Congenital renal cystic dysplasia is a broad category of congenital malformations involving metanephric malformation or congenital obstructive uropathies.

Congenital renal cystic dysplasia affects one or both kidneys. Renal cystic dysplasia may be an isolated congenital anomaly, or it may be part of a malformation syndrome (ie, associated with other clinical features—see Table 1: Cystic Kidney Disease: Major Groups of Cystic Nephropathies). Associated urologic abnormalities may include ureteropelvic and ureterovesicular junction obstruction, neurogenic bladder, ureterocele, posterior urethral valves, and prune-belly syndrome (a triad of abdominal wall muscle defects, urinary tract abnormalities [eg, dilated ureters, enlarged bladder and urethra], and bilateral cryptorchidism—see Congenital Renal and Genitourinary Anomalies: Prune-Belly Syndrome).

Symptoms and signs vary by how much renal parenchyma is preserved and whether involvement is unilateral or bilateral. Some degree of renal insufficiency or renal failure may develop. Congenital renal cystic dysplasia is commonly discovered by ultrasonography prenatally or during early childhood.

Prognosis is highly unpredictable due to an inability to quantify residual functional parenchyma. Treatment is surgical correction of any associated GU abnormalities and, if renal insufficiency or renal failure is present, renal replacement therapy.

Last full review/revision July 2009 by Drew C. Cutler, MD

Content last modified February 2012