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Hepatobiliary Scintigraphy in Children with Cystic Fibrosis and Liver Disease

Divisions of Nuclear Medicine and Gastroenterology, The Children's Memorial Medical Center, Chicago, Illinois

Correspondence: For correspondence or reprints: James J. Conway, MD, The Children's Memorial Hospital, Division of Nuclear Medicine #42, 2300 Children's Plaza, Chicago, IL 60614-3394.

ABSTRACT

Intra- and extrahepatic impairment of biliary drainage is important in the pathogenesis of liver disease in cystic fibrosis. Distal common bile duct obstruction is reported to occur in 13% to 96% of these patients. Between 1975 and 1993, 17 of 372 children (4.5%) with cystic fibrosis attending The Children's Memorial Medical Center in Chicago had liver disease based on clinical and laboratory findings. Methods: Hepatobiliary scintigraphy (HBS) with ^99mTc-DISIDA was performed on 12 of the 17 children (mean age at the time of exam was 9 yr, with a range of 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 patients), nonvisualization of bowel in two, delayed peaking time in the liver (>10 min) in four patients, and delayed clearance from the liver parenchyma (T_1/2 > 20 min) in 11. There appears to be a spectrum of abnormal HBS findings in cystic fibrosis patients with liver disease. These 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: Quantitative hepatobiliary scintigraphy is a valuable tool in the evaluation and management of the liver disease in this patient population.

Key Words: hepatobiliary scintigraphy • ^99mTc-DISIDA • cystic fibrosis