Congenital Ureter Anomalies

Definition

The ureter drains urine from the kidney into the bladder. It is not simply a tube but an active organ that propels urine forward by muscular action. It has a valve at its bottom end that prevents urine from flowing backward into the kidney. Normally there is one ureter on each side of the body for each kidney. However, among the many abnormalities of ureteral development, duplication is quite common. Ureters may also be malformed in a variety of ways-some harmful, others not.

Description

The urogenital system, for some reason, is more likely than any other to have birth defects, and they can occur in endless variety. Ureters can be duplicated completely or partially, they can be in the wrong place, they can be deformed, and they can end in the wrong place. The trouble these abnormalities bring is directly related to their effect on the flow of urine. As long as urine flows normally through them, and only in one direction, no harm is done.

• Duplication of ureters is quite common, either in part or completely. Kidneys are sometimes duplicated as well. Someone may have four kidneys and four ureters or two kidneys, half of each drained by aseparate ureter, or a single kidney with two, three, or four ureters attached. As long as urine can flow easily in the correct direction, such malformations may never be detected. If, however, one of the ureters has a dead end, a stricture or stenosis (narrowing), or a leaky ureterovesical valve (valve between the ureter and bladder), infection is the likely result.
• Stricture or stenosis of a ureter prevents urine from flowing freely. Whenever flow is obstructed in the body-urine, bile, mucus, or any other liquid-infection follows. Ureters can be obstructed anywhere along their course, though the ureterovesical valve is the most common place.
• A ureter may have an ectopic (out of place) orifice (opening)-it may enter the bladder, or even another structure, where it does not belong and therefore without an adequate valve to control reflux.
• The primary ureter, or a duplicate, may not even reach the bladder, but rather terminate in a dead end. Urine will stagnate there and eventually cause infection.
• A ureter can be perfectly normal but in the wrong place, such as behind the vena cava (the large vein in the middle of the abdomen). A so-called retrocaval ureter may be pinched by the vena cava so that flow is hindered. Other aberrant locations may also lead to compression and impaired flow.

Besides infection, urine that backs up will cause the ureter and the kidney to dilate. Eventually, the kidney will stop functioning because of the back pressure. This condition is called hydronephrosis-a kidney swollen with urine.

Causes and symptoms

The causes of birth defects are multiple and often unknown. Furthermore, the precise cause of specific birth defects has only rarely been identified. Such is the case with congenital ureteral anomalies.

Practically the only symptom generated by ureteral abnormalities is urinary tract infection. A lower tract infection-in the bladder-is called cystitis. In children, it may cause fever and systemic symptoms, but in adults it causes only cloudy, burning, and frequent urine. Upper tract infections, on the other hand, can be serious for both adults and children, causing high fevers, back pain, severe generalized discomfort, and even leading to kidney failure or septicemia (infection spreading throughout the body by way of the blood stream).

In rare cases, urine from an ectopic ureter will bypass the bladder and dribble out of the bottom somewhere, through a natural orifice like the vagina or a completely separate unnatural opening.

Diagnosis

Serious or recurrent urinary infections will prompt a search for underlying abnormalities. Cystoscopy (looking into the bladder with a thin telescope-like instrument) and x rays with a contrast agent to illuminate the urinary system will usually identify the defect. Computed tomography scans (CT) and Magnetic resonance imaging (MRI) may provide additional information. Urine cultures to identify the infecting germs will be repeated frequently until the problem is corrected.

Treatment

Sometimes the recurring infections caused by flow abnormalities can be treated with repeated and changing courses of antibiotics. Over time, the infecting germs develop resistance to most treatments, especially the safer ones. If it can be done with acceptable risk, it is better to repair the defect surgically. Urologists have an arsenal of approaches to urine drainage that range from simply reimplanting a ureter into the bladder, in such a way that an effective valve is created, to building a new bladder out of a piece of bowel.

Alternative treatment

There are botanical and homeopathic treatments available for urinary tract infection. None can take the place of correcting a problem that is occurring because of a malformed or dysfunctional organ system. Once correction of the cause is addressed and there is unimpeded flow of urine, adequate fluid intake can contribute to prevention of future infections.

Prognosis

As long as damage to the kidneys from infection or back pressure has not become significant, the surgical repair of troublesome ureteral defects produces excellent long-term results in the great majority of cases. Monitoring for recurrent infections is always a good idea, and occasional checking of kidney function will detect hidden ongoing damage.

Resources

BOOKS

Bauer, Stuart B. "Anomalies of the Kidney and Ureteropelvic Junction." In Campbell's Urology, edited by Patrick C. Walsh, et al. Philadelphia: W. B. Saunders Co., 1998.

KEY TERMS

Congenital— Present at birth.

Contrast agent— A chemical or other substance placed in the body to show structures that would not otherwise be visible on x ray or other imaging studies.

Cystoscopy— Looking into the urinary bladder with a thin telescope-like instrument.

Ectopic— Out of place.

Septicemia— A serious whole body infection spreading through the blood stream.

Ureterovesical valve— A sphincter (an opening controlled by a circular muscle), located where the ureter enters the bladder, that keeps urine from flowing backward toward the kidney.

Urogenital— Both the urinary system and the sexual organs, which form together in the developing embryo.

Congenital ureter anomalies

Definition

The ureter drains urine from the kidney into the bladder. Not simply a tube, the ureter is an active organ that propels urine forward by muscular action. It has a valve at its bottom end that prevents urine from flowing backward into the kidney. Normally there is one ureter on each side of the body for each kidney. However, among the many abnormalities of ureteral development, duplication is quite common. Ureters may also be malformed in a variety of ways—some harmful, others not.

Description

There are many different types of ureter anomalies. Ureters can be duplicated completely or partially, they can be in the wrong place, they can be deformed, and they can end in the wrong place. The trouble these abnormalities bring is directly related to their effect on the flow of urine. As long as urine flows normally through them, and only in one direction, no harm is done. A description of ureter anomalies follows.

Duplication of ureters is quite common, either in part or completely. Kidneys are sometimes duplicated as well. Someone may have four kidneys and four ureters or two kidneys, half of each drained by a separate ureter, or a single kidney with two, three, or four ureters attached. As long as urine can flow easily in the correct direction, such malformations may never be detected. If, however, one of the ureters has a dead end, a stricture or stenosis (narrowing), or a leaky ureterovesical valve (between the ureter and bladder), infection is the likely result.

Stricture or stenosis of a ureter prevents urine from flowing freely. Whenever flow is obstructed in the body—urine, bile, mucus, or any other liquid—infection follows. Ureters can be obstructed anywhere along their course, though the ureterovesical valve is the most common place.

A ureter may have an ectopic (out-of-place) orifice (opening): it may enter the bladder, or even another structure, where it does not belong and therefore lack an adequate valve to control reflux.

The primary ureter, or a duplicate, may not even reach the bladder, but rather terminate in a dead end. Urine will stagnate there and eventually cause infection.

A ureter can be perfectly normal but in the wrong place, such as behind the vena cava (retrocaval ureter), the large vein in the middle of the abdomen. In this case the ureter may be pinched by the vena cava so that flow is hindered. Other abnormal locations may also lead to compression and impaired flow.

Besides infection, urine that backs up causes the ureter and the kidney to expand or dilate. Eventually, the kidney stops functioning because of the back pressure. This condition is called hydronephrosis (a kidney swollen with urine).

Demographics

The urogenital system is more likely than any other organ system to have birth defects, and they can occur in endless variety. Congenital ureter anomalies affect as many as one in every 160 individuals.

Causes and symptoms

In general, the causes of birth defects are multiple and often as of 2004 unknown. Furthermore, the precise cause of specific birth defects has only rarely been identified. Such is the case with congenital ureteral anomalies.

Practically the only symptom generated by ureteral abnormalities is urinary tract infection. A lower tract infection, in the bladder, is called cystitis . In children it may cause fever and systemic symptoms, but in adults it causes only cloudy, burning, and frequent urine. Upper tract infections, by contrast, can be serious for both adults and children, causing high fevers, back pain , severe generalized discomfort, and even leading to kidney failure or septicemia (infection spreading throughout the body by way of the blood stream).

In rare cases, urine from an ectopic ureter will bypass the bladder and dribble out of the bottom somewhere, through a natural orifice like the vagina or a completely separate unnatural opening.

Diagnosis

For children experiencing serious or recurrent urinary tract infections, the pediatrician will search for underlying abnormalities. Cystoscopy (looking into the bladder with a thin telescope-like instrument) and x rays with a contrast agent to illuminate the urinary system will usually identify the defect. Computed tomography scans (CT) and magnetic resonance imaging (MRI) may provide additional information. Urine cultures to identify the infecting germs will be repeated frequently until the problem is corrected.

KEY TERMS

Congenital —Present at birth.

Contrast agent —Also called a contrast medium, this is usually a barium or iodine dye that is injected into the area under investigation. The dye makes the interior body parts more visible on an x-ray film.

Cystoscopy —A diagnostic procedure in which a hollow lighted tube (cystoscope) is used to look inside the bladder and the urethra.

Ectopic —Out of place or located away from the normal position.

Retrocaval ureter —A ureter that is located behind the vena cava blood vessel.

Septicemia —A systemic infection due to the presence of bacteria and their toxins in the bloodstream. Septicemia is sometimes called blood poisoning.

Ureterovesical valve —A sphincter (an opening controlled by a circular muscle), located where the ureter enters the bladder, that keeps urine from flowing backward toward the kidney.

Urogenital —Refers to both the urinary system and the sexual organs, which form together in the developing embryo.

Treatment

Sometimes the recurring infections caused by flow abnormalities can be treated with repeated and changing courses of antibiotics . Over time, the infecting germs develop resistance to most treatments, especially the safer ones. If it can be done safely, it is better to repair the defect surgically. Urologists have various approaches to urine drainage that range from simply reimplanting a ureter into the bladder, in such a way that an effective valve is created, to building a new bladder out of a piece of bowel.

Alternative treatment

There are botanical and homeopathic treatments available for urinary tract infection. None can take the place of correcting a problem that is occurring because of a malformed or dysfunctional organ system. Once correction of the cause is addressed and there is unimpeded flow of urine, adequate fluid intake can contribute to prevention of future infections.

Prognosis

As long as damage to the kidneys from infection or back pressure has not become significant, the surgical repair of troublesome ureteral defects produces excellent long-term results in the great majority of cases. Monitoring for recurrent infections is always a good idea, and occasional checking of kidney function will detect hidden ongoing damage.

Prevention

The cause of congenital ureter anomalies is not known. There is no prevention.

Resources

BOOKS

Colberg, John W. "Urologic Abnormalities of the Genitourinary Tract." Chapter 21, section 16, in Rudolph's Pediatrics, 21st ed. Edited by Colin D. Rudolph and Abraham M. Rudolph. New York: McGraw-Hill, 2003, pp. 1735–39.

Joffre, F., et al. Radiological Imaging of the Ureter. Secaucus, NJ: Springer, 2003.

ORGANIZATIONS

American Association of Clinical Urologists. 1111 N. Plaza Dr., Suite 550, Schaumberg, IL 60173. Web site: <www.aacu.web.org/>.

WEB SITES

"Congenital Ureter Anomalies." Dr. Joseph F. Smith Medical Library. Available online at <www.chclibrary.org/micromed/00043640.html> (accessed December 9, 2004).

"Genitourinary Tract." Pediatrics. Available online at <http://pediatrics.aappublications.org/cgi/collection/gynourinary_tract> (accessed December 9, 2004).

J. Ricker Polsdorfer, MD Deborah L. Nurmi, MS