Congenital Malformations of the Urinary Tract

Children can be born with kidney and urinary tract anomalies called congenital malformations of the urinary tract. This means the organs are abnormally developed — there are some distortions. These malformations can affect kidney and urinary tract function, and they are the most common cause of chronic kidney disease, according to the American Academy of Pediatrics. The urinary tract includes the kidneys, the ureters and the bladder.

Common Congenital Malformations of the Urinary Tract

Common congenital kidney anomalies include:

  • Kidneys that are fused together (horseshoe kidney)
  • Polycystic kidney disease, where one or both kidneys are filled with cysts (polycystic or multicystic kidney disease)
  • A baby is born with one or no kidneys (renal agenesis)
  • A baby is born with 1 or 2 abnormally small kidneys (renal hypoplasia)
  • One or both kidneys has not formed as they should (renal dysplasia)

Symptoms of Congenital Malformations of the Kidneys and Urinary Tracts

These birth defects are often detected in a prenatal ultrasound, but they can go undetected before birth. Children may show symptoms such as:

  • Frequent urinary tract infections (UTIs)
  • Persistent fatigue
  • Loss of appetite
  • Nausea and vomiting
  • Stunted growth
  • Edema/swelling of the belly, hands, feet, or face
  • Unexplained fever

Diagnosis and Testing

Congenital malformations of the kidney and urinary tract can usually be diagnosed by ultrasound during pregnancy. If a malformation is identified during ultrasound, then additional prenatal ultrasounds might be needed to determine the extent of the malformation.

After birth, other tests can identify the effect of the malformation on renal function. Your doctor may order additional scans of the kidneys and urinary tract, including ultrasounds, CT scans, or MRIs. Blood tests or urine tests may be needed to measure how well the kidney and urinary tract are functioning after birth. If the malformation affects the urinary tract, then a voiding cystourethrogram (VCUG) can can show the flow of urine through the bladder. This is a type of scan that uses x-rays and contrast dye to visualize urinary flow through the renal system.

Some congenital malformations are asymptomatic at birth but cause symptoms later as your child grows. If your child has a malformation that become symptomatic in childhood or later, then other types of tests might be necessary. These could include blood tests for glomerular filtration rate (GFR) and electrolyte balance, which both indicate how well the kidney is functioning. Depending on the type of malformation, a kidney biopsy could also be necessary.


Our pediatric nephrologists will work with you and your child to create a customized treatment plan, depending on their anomaly and overall health.

Treatment may include:

  • Antibiotics to prevent urinary tract infections
  • Observation
  • Surgery to address any blockages.

It’s important to treat congenital abnormalities promptly because they can lead to chronic kidney disease or kidney failure.

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Cooperman Barnabas Medical Center
94 Old Short Hills Road
Livingston, NJ 07039
(973) 322-5000
Children's Hospital of New Jersey at Newark Beth Israel Medical Center
201 Lyons Avenue at Osborne Terrace
Newark, NJ 07112
(973) 926-7000
The Bristol-Myers Squibb Children's Hospital at RWJUH
200 Somerset Street
New Brunswick, NJ 08901
(732) 828-3000
The Unterberg Children's Hospital at Monmouth Medical Center
300 2nd Avenue
Long Branch, NJ 07740
(732) 923-7250

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