the infant is over 1 month old. At times, the surgeon may advise to wait for a little longer than that (usually after the child is 1 year old) in order to perform ureteral reimplantation.

What are the risk factors for an ectopic pregnancy?

There are no known risk factors that determine the development of ectopic ureter. This is a congenital (birth) defect that is believed to occur due to abnormal connection between bladder and ureter.

Was this caused by something during pregnancy?

There is currently no evidence to prove that this condition is caused due to anything that the mother does or was exposed to during pregnancy.

Does an ectopic ureter have any impact on my child’s future sexual function?

Although an ectopic ureter drains directly into the genital tract there is no adverse effect on the sexual functioning and rarely causes infertility problems. For boys, there may be cases where the genital tract may be on the same side as the ectopic ureter but this does not affect fertility if the other side remains unaffected (which is normal in most cases).

If a portion or the entire kidney is functioning poorly or is removed, will my child have long-term kidney problems?

No, as long as the other kidney functions normally. Most of the times, the ectopic ureter affects the upper portion of one kidney which is responsible for providing around 1/3rd functioning. Even if the entire kidney is affected the risk of long-term kidney problems is highly unlikely.

The only complication is that the child will not have a ‘spare’ kidney but the other functions of the body remain normal with a single healthy kidney as well.