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Congenital Cystic Dysplasia of the Kidneys

(Congenital Renal Cystic Dysplasia)

By Navin Jaipaul, MD, MHS, Associate Professor of Medicine, Loma Linda University School of Medicine; Chief, Nephrology, VA Loma Linda Healthcare System

Congenital cystic dysplasia of the kidneys is a broad category of birth defects involving the kidneys and/or urinary tract and that may cause blockage of the flow of urine.

Congenital cystic dysplasia affects one or both kidneys. Cystic dysplasia of the kidneys may be the only birth defect, or it may be part of a syndrome and occur together with other abnormalities. Abnormalities may affect other parts of the urinary tract, including the ureter, bladder, or rarely the urethra, testes, or abdominal wall muscles. (See figure: Viewing the Urinary Tract.)

If enough kidney tissue is damaged, chronic kidney disease (CKD) can result, sometimes causing symptoms, often with slowed growth. Congenital cystic dysplasia of the kidneys is commonly discovered when routine ultrasonography is done before birth or is done during early childhood for a reason unrelated to the kidneys.

Prognosis is highly unpredictable because it may be difficult for doctors to tell how much kidney tissue is unaffected and functions normally.

Treatment is surgical correction if needed for any associated defects of the urinary tract. If chronic kidney disease progresses to end-stage renal disease (end-stage kidney failure), dialysis or kidney transplantation may be needed.