HEPATOBILIARY SCINTIGRAPHY IN CHILDREN WITH CYSTIC-FIBROSIS AND LIVER-DISEASE

Intra- and extrahepatic impairment of biliary drainage is important in the pathogenesis of liver disease in cystic fibrosis. Distal common b ile duct obstruction is reported to occur in 13% to 96% of these patie nts. Between 1975 and 1993, 17 of 372 children (4.5%) with cystic fibr osis attending The Children's Memorial Medical Center in Chicago had l iver disease based on clinical and laboratory findings. Methods: Hepat obiliary scintigraphy (HBS) with Tc-99m-DISIDA was performed on 12 of the 17 children (mean age at the time of exam was 9 yr, with a range o f 1 mo to 21 yr). Results: All had hepatomegaly, four had splenomegaly and two had bleeding esophageal varices. Twenty HBS exams on these 12 patients documented nonvisualization of the gallbladder in 7, dilated intrahepatic ducts in 6 (only the left lobe was involved in 3 patient s), nonvisualization of bower in two, delayed peaking time in the live r (> 10 min) in four patients, and delayed clearance from the liver pa renchyma (T-1/2 > 20 min) in 11. There appears to be a spectrum of abn ormal HBS findings in cystic fibrosis patients with liver disease. The se are delayed clearance of liver parenchyma, nonvisualization of the gallbladder and dilated intrahepatic ducts with a predilection for the left lobe of the liver. These abnormal findings fluctuate in time and may not correlate with the findings on ultrasonography. Conclusion: Q uantitative hepatobiliary scintigraphy is a valuable tool in the evalu ation and management of the liver disease in this patient population.