Children's Hospital Colorado

Congenital Anomalies of the Kidney and Urinary Tract (CAKUT)

What is CAKUT?

CAKUT stands for "congenital anomalies of the kidney and urinary tract." Congenital means that the condition exists at birth. The acronym describes a group of anomalies (abnormalities) affecting the kidneys and other parts of the urinary tract, which consist of the ureter, bladder and urethra. The ureters are tubes that carry urine from the kidneys to the bladder and the urethra is the tube that carries the urine from the bladder to outside of the body.

Common conditions that fall under the CAKUT group include:

What causes CAKUT?

CAKUT is caused by a combination of environmental and genetic factors that affect the formation and development of the kidneys and urinary tract prenatally (during pregnancy).

Who gets CAKUT?

CAKUT is one of the most common groups of anomalies diagnosed in newborns with about 45 infants affected in every 10,000 births. CAKUT is more common in boys and more often occurs in children born to mothers with gestational diabetes mellitus. CAKUT accounts for about 35% of cases of end-stage kidney disease in children, also called end-stage renal disease (ESRD), that require kidney transplantation.

What are the signs and symptoms of CAKUT?

Some children may have urinary tract infections, difficulty urinating or difficulty with potty training. Symptoms of urinary tract infections in infants and young children include:

While these may be the signs and symptoms of CAKUT once a child is born, the diagnosis is usually made prenatally, based on prenatal imaging using an ultrasound.

What tests are used to diagnose CAKUT?

Once born, your child's doctor will use more detailed images to determine how the kidney and urinary tract are affected. This will aid them in giving a more accurate diagnosis.

Ultrasounds of the kidney and bladder can show your child's doctors how they are formed and if there is a blockage. In addition to an ultrasound and computed tomography (CT) scans, these types of urologic diagnostic tests can provide information on the urinary tract and show how well the kidneys and bladder drain: bladder scans, voiding cystourethrogram (VCUG), lasix renograms and uroflow. If your child has kidney disease, blood tests will help your child's doctor to diagnose the stage of disease.

How is CAKUT treated?

Treatment for children with CAKUT depends on the severity of the condition. Mild forms of the disease can result in recurring urinary tract infections or a blockage of urine, which can damage the kidneys or other structures. A severe case of CAKUT can result in kidney failure and ESRD.

Treatment for children with CAKUT may include working on ways to empty their urine and stool, which is called "voiding." Working on these voiding habits can include using different positions when going to the bathroom, taking a daily medication or catheterizing the urinary tract to help with voiding of urine. Treatment may also include surgery on parts of the urinary tract.

Ongoing laboratory and imaging follow-up with the CAKUT team is an important component of the treatment plan. More severe cases of CAKUT resulting in poor kidney function may eventually require dialysis or kidney transplantation. Should dialysis be necessary, our providers will determine the best type of dialysis for each individual child. Dialysis is typically a bridge to the ultimate goal of successful kidney transplantation.

Why choose us for CAKUT treatment?

A multidisciplinary approach is the most effective way to treat children with CAKUT. At Children's Hospital Colorado, we offer a CAKUT Clinic that utilizes our Urologic Tumor Program, Pediatric Kidney Center, Pediatric General Surgery, as well as dietitians, social workers and psychologists. Using this approach, we can provide comprehensive care for children with varying levels of CAKUT.

Our nephrologists (kidney doctors) are also very experienced at treating the various stages of kidney disease associated with severe forms of CAKUT. For those who reach ESRD and require a kidney transplant, our kidney transplant team has some of the best outcomes in the nation for pediatric kidney transplantation.

When we make a prenatal diagnosis for CAKUT, which is often the case, our team meets with the parents to discuss options and explain what they should expect. These prenatal consultations help to prepare you if you are expecting a child with CAKUT and we can continue to provide this comprehensive care throughout the stages of childhood and into early adulthood.

Our team approach not only allows us to care for children with potential complex diagnoses, but tests such as the point-of-care ultrasounds, ambulatory blood pressure monitoring, uroflow and urodynamics also improve our ability to provide the best consultation and outcomes for your child.