(Radiographics. 2000;20:767-777.)
© RSNA, 2000
Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances1
Leonard J. King, MRCP, FRCR ,
Erica D. Scurr, DCR, BSc,
Natarajan Murugan, MRCPI ,
Simon G. J. Williams, MD, MRCP,
David Westaby, FRCP and
Jeremiah C. Healy, MRCP, FRCR
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.
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Abstract
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Magnetic resonance (MR) imaging and MR cholangiopancreatography are useful, noninvasive techniques for the assessment of pancreatic and hepatobiliary complications in cystic fibrosis. Abnormalities of the pancreas in cystic fibrosis are typically characterized by fat deposition, which has increased signal intensity on T1-weighted MR images, and pancreatic fibrosis, which has low signal intensity on both T1- and T2-weighted images. Pancreatic cysts are a relatively common finding; these cysts are typically quite small but are well demonstrated at MR imaging and MR cholangiopancreatography. Pancreatic duct abnormalities are also occasionally seen. Hepatic manifestations range from hepatomegaly and diffuse fatty infiltration to severe cirrhosis with fibrotic change, regenerative nodules, and portal hypertension. Splenomegaly is often characterized by siderotic nodules that manifest as multiple focal areas of abnormal low signal intensity within the spleen. Biliary manifestations include cholelithiasis, stricturization, and narrowing or dilatation of intra- and extrahepatic bile ducts. Gallbladder abnormalities including microgallbladder are also readily demonstrated. MR cholangiopancreatography can be used to help determine the presence and severity of biliary complications without resorting to more invasive procedures and, in conjunction with MR imaging, may prove useful in the assessment of patients with cystic fibrosis who present with abdominal symptoms that suggest hepatobiliary involvement.
Index Terms: Bile ducts, diseases, 76.1496, 76.28 • Fibrosis, cystic, 76.1496, 77.1496 • Liver, diseases, 761.1496, 761.371, 761.794 • Pancreas, diseases, 770.1496 • Spleen, diseases, 775.1496, 775.372
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Introduction
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Pancreatic and hepatobiliary disease are common features of cystic fibrosis. Pancreatic disease results in exocrine insufficiency, with clinically apparent dysfunction seen in 85%–90% of patients (1). Endocrine pancreatic insufficiency is less common, but 30%–50% of patients with cystic fibrosis have glucose intolerance and 1%–2% require insulin therapy (1,2). The severity of the pancreatic and hepatobiliary changes has been shown to increase with age (3), and advances in the management of pulmonary complications of cystic fibrosis, along with better treatment of patients with pancreatic exocrine insufficiency, have led to increased survival rates. Consequently, there is an increasing prevalence of chronic liver disease, which is seen in up to 24% of adult patients with cystic fibrosis in clinical series (1,4–6) and up to 72% in postmortem studies (7).
Several studies have described the magnetic resonance (MR) imaging appearances of pancreatic disease in patients with cystic fibrosis (8–12), although hepatobiliary manifestations at MR imaging are less well documented (9).
In this article, we describe and illustrate the MR imaging and MR cholangiopancreatographic appearances of pancreatic and hepatobiliary disease in cystic fibrosis. These findings are based on a review of the literature and on our experience with abdominal MR imaging in more than 40 patients with cystic fibrosis.
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Pancreatic Manifestations
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Abnormalities of the pancreas in cystic fibrosis are typically characterized by fat deposition and pancreatic fibrosis. Fat deposition has increased signal intensity on T1-weighted MR images, whereas pancreatic fibrosis has low signal intensity on both T1- and T2-weighted images. Tham et al (11) described three MR imaging patterns in pancreatic abnormalities: (a) a lobulated, enlarged pancreas with complete fatty replacement and subtle, lower-signal-intensity septations or streaks (Fig 1), (b) a small, atrophic pancreas with partial fatty replacement (Fig 2), and (c) diffuse pancreatic atrophy without fatty replacement (Fig 3). Two additional MR imaging patterns have been described by other authors: complete fatty replacement of the pancreas with loss of lobular contour (Fig 4) (12) and complete pancreatic fibrosis (10).
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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).
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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).
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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).
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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.
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Occasionally, the pancreas may be markedly enlarged in association with fatty replacement. This combination of features has been referred to as lipomatous pseudohypertrophy (13).
Complete fatty replacement is the most common MR imaging finding in pancreatic abnormalities (8–12). This finding correlates well with findings at pathologic analysis of mature adipose tissue separated by fibrous septa and containing isolated clusters of islets of Langerhans and a few residual pancreatic ductules (14). The high-signal-intensity appearance of complete fatty replacement on T1-weighted images correlates relatively well with clinically advanced pancreatic disease (8,10), and a relationship has been demonstrated between reduction in T1 values and the degree of clinical compromise due to exocrine insufficiency (12). However, some cystic fibrosis patients with a normal pancreas at MR imaging have clinically apparent exocrine insufficiency (9,10); consequently, the clinical utility of this observation is limited.
The prevalence of pancreatic fatty replacement in cystic fibrosis varies with age and is most frequently identified in older patients. In an autopsy series of 27 cases of cystic fibrosis that spanned a period of 5 years, diffuse fatty replacement was reported in 15 cases (56%) (14). The mean age of patients with fatty replacement was 17 years, whereas the mean age of patients without fatty replacement was only 11 years. At MR imaging, the reported prevalence of diffuse fatty change varies from 51% to 75% (8–12), with partial fatty replacement seen in 7%–29% of cases and pancreatic atrophy in 27%–35% (9,11,12). Tham et al (11) reported pancreatic enlargement in association with fatty change at MR imaging in nine of 17 adult patients (53%) with cystic fibrosis. Our experience has shown that it can be difficult to distinguish the fat-replaced pancreas from normal retroperitoneal fat, making it difficult to assess the size of the gland. However, diffuse pancreatic enlargement was apparent in five of 34 patients (15%) with cystic fibrosis who had undergone pancreatic MR imaging at our institution. Fatty infiltration of the pancreas can also be readily identified at computed tomography, but this modality involves the use of ionizing radiation. Typical ultrasonographic (US) appearances, including increased parenchymal echogenicity and pancreatic atrophy, have also been described by several authors (3,15–18), although MR imaging is superior to US in demonstrating fatty infiltration (9).
A disadvantage of MR imaging is the inability to reliably demonstrate small calcifications within the pancreas (8), which are also known to occur in patients with cystic fibrosis (19). These calcifications vary in size and shape from very fine punctate concretions to granular or flecked areas of increased opacity and are generally found within dilated pancreatic ducts following ductal and ductular obstruction by calcium-rich inspissated material (19). The reported prevalence of pancreatic calcification varies, with a prevalence of up to 8% reported by Iannaccone and Antonelli (19) in a series of 60 patients with cystic fibrosis who underwent abdominal radiography.
Apparent diffuse pancreatic fibrosis, which has very low signal intensity with all imaging sequences, was reported in three of 18 patients (17%) by Murayama et al (10). To our knowledge, however, this disease entity has not been described at MR imaging by any other authors. Furthermore, it has not been demonstrated in any of our patients with cystic fibrosis.
Pancreatic cysts are a relatively common finding in patients with cystic fibrosis and probably occur secondary to duct obstruction by inspissated secretions (20). The cysts are typically small (1–3 mm) or even microscopic but can reach a diameter of several centimeters (21) and are well demonstrated at MR imaging and MR cholangiopancreatography (Fig 5). Pancreatic cystosis is a rare manifestation of cystic fibrosis in which the pancreas is replaced by epithelium-lined cysts of varying size that may cause mass effect within the abdomen (20).
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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).
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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).
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In our experience, the pancreatic duct is not routinely visualized at MR cholangiopancreatography in patients with cystic fibrosis, which may be due to generalized narrowing. This is consistent with the results of a prospective study by Graham et al (16), in which US demonstrated the pancreatic duct in only 11 of 35 patients (31%) with cystic fibrosis. In cases in which the duct is visualized at MR cholangiopancreatography, abnormalities that are typically identified include duct narrowing, dilatation, stricturization, and beading (Fig 6) as well as cysts related to the pancreatic duct system as described earlier.
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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).
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Hepatobiliary Manifestations
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Recognized features of cystic fibrosis–related hepatobiliary disease include fatty change, focal biliary cirrhosis that may progress to frank liver cirrhosis with portal hypertension, abnormalities of intra- and extrahepatic bile ducts, thickening or contraction of the gallbladder, and gallstones (4,6,21–24).
Clinical examination and liver biochemical analysis are known to have a low sensitivity and specificity for diagnosing cystic fibrosis–related hepatobiliary disease (6,25), and liver biopsy can be unreliable due to sampling errors caused by the focal distribution of cirrhotic change (6,22,26). A US scoring system for assessing the presence and severity of liver disease in patients with cystic fibrosis has been proposed (5) and is probably more accurate than clinical assessment. Our initial experience suggests that MR imaging is at least as good as US in determining the presence and severity of cystic fibrosis–related liver disease, although this has not yet been established with a blinded, prospective study.
The Table shows the MR imaging findings in a series of 20 consecutive patients with cystic fibrosis aged 11–51 years (mean, 28 years) with abdominal symptoms that raised suspicion for hepatobiliary disease. Several patients were already known to have clinically apparent hepatobiliary disease. All imaging was performed with a 1.0-T Magnetom Impact Expert imager (Siemens, Erlangen, Germany). MR imaging was performed with T1-weighted FLASH (160/6.6, 75° flip angle, one signal acquired, 129 x 256 matrix, 350-mm field of view, 5-mm section thickness) and T2-weighted turbo spin-echo (3,300/138, echo train length of 29, one signal acquired, 116 x 256 matrix, 350-mm field of view, 5-mm section thickness) sequences. MR cholangiopancreatography was performed with axial and coronal single-breath-hold T2-weighted half-Fourier rapid acquisition with relaxation enhancement (RARE) sequences (10.92/87, one signal acquired, 240 x 256 fixed matrix, 350–400 mm field of view, 5-mm section thickness) with maximum intensity projection. A coronal half-Fourier RARE single slab (2,800/1,100, one signal acquired, 240 x 256 matrix, 260-mm field of view, 60-mm thickness) was also obtained. Satisfactory images were obtained with these fast-breath-hold sequences in all cases despite severe breathlessness in several patients due to respiratory complications of cystic fibrosis, including bronchiolitis obliterans following lung transplantation. Cirrhotic change with fibrosis, regenerative nodules (Fig 7a), and features of portal hypertension (Fig 8) were clearly demonstrated in eight of 20 patients (40%). Minor liver abnormalities such as fatty change were apparent in an additional five cases (Fig 9).
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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).
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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).
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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).
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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).
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Increased periportal echogenicity at US has been described by several authors (3,5,15,16,27) and is thought to correspond to findings at pathologic analysis of bile duct proliferation, inflammatory changes, edema, or fibrosis (3,28). In our series, however, low-signal-intensity periportal fibrotic tissue was an uncommon finding at MR imaging, being demonstrated in only two patients with cirrhosis and extensive generalized fibrosis (Fig 7b). A more common finding at T1-weighted MR imaging was high-signal-intensity periportal tissue (Fig 10), which is suppressed with fat-saturated sequences and indicates fat deposition. This may be explained by findings at pathologic analysis of cells containing fat globules in the fibrotic portal tracts and Ito cells containing large, fat-laden vacuoles in the periportal areas (28).
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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.
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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.
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Five of the 10 patients with splenomegaly at MR imaging demonstrated multiple focal areas of abnormal low signal intensity within the spleen (Fig 7a), a finding that is typical of siderotic nodules (29,30). These nodules are referred to as Gamna-Gandy bodies and represent organized foci of hemorrhage caused by portal hypertension. These nodules typically have low signal intensity on T1-, T2-, and proton-density–weighted images and are most frequently demonstrated with gradient-echo sequences due to magnetic susceptibility effects (29,30).
Biliary duct abnormalities have been described in patients with cystic fibrosis at endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography, and scintigraphy (4,22–24,31). Typical appearances at endoscopic retrograde cholangiopancreatography range from minor irregularity and tapering of the smaller intrahepatic biliary radicles to stricturization, beading, and segmental dilatation of the large bile ducts (4,24,31). Abnormalities of the extrahepatic biliary system have also been described, although there has been some debate regarding the presence and significance of distal common bile duct strictures (4,22–24). Gaskin et al (22) reported bile duct strictures at scintigraphy in 31 of 45 patients (69%) with cystic fibrosis with clinical features of liver disease. These findings were confirmed with percutaneous transhepatic cholangiography in 18 cases; however, they were not supported by findings in a study by Nagel et al (4), who identified common bile duct strictures in only two of 15 patients (13%) with cystic fibrosis with hepatic dysfunction, or by those in a study by O'Brien et al (24), who found no evidence of bile duct strictures at endoscopic retrograde cholangiopancreatography in seven patients in whom scintigraphy had suggested a stricture.
In our series, 13 of 20 patients (65%) demonstrated abnormalities of the intra- or extrahepatic ducts at MR cholangiopancreatography, which patients underwent following a 4-hour fast. Typical appearances included stricturization, beading, narrowing, or dilatation of the intrahepatic ducts; diffuse narrowing or focal stricturization of the common bile duct; and calculi in the intra- and extrahepatic bile ducts (Figs 11–14).
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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).
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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).
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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.
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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.
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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).
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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).
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Gallbladder abnormalities are also readily demonstrated at MR imaging or MR cholangiopancreatography. In our series, gallbladder stones and a shrunken or microgallbladder (Fig 15) were apparent in 10 of 20 patients (50%), although it has been suggested that US may be more accurate in the diagnosis of gallbladder abnormalities (9). Microgallbladders containing gelatinous material or mucus have been described at autopsy in 23% of patients with cystic fibrosis (32) and can result in false-positive results at hepatobiliary scintigraphy (33). Atresia or stenosis of the cystic duct may result from inspissated mucus or mucosal hyperplasia, leading to gallbladder atrophy, although small gallbladders have also been described in patients with a patent cystic duct (32).
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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.
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In some cases, the MR cholangiopancreatographic features of biliary disease are similar to those described in sclerosing cholangitis (34), including the presence of intrahepatic duct calculi. Although they are not typically seen in sclerosing cholangitis, intrahepatic duct calculi were identified in 14 of 184 cases (7.6%) in a retrospective study by Dodd et al (35). MR cholangiopancreatographic findings correlated well with findings at endoscopic retrograde cholangiopancreatography in the six patients who underwent both procedures at our institution (Fig 13).
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Conclusions
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MR imaging is a useful, noninvasive technique for the assessment of pancreatic and hepatobiliary complications in cystic fibrosis. A variety of related pancreatic and hepatic abnormalities can be clearly demonstrated with MR imaging. However, a prospective study is still required to determine whether MR imaging is significantly better than US in assessing hepatobiliary complications and guiding further management. MR cholangiopancreatography can be used to help determine the presence and severity of biliary complications and distinguish between intrahepatic and extrahepatic manifestations in patients with cystic fibrosis without resorting to more invasive procedures such as endoscopic retrograde cholangiopancreatography. In conjunction with standard abdominal MR imaging, MR cholangiopancreatography may prove useful in the assessment of patients with cystic fibrosis who present with abdominal symptoms that suggest hepatobiliary involvement (eg, right upper quadrant pain), particularly if US findings are inconclusive. Repeat examinations can also be performed to assess disease progression or response to therapy without radiation exposure, the risks associated with endoscopic retrograde cholangiopancreatography, or the difficulties encountered in comparing findings in serial US examinations performed by different observers.
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Footnotes
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Abbreviations: FLASH = fast low-angle shot,
RARE = rapid acquisition with relaxation enhancement
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Abstract
Introduction
