ECTOPIC Ureter

Ectopic Ureter (EU) is a congenital malformation of the renal/urinary system, where the two ureters bringing urine from the kidney do not attach at the normal trigonal location in the bladder.  It is a condition common to humans and dogs - likely most mammals.  Clinical presentations can range from no clinical signs, occasional leaking of urine, constant dribbling of urine, all the way to renal failure and death. The most common clinical presentation is slight urine leaking with occasional bladder infections, noticed especially during the house training stage.  If your puppy is not learning house training in a reasonable amount of time, ask your veterinarian about this possibility.  

Diagnosis of Ectopic Ureter can be achieved in a few different ways.  Historically, in North America, a full internal scan of organs was conducted.  This is a very invasive procedure and is a last resort effort to resolve significant health issues, beyond a little occasional leakage.  Often, the dog would be sedated, examined by scope and a repair attempted in a single procedure.  

Treatment for Ectopic Ureter can vary depending upon the severity of the abnormalities. For dogs with minimal abnormalities and clinical signs, incontinence medication is the extent of treatment. In some individuals, surgical removal of an affected kidney and ureter is indicated. When critically afflicted individuals are diagnosed early with prompt surgical removal of a kidney and ureter, they can lead long happy lives. Rarely, there are severely afflicted dogs where both kidneys are affected and the prognosis is very poor.

The “silence” of ectopic ureters in Entlebuchers makes it difficult, almost impossible at this time, for breeders to know if their breeding dogs have an increased risk for producing EU in offspring.  The Entlebucher clubs in Europe participate in an EU screening and grading program and now most puppies come with a health rating for EU.  

The European clubs believe that EU is often transferred to offspring via an asymptomatic father, which makes it difficult to trace.  Their work shows that by identifying the degree of EU in the mating dogs, they can selectively breed to greatly reduce the incidence of EU in their population.  They grade screened dogs based on the degree of abnormality in the ureter to bladder junction.  Dogs with normal connections are graded "A".  If only one ureter is misplaced a short distance, they receive a grade of "B".  If both ureters are involved or the displacement is severe, they receive a grade of "C".  They have greatly reduced the incidence of EU by limiting breeding to A-A and A-B pairs.  

In North America, we are not there yet.  The process in Europe started with just a couple of veterinarians, very experienced in ultrasound.  They developed a protocol for diagnosis and screening in the Entlebucher.  In North America, we have just (May 2026) found two veterinarians willing to work on this important project.  We hope that we too may be able to screen breeding dogs very soon!  

What are the common clinical signs of EU?

Signs may include:

• abnormal urination patterns such as difficulty with house training or incontinence when sleeping or when fatigued
• frequent urination with large volumes of urine
• a bloated, overly distended abdomen
• failure to thrive
• recurring bladder infections
• sudden repetitive vomiting

What is known about EU?

EUS appears to be characterized by heritable abnormalities throughout the urinary tract that include some combination of ureteral ectopia, ureteral obstruction, hydroureter, and hydronephrosis (see diagram with definitions). Ureteral ectopia is the most minor of these anatomical abnormalities. It can occur in many breeds and is not life threatening.

In normal dogs, the urine flows from the kidney, down the ureter and into the bladder where the urine is held until the dog urinates. With ectopic ureters, the ureter joins the bladder in an abnormal place, usually in the bladder neck or even farther down in the urethra. In most dogs, this abnormal placement is characterized by incontinence (leaking urine). However, the presentation in Entlebucher dogs is unique. EU research study, at Michigan State University (MSU), has shown that Entlebuchers can have ectopic ureters and have NO clinical signs. These dogs do not leak or dribble and can produce offspring that do not leak or dribble. Entlebuchers with isolated ectopic ureters typically lead long lives without showing any signs of disease.

However, MSU research has also shown that some of the Entlebuchers with ectopic ureters do leak or dribble. These dogs respond well to medical management with Veterinary prescribed incontinence medication.

Unfortunately, the Entlebucher is also unique in that this abnormality can include not only abnormal ureter placement, but also an abnormality of the physical junction of the ureter. This can lead to a severe case of EU with abnormal dilation of the ureter and the kidney. When only one side is affected, surgical intervention can be life saving. Often times, in these cases, the affected kidney and ureter need to be surgically removed. Surgically realignment of the other ureter may also be necessary. Very rarely, both ureters and kidneys are affected and the prognosis is grave.

How common is EU?

All forms of EU are uncommon with the mildest condition of urine leaking occurring with the most frequency. Fortunately, it is uncommon for Entlebuchers with EU to have one or both kidneys affected. The precise estimate is not known, but we estimate less than5% of all Entlebuchers in the United States and Canada have been afflicted. Nevertheless, NEMDA breeders continue to monitor carefully for any new puppies with the syndrome.

Which dogs produce puppies with EU?

While the NEMDA club has been aware of EU for over 15 years and despite careful selection in breeding pairs, we presently have no means for accurately predicting EUS either symptomatic or asymptomatic within the breed. To our knowledge, there are no breeding lines that can guarantee an EU-free puppy.

What can NEMDA breeders and puppy owners do?

• Be alert to signs and symptoms of EU in puppies within the first year or two of life.
• Keep open lines of communication between puppy owners, breeders, Veterinarians, and the chairs of the NEMDA Health & Genetics committee.
• Breeders have EU leaflets to distribute to their new puppy owners. Puppy owners should ask their Veterinarian to place a copy of this information on the puppy’s chart.
• If a new EU puppy is suspected or identified, please contact the NEMDA Health & Genetics chair.
• It may be appropriate to ask your local Veterinarian to contact the veterinary researchers at MSU to determine if collaboration could be mutually beneficial. The Health & Genetics Committee chair can help you to do so and provide other support as needed.