Cystic diseases of the kidney

Abstract Cystic disease of the kidney is quite common. The spectrum includes genetic, dysplastic, and acquired conditions. Autosomal recessive polycystic renal disease (ARPKD) and multicystic dysplastic kidney (MCDK) are the most clinically significant in childhood, although it is important to be aware of the less common problems of simple renal cyst and multilocular renal cyst (cystic nephroma). ARPKD is life-threatening and affected children need multidisciplinary specialized care. MCDK is now generally diagnosed by antenatal ultrasonography and the vast majority are managed conservatively, awaiting spontaneous involution. Simple renal cysts are usually asymptomatic, but can grow and cause pain and occasionally may need surgical management. Multilocular renal cyst must be differentiated from cystic Wilms tumour; however, the former is non-malignant and can be managed by nephrectomy.