From the archives of the AFIP - Autosomal recessive polycystic kidney disease: Radiologic-pathologic correlation

Autosomal recessive polycystic kidney disease is a heritable but phenotypic ally variable disorder characterized by varying degrees of nonobstructive r enal collecting duct ectasia, hepatic biliary duct ectasia and malformation , and fibrosis of both liver and kidneys. In the kidney, the dilated collec ting ducts and interstitial fibrosis, when severe, may significantly impair renal function and result in hypertension and renal failure. Imaging typic ally shows large but reniform kidneys, diffusely increased renal parenchyma l echogenicity at ultrasonography and a, striated nephrogram after contrast material administration. In the liver, periportal fibrosis accompanies the malformed and dilated bile ducts; this may result in portal hypertension. The liver may appear normal or may show intrahepatic biliary dilatation; on ce portal hypertension develops, splenomegaly and varices are usually evide nt. The relative degrees of kidney and liver involvement tend to be inverse : Children with severe renal disease usually have milder hepatic disease, a nd those with severe hepatic disease tend to evidence mild renal impairment . Presently, treatment consists of supportive management and control of hyp ertension. Replacement therapy for renal failure (dialysis or kidney transp lantation) and control of portal hypertension (portal circulatory diversion or liver transplantation) may be necessary.