The Value of Radionuclide Studies in Children with Autosomal Recessive Polycystic Kidney Disease

Abstract

To describe and analyze the appearances of autosomal recessive polycystic kidney disease (ARPKD) on Tc-99m DMSA and Tc-99m HIDA scintigraphy. The authors evaluated scintigraphic findings for 13 boys and 9 girls (age range, 2 months to 22.75 years; mean, 7.5 years) with ARPKD. Fourteen children underwent Tc-99m DMSA and 20 underwent Tc-99m HIDA scintigraphy according to European guidelines. Kidney outline, internal structure, tracer uptake, and differential function were analyzed on Tc-99m DMSA images, whereas relative liver lobe sizes, hepatocyte tracer uptake, time to peak, and excretion into the biliary tree and gut were evaluated on Tc-99m HIDA scans. On Tc-99m DMSA images, loss of kidney outline and internal structure was seen in 75% of the scans, and patchy tracer uptake with focal defects throughout the kidneys, particularly at the poles, was evident in 93%. In 85% of the cases, the Tc-99m DMSA changes did not correlate with the ultrasonographic findings where the kidneys are uniformly affected. Characteristic findings on Tc-99m HIDA scans were enlarged left liver lobe in 80%, a delay in maximal hepatocyte uptake in 68%, delayed tracer excretion into the biliary tree in 32% (with stasis in the prominent intrahepatic biliary ducts in 50% or pooling into the segmentally dilated biliary ducts in 25%), and delayed excretion into the gut in 40% of patients. In a child with clinically enlarged kidneys that appear diffusely hyperechoic on ultrasound, the appearances on Tc-99m DMSA imaging strongly support the diagnosis of ARPKD. The Tc-99m HIDA findings, especially of an enlarged left lobe of the liver with bile stasis or dilatation, further support the diagnosis.