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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
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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 Major Groups of Cystic Nephropathies Major Groups of Cystic Nephropathies Cystic kidney disease may be congenital or acquired. Congenital disorders may be inherited as autosomal dominant disorders or autosomal recessive disorders or have other causes (eg, sporadic... read more ). Associated urologic abnormalities may include ureteropelvic and ureterovesicular junction obstruction, neurogenic bladder, Neurogenic Bladder Neurogenic bladder is bladder dysfunction (flaccid or spastic) caused by neurologic damage. Symptoms can include overflow incontinence, frequency, urgency, urge incontinence, and retention.... read more ureterocele, posterior urethral valves, and prune-belly syndrome Prune-Belly Syndrome Prune-belly syndrome consists of abdominal muscle deficiency, urinary tract abnormalities, and intra-abdominal undescended testes. The name prune-belly syndrome derives from the characteristic... read more Prune-Belly Syndrome (a triad of abdominal wall muscle defects, urinary tract abnormalities [eg, dilated ureters, enlarged bladder and urethra], and bilateral cryptorchidism).

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 Chronic Kidney Disease Chronic kidney disease (CKD) is long-standing, progressive deterioration of renal function. Symptoms develop slowly and in advanced stages include anorexia, nausea, vomiting, stomatitis, dysgeusia... read more Chronic Kidney Disease 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 genitourinary abnormalities and, if renal insufficiency or renal failure is present, renal replacement therapy Overview of Renal Replacement Therapy Renal replacement therapy (RRT) replaces nonendocrine kidney function in patients with renal failure and is occasionally used for some forms of poisoning. Techniques include continuous hemofiltration... read more .

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