Ectopic Ureter

What is an ectopic ureter?

The ureters are a pair of narrow tubes that carry urine (pee) from the two kidneys to your bladder. Usually, a person has one ureter attached to each kidney and each connects to one side of their bladder.

An ectopic ureter is a congenital abnormality of the ureter. A person is born with a ureter that connects to the wrong place. That is, an ectopic ureter carries urine to somewhere other than the bladder, such as:

• Bladder neck (at the bottom of the bladder, where urine enters the urethra and then leaves your body).
• Rectum (the end of the large intestine, leading to the anus).
• Seminal vesicles (glands that help produce semen in males).
• Urethra (the tube leading out of the bladder).
• Uterus (womb), cervix (lower part of the uterus) or vagina in females.
• Vas deferens or ejaculatory ducts, which carry sperm in males.

How can an ectopic ureter affect my body?

An ectopic ureter can cause:

• Urinary incontinence (leaking pee, trouble controlling your bladder).
• Urinary tract infections (UTIs, bacterial infections that can make it painful to pee).
• Hydronephrosis (swollen kidney because it can’t fully empty).

In rare, severe cases that aren’t treated, the effects of an ectopic ureter may damage your kidneys.

Who might have an ectopic ureter?

Anyone can be born with an ectopic ureter. This congenital defect can run in the family, so if a parent has an ectopic ureter, a child has a higher chance of having one.

For unknown reasons, ectopic ureters are about 10 times more common in girls.

Sometimes ectopic ureters happen with other structural abnormalities with the kidneys or urinary system. For example, a person may have a duplex kidney, meaning a kidney has two ureters instead of one.

How common are ectopic ureters?

About 1 in 1,900 people are diagnosed with an ectopic ureter. But it’s probably even more common because it’s often not diagnosed or misdiagnosed.

What causes an ectopic ureter?

Ectopic ureter has no known cause. Scientists don’t yet understand why ureters sometimes form differently while a fetus is developing. However, this congenital abnormality often runs in families. If you have an ectopic ureter, each of your children has a 50% chance of developing it.

What are the symptoms of an ectopic ureter?

The signs of ectopic ureter include:

• Frequent UTIs, including burning or pain when peeing.
• Incontinence.
• Swelling in the abdomen caused by hydronephrosis.

How is an ectopic ureter diagnosed?

Sometimes an ectopic ureter is diagnosed before birth, during a prenatal ultrasound. For those that aren’t, an ectopic ureter can be difficult to diagnose because other problems have similar signs and symptoms. Even though it’s present at birth, the condition is often missed or misdiagnosed for years.

To make a diagnosis, a healthcare provider will:

• Ask about your symptoms and how long you've had them.
• Ask you whether any family members have had similar problems.
• Conduct a physical exam.
• They may order blood tests to test kidney function and urine tests to check for infection.

The healthcare provider also might order some other tests:

• Imaging tests: A CT scan, MRI or ultrasound may be used to take pictures of the urinary system.
• Cystoscopy: A cystoscopy is used to examine the urethra and bladder. The provider inserts a thin tube with a small camera through the urethra to look inside your bladder.
• Renal flow scan: This test is performed to see how well your kidneys are working and check for abnormalities. A provider injects a small amount of radioactive dye into the body. A gamma camera shows how the dye flows through the urinary system.
• Urography: This test also uses a dye injected into the body to examine the urinary system. X-rays, a CT scan or MRI takes images of the kidneys, bladder and ureters as the dye moves through them.
• Voiding cystourethrogram (VCUG): A VCUG is done to look for bladder and urethra abnormalities and determine whether you have reflux.

How is an ectopic ureter treated?

The only treatment for an ectopic ureter is surgery. The goal is to correct the congenital defect or drain urine away from the kidney to prevent damage.

Different types of surgery may be used:

• Ureteroureterostomy: This surgery creates a new connection to correct the problem. For example, it may connect an ectopic ureter with a normal one.
• Ureteral reimplantation: This surgery attaches the ureter to the correct position in the bladder wall.
• Ureterostomy: Ureterostomycreates an opening (stoma) in the surface of the skin on the abdomen. Then urine drains into a pouch or diaper. This will require a second procedure at a later time.
• Nephrectomy: Nephrectomy removes part of the kidney (partial nephrectomy) or the entire kidney (radical nephrectomy). This surgery is used as a last option, when the kidney function is low and the other kidney is healthy.

How can I prevent ectopic ureter?

There’s no way to prevent an ectopic ureter. But if you have it, consider mentioning it to your children’s healthcare providers. This congenital abnormality can run in families. Your obstetrician or pediatrician may recommend tests to diagnose ectopic ureter early.

What is the outlook for people with an ectopic ureter?

The outlook for people with an ectopic ureter is generally good after treatment.

Even if part or all of a kidney is removed, the other kidney can do the work. And an ectopic ureter rarely affects sexual function or a person’s ability to have children.

What else should I ask my healthcare provider?

Consider asking your healthcare provider some questions to understand your specific situation:

• Where is the ectopic ureter located, and where is it connected?
• Where is the urine draining?
• Are there any other abnormal structures in that area?
• Should I consider surgery?
• What type of surgery would be best?
• What is the recovery like after that kind of surgery?
• Will I/my child have enough kidney function after surgery?
• Will this affect my/my child’s sexual function or ability to have children?

A note from Cleveland Clinic

An ectopic ureter is a congenital abnormality. The condition is present at birth but can be hard to diagnose. An ectopic ureter can cause urinary incontinence, recurring UTIs, swelling in the abdomen and even kidney damage. Talk to your healthcare provider for diagnosis and options for surgery.