Autosomal Recessive Polycystic Kidney Disease

  • Etiology: microcystic disease occurring in the distal convoluted tubules and collecting ducts
  • Imaging: bilateral enlarged / echogenic / nonfunctioning kidneys, may also have hepatic fibrosis and intra + extrahepatic biliary dilation with there being an inverse relationship between hepatic + renal involvement, individual renal microcyts may be difficult to resolve
  • Clinical: may present with oligohydramnios, pulmonary hypoplasia, respiratory failure

Cases of Autosomal Recessive Polycystic Kidney Disease

US of autosomal recessive polycystic kidney disease
Transverse and sagittal US images of the right kidney shows it to be enlarged and echogenic. The left kidney was identical in appearance.