What is Ectopic Ureter?

Male Urinary Tract
Male Urinary Tract
Medical Illustration Copyright © 2015 Nucleus Medical Media, All rights reserved

Female Urinary Tract
Female Urinary Tract
Medical Illustration Copyright © 2015 Nucleus Medical Media, All rights reserved

Ectopic Ureter
Ectopic Ureter
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Most of us are born with 2 ureters, the tube that drains the urine from each kidney into the bladder.

But some babies are born with 2 ureters that drain a single kidney. In these cases, one ureter drains the upper part of the kidney and the second ureter drains the lower part of the kidney. As long as they both enter the bladder, this extra ureter is usually not a problem.

But if a child is born with a ureter that does not connect with the bladder, it can drain somewhere outside the bladder. This is called an ectopic ureter. In girls, the ectopic ureter can drain into the urethra (the tube leading out of the bladder that we use to pee out of) or even the vagina. In boys, it often drains into the urethra near the prostate or into the sex organs.



What Causes Ectopic Ureter?

There are no known causes for this birth defect. This extra ureter just develops from a single kidney. It can happen in boys or girls, but is more common in girls. There is also no proof that something during pregnancy causes it.

It is known to be hereditary (passed down in a family). If you had an ectopic ureter, then each of your children has a one-half chance of having this duplicated system.



What are the Signs of Ectopic Ureter?

The general signs of ectopic ureter are:

Incontinence

A blocked ureter or incontinence (leaking urine) can be signs of an ectopic ureter.

  • Boys with ectopic ureters do not often have incontinence, since the ureter drains inside the body. But they may still have signs of swelling or a UTI.
  • Girls with ectopic ureters may leak urine since the ureter drains into or near the vagina. This problem is clear after toilet training. It is unlike other types of incontinence. Instead, there is a general, all-over moistness rather than events where the bladder looses control. Some girls will be treated with drugs and other things for many years before the right diagnosis is made.

Swelling

The ureter and part of the kidney can swell when it cannot drain properly. This is called hydronephrosis and it is easy to see on ultrasound.

In many babies, an ectopic ureter is found when the pregnant mother has a prenatal ultrasound. But not all ectopic ureters are swollen, so other tools may be better for a clear diagnosis.

Urinary Tract Infection

Poor drainage from an ectopic ureter may make children more likely to have UTIs. A UTI is when bacteria gets into the urine and travels up to the bladder. It becomes painful to urinate.

Vesicoureteral Reflux (VUR)

In boys with an ectopic ureter, there may also be a slight flaw in the ureter. This can result in VUR. VUR is when some urine flows backward into the kidney when the bladder fills or empties. This makes the risk of kidney infections higher. It is one more reason some children with ectopic ureters show signs of a UTI.



How is Ectopic Ureter Diagnosed?

It is most often found in children, and how it is found depends on how the problem first shows up. This issue is not always easy to pinpoint. Other problems have similar signs, so sometimes it is not correctly diagnosed for years.

If the doctor thinks there may be an ectopic ureter but can’t see it at first, then other tests may be done for a clear diagnosis.

There are some basic tests to see the ureters, kidneys and bladder are:

Ultrasound

  • If the ultrasound before birth shows swelling, then the test is often done again after the child is born.

Bladder X-ray (Voiding Cystourethrogram, or VCUG)

  • This test is then done to rule out VUR as the cause of the swelling of the kidney and ureter. This test is also used to see if there is reflux in a second ureter linked to the ectopic ureter.

Renal Flow Scan (MAG-3 or DMSA) or Magnetic Resonance Urogram

  • The kidney or part of the kidney drained by the ectopic ureter often doesn’t work well. In these tests, contrast dye liquid is injected. A special camera is used to spot radioactivity in the dye. MRI (magnetic resonance imaging) can also be used.

Cystoscopy

  • In this test, often done with general anesthesia, a small scope is placed into the urethra. The openings of the ureters from both kidneys are found. X-ray liquid dye is injected through tubes placed temporarily in the ureters to show the anatomy. Unfortunately, the opening of the ectopic ureter cannot always be seen. But by pinpointing the number and location of the other ureteral openings, the doctor can be sure.

But if the ureter is not swollen and there is no reflux, the ultrasound and VCUG may be normal. MRI and CT scan are better at finding slight swelling of the ureter and the part of the kidney it drains.



How is Ectopic Ureter Treated?

The treatment for ectopic ureter is surgery. To prevent infection, the patient may be put on a low dose of antibiotics before surgery. The 3 types of surgery to fix this problem are:

  • Nephrectomy
  • Ureteroureterostomy
  • Ureteral reimplantation

Each option has pros and cons. They are also done at certain ages. Nephrectomy can be done any time. But if there is no infection, surgery can be put off until the age of 6 months. Some surgeons like to wait until a child is even older, often after the first birthday, for ureteral re-implantation.

Nephrectomy (upper pole heminephrectomy)

In this surgery, the kidney (or the part of it drained by the ectopic ureter) is removed. This stops the flow of urine into the ectopic ureter. Now there will be no more incontinence, and less chance of infection.

This is a good method when the kidney (or the part of it) draining through the ectopic ureter is not working well. It may also be used when that part of the kidney works well and the other kidney is normal. The surgeon may use laparoscopy (surgery using keyhole-sized incisions and long, chopstick-like instruments) since most of the abnormal ureter can be removed.

The main risks of this surgery are that good kidney tissue may be removed, or the blood supply may be injured. If there is no VUR into the ectopic ureter, there is less risk for infection.

Ureteroureterostomy

For this surgery, the ectopic ureter is split near the bladder. It is then sewn into the normal ureter of the lower kidney. This lets the urine from the upper part of the kidney drain normally. The kidney tissue is also protected.

The risk of harm to the lower ureter is very small with this surgery. There is a small risk of urine leak. If a temporary internal drainage stent (tube) is placed, more anesthetic may be used to remove it.

Ureteral Re-implantation

For this surgery, the ectopic ureter is split near the bottom and sewn into the bladder. This is done so that urine drains well and does not flow backwards. This surgery often requires a cut above the pubic bone.

This method has a slightly higher chance of problems than the other surgeries. It can be difficult to perform in small infants. The surgeon needs to make the ureter smaller, so there is less risk of VUR later on. This increases the risk of blockage. But like ureteroureterostomy, this operation preserves all kidney tissue.



What Can Be Expected After Treatment?

Recovery

Recovery depends on which operation was done. Infants and small children usually stay in the hospital from 1 to 5 days after to heal.

  • If a catheter (tube) was used, it will be taken out easily before the child goes home or in the office at a checkup. The area where the catheter went in will heal on its own. Stitches will not be needed.
  • If an internal drain was used, it will be removed 3 to 4 weeks after surgery.

Imaging after Treatment

A renal ultrasound is usually done 4 to 6 weeks after surgery to see if the surgery worked. If ureteral re-implantation was done, VCUG may be used to see if there is any reflux.

Future Kidney Problems

If part or all of a kidney is working poorly or removed, but the other kidney is normal, the child will not have lifelong kidney problems. Most often just the upper part of one kidney is affected. This means the rest of the kidney can function well. Even when a whole kidney is affected, long-term problems are not likely.

One normal kidney is all we need for good health. Children are often born with a single kidney and no one knows it. People who donate a kidney also do fine. The only issue is that you no longer have a spare kidney. If your remaining kidney is seriously injured (for any reason) then you would have kidney failure and require dialysis.

Sexual Function

Though an ectopic ureter can drain to the sex organs, it does not affect sexual function. This issue rarely impacts a person’s ability to have a baby.

In boys, the genital tract on the same side of the ectopic ureter may be abnormal. But if the other side is not affected (which is most often the case), then fertility should still be normal.