technetium-tc-99m-disofenin and Cystic-Fibrosis

Gallbladder nonvisualization on hepatobiliary scintigraphy in cystic fibrosis is generally secondary to cystic duct obstruction from inspissated bile, mucus, or gallstones. We report gallbladder nonvisualization on hepatobiliary imaging in two patients with cystic fibrosis who had contracted gallbladders on ultrasonography. Repeat ultrasonography at 6 months revealed persistent gallbladder contraction. A contracted gallbladder therefore is a potential cause of a false-positive hepatobiliary scan which can be treated with conservative management.

Topics: Abdominal Pain; Adult; Biliary Tract; Child; Cystic Fibrosis; False Positive Reactions; Female; Gallbladder; Gallbladder Diseases; Humans; Imino Acids; Liver; Male; Organotechnetium Compounds; Radionuclide Imaging; Technetium Tc 99m Disofenin; Ultrasonography

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.. 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).. 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 (T1/2 > 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.. Quantitative hepatobiliary scintigraphy is a valuable tool in the evaluation and management of the liver disease in this patient population.

Topics: Bile Duct Diseases; Bile Ducts, Intrahepatic; Child; Cystic Fibrosis; Female; Gallbladder; Humans; Imino Acids; Liver; Liver Diseases; Male; Organotechnetium Compounds; Radionuclide Imaging; Technetium Tc 99m Disofenin

Topics: Bile Ducts; Child; Cystic Fibrosis; Humans; Imino Acids; Liver; Liver Diseases; Liver Function Tests; Organotechnetium Compounds; Radionuclide Imaging; Technetium Tc 99m Disofenin

One hundred and four adult patients with cystic fibrosis were evaluated for the presence of liver disease as defined by abnormal liver function tests of six months' duration, histological evidence of fibrosis or cirrhosis, or the presence of portal hypertension, or both. Twenty patients fulfilled these criteria and were evaluated further for the presence of biliary tract abnormalities with biliary scintigraphy using 99Tc diisopropylphenyl-carboxymethyl iminodiacetic acid (DISIDA) and endoscopic retrograde cholangiography. Clearance of 99Tc DISIDA from the liver and biliary tree was diminished at 45 (E45) and 60 (E60) minutes in the patients with liver disease compared with those without liver disease; E45 = 37.8% and 65.8%, p less than 0.01; E60 = 48.2% and 77.5%, p less than 0.01 respectively. Serial analogue images of the extrahepatic biliary tree were consistent with common bile duct obstruction with retention of DISIDA and tapering of the common bile duct in seven of 18 patients with and two of 10 patients without liver disease. Endoscopic retrograde cholangiography showed changes consistent with sclerosing cholangitis, with beading and stricturing of the intrahepatic ducts in 12 of the 14 patients. In all 14 patients, including those in whom biliary scintigraphy had suggested obstruction, no abnormality of the common bile duct was identified. These results indicate that abnormalities of the bile ducts in patients with cystic fibrosis related liver disease are confined to the intrahepatic biliary tree and that common bile duct strictures do not contribute to either the progression or development of liver disease in these patients.

Topics: Adolescent; Adult; Biliary Tract Diseases; Child; Cholangiopancreatography, Endoscopic Retrograde; Cystic Fibrosis; Female; Humans; Hypertension, Portal; Imino Acids; Liver Cirrhosis; Liver Diseases; Male; Organotechnetium Compounds; Radionuclide Imaging; Technetium Tc 99m Disofenin