DOI: 10.1148/rg.266065013
Polycystic Liver Disease: Multimodality Imaging for Complications and Transplant Evaluation1
Desiree E. Morgan, MD,
Mark E. Lockhart, MD, MPH,
Cheri L. Canon, MD,
M. Paul Holcombe, MD and
J. Stephenson Bynon, MD
1 From the Departments of Radiology (D.E.M., M.E.L., C.L.C.) and Surgery (J.S.B.), University of Alabama at Birmingham, JTN322, 619 S 19th St, Birmingham, AL 35233; and the Baptist Health Center, Birmingham, Alabama (M.P.H.). Recipient of a Certificate of Merit award for an education exhibit at the 2005 RSNA Annual Meeting. Received February 3, 2006; revision requested March 6 and received April 11; accepted April 13. All authors have no financial relationships to disclose.
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Figure 1a. PLD in an asymptomatic 44-year-old woman undergoing screening for ADPKD. Coronal gadolinium-enhanced gradient-echo T1-weighted magnetic resonance (MR) image (a) and half Fourier acquisition single-shot turbo spin-echo T2-weighted MR image (b) demonstrate cysts of varying size throughout all segments of the liver.
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Figure 1b. PLD in an asymptomatic 44-year-old woman undergoing screening for ADPKD. Coronal gadolinium-enhanced gradient-echo T1-weighted magnetic resonance (MR) image (a) and half Fourier acquisition single-shot turbo spin-echo T2-weighted MR image (b) demonstrate cysts of varying size throughout all segments of the liver.
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Figure 2a. Symptomatic PLD and ADPKD. (a) Computed tomographic (CT) scan obtained with orally administered contrast material demonstrates polycystic liver and kidney disease, with multiple calcifications along the cyst wall in the left kidney. (b) CT scan obtained inferior to a reveals mass effect on the hepatic flexure of the colon (arrow). Many patients with PLD have renal insufficiency due to ADPKD. Mass effect can be well demonstrated at un-enhanced CT; however, vascular anatomy and patency and biliary dilatation are not well depicted without intravenous contrast material in these patients.
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Figure 2b. Symptomatic PLD and ADPKD. (a) Computed tomographic (CT) scan obtained with orally administered contrast material demonstrates polycystic liver and kidney disease, with multiple calcifications along the cyst wall in the left kidney. (b) CT scan obtained inferior to a reveals mass effect on the hepatic flexure of the colon (arrow). Many patients with PLD have renal insufficiency due to ADPKD. Mass effect can be well demonstrated at un-enhanced CT; however, vascular anatomy and patency and biliary dilatation are not well depicted without intravenous contrast material in these patients.
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Figure 3a. PLD in a 47-year-old woman with massive abdominal distention due to hepatomegaly. (a, b) Frontal (a) and lateral (b) CT scanograms demonstrate a markedly protuberant abdomen. (c) Unenhanced CT scan demonstrates marked displacement of the stomach posteriorly (arrow). (d) Intravenous contrast material–enhanced CT scan obtained at the same level as c clearly depicts the right hepatic artery (arrow) replaced to the superior mesenteric artery. The patient suffered from early satiety and progressive immobility and required liver transplantation despite essentially normal liver function tests. The explanted liver weighed 10,190 g and measured 42 x 40 x 20 cm. Of note, the patient had a normal creatinine level. Three of her siblings had already undergone kidney transplantation for ADPKD, but she was the only sibling with PLD.
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Figure 3b. PLD in a 47-year-old woman with massive abdominal distention due to hepatomegaly. (a, b) Frontal (a) and lateral (b) CT scanograms demonstrate a markedly protuberant abdomen. (c) Unenhanced CT scan demonstrates marked displacement of the stomach posteriorly (arrow). (d) Intravenous contrast material–enhanced CT scan obtained at the same level as c clearly depicts the right hepatic artery (arrow) replaced to the superior mesenteric artery. The patient suffered from early satiety and progressive immobility and required liver transplantation despite essentially normal liver function tests. The explanted liver weighed 10,190 g and measured 42 x 40 x 20 cm. Of note, the patient had a normal creatinine level. Three of her siblings had already undergone kidney transplantation for ADPKD, but she was the only sibling with PLD.
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Figure 3c. PLD in a 47-year-old woman with massive abdominal distention due to hepatomegaly. (a, b) Frontal (a) and lateral (b) CT scanograms demonstrate a markedly protuberant abdomen. (c) Unenhanced CT scan demonstrates marked displacement of the stomach posteriorly (arrow). (d) Intravenous contrast material–enhanced CT scan obtained at the same level as c clearly depicts the right hepatic artery (arrow) replaced to the superior mesenteric artery. The patient suffered from early satiety and progressive immobility and required liver transplantation despite essentially normal liver function tests. The explanted liver weighed 10,190 g and measured 42 x 40 x 20 cm. Of note, the patient had a normal creatinine level. Three of her siblings had already undergone kidney transplantation for ADPKD, but she was the only sibling with PLD.
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Figure 3d. PLD in a 47-year-old woman with massive abdominal distention due to hepatomegaly. (a, b) Frontal (a) and lateral (b) CT scanograms demonstrate a markedly protuberant abdomen. (c) Unenhanced CT scan demonstrates marked displacement of the stomach posteriorly (arrow). (d) Intravenous contrast material–enhanced CT scan obtained at the same level as c clearly depicts the right hepatic artery (arrow) replaced to the superior mesenteric artery. The patient suffered from early satiety and progressive immobility and required liver transplantation despite essentially normal liver function tests. The explanted liver weighed 10,190 g and measured 42 x 40 x 20 cm. Of note, the patient had a normal creatinine level. Three of her siblings had already undergone kidney transplantation for ADPKD, but she was the only sibling with PLD.
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Figure 4a. PLD associated with ADPKD in a 56-year-old woman. The patient underwent combined liver transplantation, kidney transplantation, and right nephrectomy. (a) Unenhanced CT scan shows nearly complete replacement of the hepatic parenchyma by cysts. The explanted liver weighed 8285 g. (b) Photograph of the sectioned specimen shows sparing of a small portion of the right lobe (arrow), a finding that corresponds to the island of parenchyma seen at CT.
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Figure 4b. PLD associated with ADPKD in a 56-year-old woman. The patient underwent combined liver transplantation, kidney transplantation, and right nephrectomy. (a) Unenhanced CT scan shows nearly complete replacement of the hepatic parenchyma by cysts. The explanted liver weighed 8285 g. (b) Photograph of the sectioned specimen shows sparing of a small portion of the right lobe (arrow), a finding that corresponds to the island of parenchyma seen at CT.
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Figure 5. PLD in a 58-year-old man undergoing peritoneal dialysis for end-stage ADPKD. Intravenous contrast–enhanced CT scan through the hepatic hilum demonstrates enhancement of the right and left portal veins. There are multiple small peribiliary cysts (arrows) with a "string of pearls" distribution along the portal vessels. A few intrahepatic cysts are also present. No definite biliary dilatation is seen.
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Figure 6a. ADPKD in a 63-year-old man who presented with increasing upper abdominal pain and limited mobility ("inability to tie his shoes"). The patient had undergone left nephrectomy and kidney transplantation in 1987. (a) Coronal gradient-echo T1-weighted MR image shows typical low-signal-intensity fluid within multiple small cysts throughout the liver. High-signal-intensity fluid is seen within the dominant cyst (arrow), which involves the posterior right lobe superiorly. (b) Axial fast spin-echo T2-weighted MR image shows scattered intrahepatic cysts, all with similar high signal intensity. (c) Axial gradient-echo T1-weighted MR image obtained at the same level as b once again demonstrates high signal intensity of the dominant cyst (arrow), a finding that indicates possible complication from infection or hemorrhage. The patient was symptomatic but was a poor surgical candidate due to cardiac disease. On the basis of the imaging findings, temporizing percutaneous aspiration of the dominant cyst was selected as the best therapeutic option.
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Figure 6b. ADPKD in a 63-year-old man who presented with increasing upper abdominal pain and limited mobility ("inability to tie his shoes"). The patient had undergone left nephrectomy and kidney transplantation in 1987. (a) Coronal gradient-echo T1-weighted MR image shows typical low-signal-intensity fluid within multiple small cysts throughout the liver. High-signal-intensity fluid is seen within the dominant cyst (arrow), which involves the posterior right lobe superiorly. (b) Axial fast spin-echo T2-weighted MR image shows scattered intrahepatic cysts, all with similar high signal intensity. (c) Axial gradient-echo T1-weighted MR image obtained at the same level as b once again demonstrates high signal intensity of the dominant cyst (arrow), a finding that indicates possible complication from infection or hemorrhage. The patient was symptomatic but was a poor surgical candidate due to cardiac disease. On the basis of the imaging findings, temporizing percutaneous aspiration of the dominant cyst was selected as the best therapeutic option.
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Figure 6c. ADPKD in a 63-year-old man who presented with increasing upper abdominal pain and limited mobility ("inability to tie his shoes"). The patient had undergone left nephrectomy and kidney transplantation in 1987. (a) Coronal gradient-echo T1-weighted MR image shows typical low-signal-intensity fluid within multiple small cysts throughout the liver. High-signal-intensity fluid is seen within the dominant cyst (arrow), which involves the posterior right lobe superiorly. (b) Axial fast spin-echo T2-weighted MR image shows scattered intrahepatic cysts, all with similar high signal intensity. (c) Axial gradient-echo T1-weighted MR image obtained at the same level as b once again demonstrates high signal intensity of the dominant cyst (arrow), a finding that indicates possible complication from infection or hemorrhage. The patient was symptomatic but was a poor surgical candidate due to cardiac disease. On the basis of the imaging findings, temporizing percutaneous aspiration of the dominant cyst was selected as the best therapeutic option.
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Figure 7a. Hepatic cysts in a 61-year-old man with symptomatic liver disease and laboratory test results indicating biliary obstruction. (a) Intravenous contrast–enhanced CT scan through the upper liver obtained in 2003 demonstrates complete replacement of the lateral segment of the left hepatic lobe by innumerable small cysts. (b) Contrast-enhanced CT scan through the upper liver obtained in 2005 demonstrates similar findings in the left hepatic lobe and new intrahepatic duct dilatation (arrow). Islands of normal parenchyma are seen in the right lobe. (c) Contrast-enhanced CT scan through the inferior right hepatic lobe demonstrates a very large cyst located along the surface. This dominant cyst appears amenable to fenestration. The small cysts in the lateral segment and the deep portion of the right lobe are not as amenable. On the basis of imaging findings, the patient underwent partial resection of the left lateral segment and anterior right lobe (nonsegmental resection plane), along with fenestration of the dominant cyst.
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Figure 7b. Hepatic cysts in a 61-year-old man with symptomatic liver disease and laboratory test results indicating biliary obstruction. (a) Intravenous contrast–enhanced CT scan through the upper liver obtained in 2003 demonstrates complete replacement of the lateral segment of the left hepatic lobe by innumerable small cysts. (b) Contrast-enhanced CT scan through the upper liver obtained in 2005 demonstrates similar findings in the left hepatic lobe and new intrahepatic duct dilatation (arrow). Islands of normal parenchyma are seen in the right lobe. (c) Contrast-enhanced CT scan through the inferior right hepatic lobe demonstrates a very large cyst located along the surface. This dominant cyst appears amenable to fenestration. The small cysts in the lateral segment and the deep portion of the right lobe are not as amenable. On the basis of imaging findings, the patient underwent partial resection of the left lateral segment and anterior right lobe (nonsegmental resection plane), along with fenestration of the dominant cyst.
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Figure 7c. Hepatic cysts in a 61-year-old man with symptomatic liver disease and laboratory test results indicating biliary obstruction. (a) Intravenous contrast–enhanced CT scan through the upper liver obtained in 2003 demonstrates complete replacement of the lateral segment of the left hepatic lobe by innumerable small cysts. (b) Contrast-enhanced CT scan through the upper liver obtained in 2005 demonstrates similar findings in the left hepatic lobe and new intrahepatic duct dilatation (arrow). Islands of normal parenchyma are seen in the right lobe. (c) Contrast-enhanced CT scan through the inferior right hepatic lobe demonstrates a very large cyst located along the surface. This dominant cyst appears amenable to fenestration. The small cysts in the lateral segment and the deep portion of the right lobe are not as amenable. On the basis of imaging findings, the patient underwent partial resection of the left lateral segment and anterior right lobe (nonsegmental resection plane), along with fenestration of the dominant cyst.
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Figure 8a. PLD and venous thrombosis. (a) Intravenous contrast–enhanced CT scan through the liver demonstrates marked intrahepatic compression of the inferior vena cava (arrow) in the caudate lobe. This compression resulted in stasis. (b) CT scan obtained inferior to a shows a nonocclusive thrombus (arrow) within the infrahepatic inferior vena cava. (c) Intravenous contrast–enhanced CT scan reveals thrombus (arrow) in the lateral segmental portal vein branches. (d) CT scan obtained at the hilum shows patent main and right portal veins without thrombus. The explanted liver weighed 4850 g and was 90% replaced by cysts. At surgery, multiple cysts had to be ruptured to mobilize the liver and remove it from the peritoneal cavity. The portal vein was elongated due to distortion by the cysts and had to be shortened to avoid kinking at the portal anastomosis.
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Figure 8b. PLD and venous thrombosis. (a) Intravenous contrast–enhanced CT scan through the liver demonstrates marked intrahepatic compression of the inferior vena cava (arrow) in the caudate lobe. This compression resulted in stasis. (b) CT scan obtained inferior to a shows a nonocclusive thrombus (arrow) within the infrahepatic inferior vena cava. (c) Intravenous contrast–enhanced CT scan reveals thrombus (arrow) in the lateral segmental portal vein branches. (d) CT scan obtained at the hilum shows patent main and right portal veins without thrombus. The explanted liver weighed 4850 g and was 90% replaced by cysts. At surgery, multiple cysts had to be ruptured to mobilize the liver and remove it from the peritoneal cavity. The portal vein was elongated due to distortion by the cysts and had to be shortened to avoid kinking at the portal anastomosis.
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Figure 8c. PLD and venous thrombosis. (a) Intravenous contrast–enhanced CT scan through the liver demonstrates marked intrahepatic compression of the inferior vena cava (arrow) in the caudate lobe. This compression resulted in stasis. (b) CT scan obtained inferior to a shows a nonocclusive thrombus (arrow) within the infrahepatic inferior vena cava. (c) Intravenous contrast–enhanced CT scan reveals thrombus (arrow) in the lateral segmental portal vein branches. (d) CT scan obtained at the hilum shows patent main and right portal veins without thrombus. The explanted liver weighed 4850 g and was 90% replaced by cysts. At surgery, multiple cysts had to be ruptured to mobilize the liver and remove it from the peritoneal cavity. The portal vein was elongated due to distortion by the cysts and had to be shortened to avoid kinking at the portal anastomosis.
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Figure 8d. PLD and venous thrombosis. (a) Intravenous contrast–enhanced CT scan through the liver demonstrates marked intrahepatic compression of the inferior vena cava (arrow) in the caudate lobe. This compression resulted in stasis. (b) CT scan obtained inferior to a shows a nonocclusive thrombus (arrow) within the infrahepatic inferior vena cava. (c) Intravenous contrast–enhanced CT scan reveals thrombus (arrow) in the lateral segmental portal vein branches. (d) CT scan obtained at the hilum shows patent main and right portal veins without thrombus. The explanted liver weighed 4850 g and was 90% replaced by cysts. At surgery, multiple cysts had to be ruptured to mobilize the liver and remove it from the peritoneal cavity. The portal vein was elongated due to distortion by the cysts and had to be shortened to avoid kinking at the portal anastomosis.
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Figure 9a. Portal hypertension associated with PLD. (a) Longitudinal US image through the right hepatic lobe demonstrates multiple cysts of varying size and a moderate amount of ascites. (b) Longitudinal US image of the spleen demonstrates moderate splenomegaly.
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Figure 9b. Portal hypertension associated with PLD. (a) Longitudinal US image through the right hepatic lobe demonstrates multiple cysts of varying size and a moderate amount of ascites. (b) Longitudinal US image of the spleen demonstrates moderate splenomegaly.
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Figure 10a. Liver transplantation for PLD. (a) Intraoperative photograph demonstrates a protuberant abdominal contour. (b) On an intraoperative photograph taken after transverse abdominal incision, the markedly enlarged polycystic liver is exposed. To mobilize and remove the liver, many of the cysts had to be ruptured. (c) Photograph shows the explanted liver, which is much smaller than it was in situ prior to cyst ruptures.
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Figure 10b. Liver transplantation for PLD. (a) Intraoperative photograph demonstrates a protuberant abdominal contour. (b) On an intraoperative photograph taken after transverse abdominal incision, the markedly enlarged polycystic liver is exposed. To mobilize and remove the liver, many of the cysts had to be ruptured. (c) Photograph shows the explanted liver, which is much smaller than it was in situ prior to cyst ruptures.
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Figure 10c. Liver transplantation for PLD. (a) Intraoperative photograph demonstrates a protuberant abdominal contour. (b) On an intraoperative photograph taken after transverse abdominal incision, the markedly enlarged polycystic liver is exposed. To mobilize and remove the liver, many of the cysts had to be ruptured. (c) Photograph shows the explanted liver, which is much smaller than it was in situ prior to cyst ruptures.
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Figure 11a. Ascites and bile leaks after partial nonsegmental resection and cyst fenestration in the same patient as in Figure 7. (a) Intravenous contrast–enhanced CT scan obtained after removal of the majority of the lateral segment demonstrates postsurgical changes. A small amount of biliary air is present, and ascites is seen surrounding the spleen. (b) CT scan obtained inferior to a shows that portions of the anterior segment of the right hepatic lobe have been removed, and the dominant cyst has been opened. Soft tissue in the anterior aspect of the cyst (arrows) could represent redundant cyst wall or clot. (c) Spot radiograph obtained during endoscopic retrograde cholangiography demonstrates opacification of the intrahepatic system, which is splayed around the remaining cysts. Extravasation of contrast material (arrow) from the left intrahepatic ducts is seen near the surgical resection margin. (d) Magnified spot radiograph obtained during endoscopic retrograde cholangiography demonstrates extravasation of contrast material (arrow) from the right intrahepatic ducts in the anterior segment region near the surgical drain.
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Figure 11b. Ascites and bile leaks after partial nonsegmental resection and cyst fenestration in the same patient as in Figure 7. (a) Intravenous contrast–enhanced CT scan obtained after removal of the majority of the lateral segment demonstrates postsurgical changes. A small amount of biliary air is present, and ascites is seen surrounding the spleen. (b) CT scan obtained inferior to a shows that portions of the anterior segment of the right hepatic lobe have been removed, and the dominant cyst has been opened. Soft tissue in the anterior aspect of the cyst (arrows) could represent redundant cyst wall or clot. (c) Spot radiograph obtained during endoscopic retrograde cholangiography demonstrates opacification of the intrahepatic system, which is splayed around the remaining cysts. Extravasation of contrast material (arrow) from the left intrahepatic ducts is seen near the surgical resection margin. (d) Magnified spot radiograph obtained during endoscopic retrograde cholangiography demonstrates extravasation of contrast material (arrow) from the right intrahepatic ducts in the anterior segment region near the surgical drain.
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Figure 11c. Ascites and bile leaks after partial nonsegmental resection and cyst fenestration in the same patient as in Figure 7. (a) Intravenous contrast–enhanced CT scan obtained after removal of the majority of the lateral segment demonstrates postsurgical changes. A small amount of biliary air is present, and ascites is seen surrounding the spleen. (b) CT scan obtained inferior to a shows that portions of the anterior segment of the right hepatic lobe have been removed, and the dominant cyst has been opened. Soft tissue in the anterior aspect of the cyst (arrows) could represent redundant cyst wall or clot. (c) Spot radiograph obtained during endoscopic retrograde cholangiography demonstrates opacification of the intrahepatic system, which is splayed around the remaining cysts. Extravasation of contrast material (arrow) from the left intrahepatic ducts is seen near the surgical resection margin. (d) Magnified spot radiograph obtained during endoscopic retrograde cholangiography demonstrates extravasation of contrast material (arrow) from the right intrahepatic ducts in the anterior segment region near the surgical drain.
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Figure 11d. Ascites and bile leaks after partial nonsegmental resection and cyst fenestration in the same patient as in Figure 7. (a) Intravenous contrast–enhanced CT scan obtained after removal of the majority of the lateral segment demonstrates postsurgical changes. A small amount of biliary air is present, and ascites is seen surrounding the spleen. (b) CT scan obtained inferior to a shows that portions of the anterior segment of the right hepatic lobe have been removed, and the dominant cyst has been opened. Soft tissue in the anterior aspect of the cyst (arrows) could represent redundant cyst wall or clot. (c) Spot radiograph obtained during endoscopic retrograde cholangiography demonstrates opacification of the intrahepatic system, which is splayed around the remaining cysts. Extravasation of contrast material (arrow) from the left intrahepatic ducts is seen near the surgical resection margin. (d) Magnified spot radiograph obtained during endoscopic retrograde cholangiography demonstrates extravasation of contrast material (arrow) from the right intrahepatic ducts in the anterior segment region near the surgical drain.
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Figure 12a. Massive hepatomegaly resulting in a redundant right hemidiaphragm. (a) Unenhanced CT scan through the midportion of the liver demonstrates innumerable cysts of varying size, with partial sparing of the periphery of segment IVa. The explanted liver was so large that the patient had to undergo reduction of the right hemidiaphragm at the time of transplantation because the donor liver kept falling into the chest during surgery. Arrow indicates the orientation of the celiac axis prior to transplantation. (b) Postoperative unenhanced CT scan through the midportion of the donor liver demonstrates a normal graft without complication. Splenomegaly is much more apparent now that the massively enlarged liver has been removed. Note the change in orientation of the celiac axis (arrow) after removal of the polycystic liver.
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Figure 12b. Massive hepatomegaly resulting in a redundant right hemidiaphragm. (a) Unenhanced CT scan through the midportion of the liver demonstrates innumerable cysts of varying size, with partial sparing of the periphery of segment IVa. The explanted liver was so large that the patient had to undergo reduction of the right hemidiaphragm at the time of transplantation because the donor liver kept falling into the chest during surgery. Arrow indicates the orientation of the celiac axis prior to transplantation. (b) Postoperative unenhanced CT scan through the midportion of the donor liver demonstrates a normal graft without complication. Splenomegaly is much more apparent now that the massively enlarged liver has been removed. Note the change in orientation of the celiac axis (arrow) after removal of the polycystic liver.
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Copyright © 2006 by the Radiological Society of North America.
