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Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances¹

¹ From the Departments of Radiology (L.J.K., E.D.S., J.C.H.) and Gastroenterology (N.M., S.G.J.W., D.W.), Chelsea and Westminster Hospital, London SW10 9NH, England. Presented as a scientific exhibit at the 1998 RSNA scientific assembly. Received April 15, 1999; revision requested May 11 and received July 14; accepted July 15. Address reprint requests to J.C.H.

View larger version (159K): [in a new window]   Figure 1.   Pancreatic enlargement and complete fatty replacement in a 41-year-old man with cystic fibrosis. Axial T1-weighted fast low-angle shot (FLASH) (repetition time msec/echo time msec  = 160/6.6; flip angle, 75°) MR image demonstrates enlargement and complete fatty replacement of the pancreas without loss of lobular contour. Subtle low-signal-intensity septations are also seen (arrows).

View larger version (106K): [in a new window]   Figure 2.   Pancreatic atrophy and partial fatty replacement in a 31-year-old man with cystic fibrosis. Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows diffuse pancreatic atrophy and partial fatty replacement (arrows).

View larger version (156K): [in a new window]   Figure 3.   Pancreatic atrophy in a 26-year-old man with cystic fibrosis, chronic liver disease, and pancreatic exocrine insufficiency. Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows diffuse pancreatic atrophy without fatty replacement (arrows).

View larger version (153K): [in a new window]   Figure 4.   Pancreatic enlargement and fatty infiltration in a 39-year-old woman with cystic fibrosis and pancreatic exocrine insufficiency. Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows diffuse enlargement and fatty infiltration of the pancreas with loss of lobular contour.

View larger version (137K): [in a new window]   Figure 5a.   Pancreatic cyst in a 32-year-old man with cystic fibrosis. Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image of the pancreas (a) and T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram (b) show a solitary cyst in the pancreatic head (arrows).

View larger version (141K): [in a new window]   Figure 5b.   Pancreatic cyst in a 32-year-old man with cystic fibrosis. Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image of the pancreas (a) and T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram (b) show a solitary cyst in the pancreatic head (arrows).

View larger version (120K): [in a new window]   Figure 6.   Pancreatic duct dilatation and beading in a 37-year-old man with cystic fibrosis, severe hepatobiliary disease, and diffuse fatty infiltration of the pancreas. T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram demonstrates dilatation and beading of the pancreatic duct within the uncinate process (arrows).

View larger version (123K): [in a new window]   Figure 7a.   Regenerative nodules and extensive generalized fibrosis in a 27-year-old man with cystic fibrosis, liver cirrhosis, and portal hypertension. (a) Axial T1-weighted fat-saturated FLASH (160/6.6; flip angle, 75°) MR image shows multiple regenerative nodules separated by bands of fibrosis (black arrows). The spleen is enlarged and contains multiple low-signal-intensity siderotic nodules (white arrows). (b) Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows extension of low-signal-intensity fibrosis around the portal structures (arrows).

View larger version (149K): [in a new window]   Figure 7b.   Regenerative nodules and extensive generalized fibrosis in a 27-year-old man with cystic fibrosis, liver cirrhosis, and portal hypertension. (a) Axial T1-weighted fat-saturated FLASH (160/6.6; flip angle, 75°) MR image shows multiple regenerative nodules separated by bands of fibrosis (black arrows). The spleen is enlarged and contains multiple low-signal-intensity siderotic nodules (white arrows). (b) Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows extension of low-signal-intensity fibrosis around the portal structures (arrows).

View larger version (142K): [in a new window]   Figure 8.   Varices in a 20-year-old man with cystic fibrosis, cirrhosis, portal hypertension, and splenomegaly. Coronal T2-weighted turbo spin-echo (3,300/138; flip angle, 180°) MR image shows multiple serpiginous flow voids around the splenic hilum due to varices (arrows).

View larger version (138K): [in a new window]   Figure 9.   Fatty change in the liver in a 29-year-old woman with cystic fibrosis. Axial T1-weighted fat-saturated FLASH (160/6.6; flip angle, 75°) MR image demonstrates decreased signal intensity in the right lobe. Focal sparing is seen in the left lobe (arrows).

View larger version (142K): [in a new window]   Figure 10a.   Periportal fat deposition in a 31-year-old man with cystic fibrosis and chronic liver disease. (a) US image shows increased echogenicity around the portal structures (arrows), a finding that was thought to represent periportal fibrosis. (b) Corresponding T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows high signal intensity around the portal structures (arrows), a finding that is consistent with fat deposition. The left lobe of the liver is also enlarged, and there are multiple low-signal-intensity bands of linear fibrosis. Fat deposition was confirmed with a fat-saturated sequence.

View larger version (129K): [in a new window]   Figure 10b.   Periportal fat deposition in a 31-year-old man with cystic fibrosis and chronic liver disease. (a) US image shows increased echogenicity around the portal structures (arrows), a finding that was thought to represent periportal fibrosis. (b) Corresponding T1-weighted FLASH (160/6.6; flip angle, 75°) MR image shows high signal intensity around the portal structures (arrows), a finding that is consistent with fat deposition. The left lobe of the liver is also enlarged, and there are multiple low-signal-intensity bands of linear fibrosis. Fat deposition was confirmed with a fat-saturated sequence.

View larger version (110K): [in a new window]   Figure 11.   Intrahepatic biliary duct disease in a 29-year-old woman with cystic fibrosis. T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram shows irregularity and beading of the intrahepatic ducts and multiple small filling defects due to intrahepatic gallstones (arrows).

View larger version (128K): [in a new window]   Figure 12.   Intrahepatic duct disease in a 33-year-old man with cystic fibrosis, an elevated serum alkaline phosphatase level, and no evidence of biliary obstruction at US. T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram demonstrates intrahepatic biliary duct narrowing and irregularity with a "pruned tree" appearance (arrows).

View larger version (106K): [in a new window]   Figure 13a.   Gallstones and focal stricturization of the common bile duct in a 51-year-old man with cystic fibrosis. (a) T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram demonstrates multiple filling defects in the common bile duct (solid arrows) along with a focal distal stricture (open arrows). (b) Endoscopic retrograde cholangiopancreatogram helps confirm the presence of gallstones within the common bile duct and the focal distal stricture.

View larger version (103K): [in a new window]   Figure 13b.   Gallstones and focal stricturization of the common bile duct in a 51-year-old man with cystic fibrosis. (a) T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram demonstrates multiple filling defects in the common bile duct (solid arrows) along with a focal distal stricture (open arrows). (b) Endoscopic retrograde cholangiopancreatogram helps confirm the presence of gallstones within the common bile duct and the focal distal stricture.

View larger version (138K): [in a new window]   Figure 14a.   Intrahepatic duct dilatation and irregularity in a 37-year-old man with cystic fibrosis and cholelithiasis. (a) T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram shows dilatation and irregularity of the left intrahepatic ducts (open arrows) with at least one filling defect (solid arrow). (b) Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image clearly demonstrates the dilated left intrahepatic ducts (arrowheads).

View larger version (133K): [in a new window]   Figure 14b.   Intrahepatic duct dilatation and irregularity in a 37-year-old man with cystic fibrosis and cholelithiasis. (a) T2-weighted half-Fourier RARE (2,800/1,100) MR cholangiopancreatogram shows dilatation and irregularity of the left intrahepatic ducts (open arrows) with at least one filling defect (solid arrow). (b) Axial T1-weighted FLASH (160/6.6; flip angle, 75°) MR image clearly demonstrates the dilated left intrahepatic ducts (arrowheads).

View larger version (163K): [in a new window]   Figure 15.   Microgallbladder in a 15-year-old girl with cystic fibrosis. Axial T2-weighted half-Fourier RARE (10.92/87) MR cholangiopancreatogram demonstrates a small, intrahepatic gallbladder (arrow) with no evidence of cholelithiasis.