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Congenital Cystic Dysplasia of the Kidneys

(Congenital Renal Cystic Dysplasia)

By

Enrica Fung

, MD, MPH, Loma Linda University School of Medicine

Last full review/revision Apr 2021| Content last modified Apr 2021

Congenital cystic dysplasia of the kidneys is a broad category of birth defects involving the kidneys and/or urinary tract 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 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 (ESRD, or end-stage kidney failure), dialysis or kidney transplantation may be needed.

More Information

The following are some English-language resources that may be useful. Please note that THE MANUAL is not responsible for the content of these resources.

  • American Kidney Fund (AKF): Information about kidney disease, kidney transplant, and needs-based financial assistance to help manage medical expenses
  • National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): General information on kidney diseases, including research discoveries, statistics, and community health and outreach programs
  • National Kidney Foundation (NKF): Information on everything from the basics of kidney function to access to treatment and support for people with kidney disease

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