Developmental Abnormalities of the Kidneys

Renal malformations account for 20%-30% of all prenatally diagnosed developmental anomalies and are responsible for 31% of all childhood end-stage renal disease. Patients with unilateral renal agenesis are at risk for hypertension in childhood and chronic kidney disease in adulthood. Multicystic dysplastic kidney typically presents as a collection of large renal cysts on ultrasound, and patients generally do well. Autosomal recessive polycystic kidney disease presents as bilaterally enlarged hyperechoic kidneys, often without cysts, and patients may have significant pulmonary and renal complications. The outcome for patients with renal dysplasia depends significantly on the amount of functioning renal tissue and the associated congenital anomalies. Infant dialysis has improved rapidly over recent years, and 2-year survival outcomes as high as 80% are now reported by multinational dialysis databases.