Body Computed Tomography
Diagnosis of Caroli Disease by Computed Tomography: Report of Two Cases1 Jay A. Kaiser, M.D., Jay C. Mall, M.D., Brian J. Salmen, M.D., and Jacob J. Parker, M.D. Two patients are discussed in whom Caroli disease was diagnosed with the aid of computed tomography (CT). One patient had the pure form of the disease, characterized primarily by saccular dilatation of the intrahepatic bile ducts and clinically manifesting as recurrent cholangitis. The second patient had a form characterized primarily by hepatic fibrosis and clinically manifesting as portal hypertension. These are the first patients studied with CT to be reported to the authors' knowledge; CT is recommended as the optimal noninvasive method to evaluate such patients. INDEX TERMS: Abdomen, abnormalities. Bile ducts, diseases. Computed tomography, abdomen, 7[0].1211 • (Liver, biliary system, other abnormality of biliary ducts, 7[6] .288) • (Liver, biliary system, CT, 7[6] .1211) Radiology 132:661-664, September 1979
ficult and is usually established either at surgery with operative cholangiography or at autopsy. To our knowledge, no cases have thus far been reported in which computed tomography (CT) was performed. This report demonstrates the usefulness of CT in establishing the diagnosis preoperatively.
OMMUNICATING cavernous ectasia of the biliary tract,
C
also known as Caroli disease, is a rare congenital abnormality and is almost invariably associated with cystic lesions of the kidneys (1-3). Since the disease was first described in 1958, there have been more than 50 cases reported. However, the diagnosis remains extremely dif-
Fig. 1. CASE I. a. CT scan through the liver after intravenous administration of contrast material demonstrates multiple branching, low-density, tubular structures which extend to the periphery and communicate with localized ectatic areas. b. CT scan 3 em cephalad to Figure 1a demonstrates a large, irregular, fluid-filled cystic area containing a mass of inhomogeneous material of higher density. This represents debris and/or noncalcified stones. C. After intravenous administration of contrast material, a CT scan through the kidneys demonstratesmultiple cortical and medullary cysts.
1 From the Department of Radiology, Ross General Hospital, Ross, CA, and the Department of Radiology, University of California School of jr Medicine, San Francisco, CA. Received Jan. 5, 1979; accepted and revision requested April 10; received May 25.
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Fig. 2. CASE I. Tomogram from an excretory urogram demonstrates deformed calyces and "brush-like" collections of contrast material in the renal pyramids consistent with renal tubular ectasia. The renal contours are lobulated.
CASE REPORTS CASE I: A 40-year-old man presented with a two-year history of recurrent right-upper-quadrant pain and low-grade fever. One year prior to admission, an oral cholecystogram and intravenous cholangiogram were normal, although the intrahepatic biliary radicles were not demonstrated. His history was otherwise unremarkable. Except for an elevated alkaline phosphatase, liver function tests were normal. A radionuclide liver scan showed a 4-cm filling defect in the right lobe, and a CT scan (Fig. 1)demonstrated extensive, branching, low-density areas throughout both lobes of the liver, consistent with dilated biliary ducts. Irregular cystic areas appeared attached to these structures, and one such large area was demonstrated in the right lobe, corresponding to the defect in the radionuclide scan. Within this area, punctate regions of higher density were demonstrated, which were believed to represent solid material, either noncalcified stones or debris. Stones were present in the distal common duct. Multiple small cortical and medullary cysts were seen in the kidneys (Fig. 1c). An excretory urogram (Fig. 2) was consistent with renal tubular ectasia, and a percutaneous transhepatic cholangiogram (Fig. 3) confirmed the diagnosis of Caroli disease. The patient is now being followed medically.
Fig. 3. CASE I. Percutaneous transhepatic cholangiogram demonstrates dilated intrahepatic biliary radicles as well as a large cystic area in the right lobe (black arrow) with filling defects. Radiolucent stones are present in the distal common duct (curved arrow). The gallbladder is also filled (arrowhead).
CASE II: This 27-year-old woman had been followed for 10 years with a history of mild chronic renal failure and hepatosplenomegaly of unknown etiology. She was thought to have "polycystic kidney disease" on the basis of retrograde pyelography and urography performed at the time of first presentation. Her history was negative for hepatitis or exposure to hepatotoxic agents or anesthetics. The present admission was for recurrent hematemesis secondary to esophageal varices. A CT scan (Fig. 4) demonstrated a normal liver, but revealed ascites and splenomegaly with enlargement of the portal vein. The kidneys were not enlarged, and multiple small cysts were present at the corticomedullary junctions. An excretory urogram demonstrated findings consistent with medullary sponge kidney (Fig. 5). A mesocaval shunt and
DIAGNOSIS OF CAROL! DISEASE BY CT
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Body Computed Tomography
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Fig. 4. CASE II. a. CT scan following intravenous administration of contrast material demonstrates a normal appearing liver. The spleen (8) is markedly enlarged, and the portal vein (arrow) is very prominent and probably dilated. Ascites can be seen around the liver and spleen. b. CT scan through the kidneys following intravenous administration of contrast material demonstrates multiple small, punctate, low-density areas at the corticomedullary junctions. The kidneys are small with irregular cortical margins. Ascites and splenomegaly are again demonstrated. splenectomy were performed, and a wedge biopsy of the liver demonstrated portal fibrosis with marked proliferation of moderately dilated small bile canaliculi in the portal areas. The hepatic parenchymal cells were normal. The pathologic diagnosis was congenital hepatic fibrosis.
as in CASE I, the clinical manifestations are due to biliary stone formation, cholangitis, and occasionally liver abscess. The usual presenting symptoms are repeated ept-
DISCUSSION
Caroli disease comprises a spectrum of abnormalities
(4-6) of which the 2 cases presented represent the extremes. CASE I demonstrates the pure form of the disease characterized by: (a) saccular dilatation of the intrahepatic bile ducts; (b) high incidence of stone formation and cholangitis; and (c) absence of cirrhosis and portal hypertension. CASE II fulfills the clinical and histological criteria of the opposite end of the spectrum which is characterized by: (a) proliferation of small dilated terminal intrahepatic canaliculi with minimal if any dilatation of the large intrahepatic ducts; (b) cirrhosis and portal hypertension; and (c) absence of stone formation and cholangitis. Some patients demonstrate an intermediate form of the disease. Renal tubular abnormalities are associated with all forms (2, 4, 7, 8). The extent of involvement may range from benign tubular ectasia where a small percentage of tubules are affected, to severe and generalized tubular ectasia resulting in so-called medullary sponge kidney. Associated cysts of variable size are sometimes present in the medulla and corticomedullary junction as well as in the cortex. As has been previously noted, this abnormality is thought to be inherited as an autosomal recessive trait, as opposed to polycystic liver and kidney disease which is inherited as an autosomal dominant (4, 6). The clinical manifestations of Caroli disease are most commonly recognized in children or young adults, but are occasionally seen as late as the fifth decade. The specific presentation and SUbsequent manifestations depend on whether the predominant lesion is a saccular dilatation of the intrahepatic ducts or hepatic fibrosis. If it is the former,
Fig. 5. CASE II. View of the left kidney from an excretory urogram demonstrates deformed calyces with irregular collections of contrast material in the renal pyramids consistent with medullary sponge kidney.
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sodes of crampy upper abdominal pain with occasional fever and jaundice. If the predominant lesion is hepatic fibrosis, as in CASE II, the manifestations are those of portal hypertension, and the usual presenting symptom is hematemesls. The associated renal abnormalities are usually asymptomatic. Radiologic evaluation has usually been of little help in establishing the diagnosis. In those cases in which intrahepatic biliary duct dilatation was the predominant manifestation, intravenous cholangiography has on rare occasions been helpful by the demonstration of faint, irregular, patchy collections of contrast material throughout the liver (5, 9). In general, radionuclide liver scanning is not helpful but may be abnormal in some cases where intrahepatic masses or patchy uptake can be identified. The 131 1rose bengal liver scan has been reported to be helpful in that it may demonstrate a decreased rate of bile drainage (5). Percutaneous transhepatic cholangiography is very specific in demonstrating pathological changes but is, of course, invasive. Selective hepatic angiography may demonstrate multiple avascular masses and is therefore of little value in the differential diagnosis. Excretory urography frequently demonstrates cortical cysts or renal tubular ectasia which may suggest the diagnosis; however, these renal lesions are usually found as isolated abnormalities unrelated to Caroli disease. CT scanning is an ideal technique for evaluating and establishing the diagnosis of Caroli disease. In fact, in his most recent review article, Caroli himself suggested that this would seem to be the best method, although no cases studied by CT had yet been reported (6).CT scanning is especially valuable because it may be used to assess both organ systems involved with a single noninvasive examination. When dilatation of the intrahepatic biliary ducts is the predominant feature, CT scanning can make the diagnosis with essentially 100% certainty. The findings are easily distinguished from polycystic or multiple cystic involvement of the liver because of the clear demonstration of the irregular cystic lesions communicating with the dilated biliary ducts, In polycystic liver disease the cysts are
September 1979
round and smooth and may deform but do not communicate with the biliary ducts. In simple biliary obstruction, the most dilated ducts are central, with tapering to the periphery; they do not show focal ectatic areas. When congenital hepatic fibrosis is the dominant manifestation, the diagnosis may be suggested by demonstration of a normal or slightly enlarged liver of normal density and splenomegaly with dilated splenic and portal veins in association with renal cystic disease. ACKNOWLEDGMENTS: We would like to thank Dr. Arthur Zacks for providingthe percutaneous transhepaticcholangiogramon CASE I. Also, we are indebtedto Mrs. OdetteBrinton and Mrs. Valerie Nelson for their invaluable technical assistance.
Department of Radiology Ross General Hospital 1150 Sir Francis Drake Blvd. Ross, CA 94957
REFERENCES 1. Caroli J. Soupault R. Kossakowski J, et al: La dilatation polykystique conqenltate des voles biliaires intra-hepatiques: essal de classification. Sem Hop Paris 34: 128-135. Feb 1958 2. Martin EE. Corcos V, Albano 0: La dilatation conqenltale des voles biliaires lntra-hepatiques segmentaires. Presse Med 73: 2565-2570, Oct 1965 3. Mujahed Z. Glenn F. Evans JA: Communicating cavernous ectasia of the intrahepatic ducts (Caroli's disease). Am J Roentgenol 113:21-26. Sep 1971 4. Foulk WT: Congenital malformations of the intrahepatic biliary tree in the adult. Editorial. Gastroenterology 58:253-256. Feb
1970 5. Mall JC. Ghahremani 00. Boyer JL: Caroli's disease associated with congenital hepatic fibrosis and renal tubular ectasia. A case report. Gastroenterology 66:1029-1035. May 1974 6. Caroli J: La maladie de Caroli. Med et Hyg 36:29-35. Jan
1978 7. Reilly BJ. Neuhauser EBD: Renal tubular ectasia in cystic disease of the kidneys and liver. Am J Roentgenol 84:546-554. Sep
1960 8. Six R. Oliphant M. Grossman H: A spectrum of renal tubular ectasia and hepatic fibrosis. Radiology 117:117-122. Oct 1975 9. Caroli J. Corcos V: La dilatation congllOitale des voies biliaires intrahepatiques. Rev Medicochir Mal Foie 39:1-70. Feb 1964
Diagnosis of Caroli Disease by Computed Tomography: Report of Two Cases1 Jay A. Kaiser, M.D., Jay C. Mall, M.D., Brian J. Salmen, M.D., and Jacob J. Parker, M.D. Two patients are discussed in whom Caroli disease was diagnosed with the aid of computed tomography (CT). One patient had the pure form of the disease, characterized primarily by saccular dilatation of the intrahepatic bile ducts and clinically manifesting as recurrent cholangitis. The second patient had a form characterized primarily by hepatic fibrosis and clinically manifesting as portal hypertension. These are the first patients studied with CT to be reported to the authors' knowledge; CT is recommended as the optimal noninvasive method to evaluate such patients. INDEX TERMS: Abdomen, abnormalities. Bile ducts, diseases. Computed tomography, abdomen, 7[0].1211 • (Liver, biliary system, other abnormality of biliary ducts, 7[6] .288) • (Liver, biliary system, CT, 7[6] .1211) Radiology 132:661-664, September 1979
ficult and is usually established either at surgery with operative cholangiography or at autopsy. To our knowledge, no cases have thus far been reported in which computed tomography (CT) was performed. This report demonstrates the usefulness of CT in establishing the diagnosis preoperatively.
OMMUNICATING cavernous ectasia of the biliary tract,
C
also known as Caroli disease, is a rare congenital abnormality and is almost invariably associated with cystic lesions of the kidneys (1-3). Since the disease was first described in 1958, there have been more than 50 cases reported. However, the diagnosis remains extremely dif-
Fig. 1. CASE I. a. CT scan through the liver after intravenous administration of contrast material demonstrates multiple branching, low-density, tubular structures which extend to the periphery and communicate with localized ectatic areas. b. CT scan 3 em cephalad to Figure 1a demonstrates a large, irregular, fluid-filled cystic area containing a mass of inhomogeneous material of higher density. This represents debris and/or noncalcified stones. C. After intravenous administration of contrast material, a CT scan through the kidneys demonstratesmultiple cortical and medullary cysts.
1 From the Department of Radiology, Ross General Hospital, Ross, CA, and the Department of Radiology, University of California School of jr Medicine, San Francisco, CA. Received Jan. 5, 1979; accepted and revision requested April 10; received May 25.
661
662
JAY
A.
September 1979
KAISER AND OTHERS
Fig. 2. CASE I. Tomogram from an excretory urogram demonstrates deformed calyces and "brush-like" collections of contrast material in the renal pyramids consistent with renal tubular ectasia. The renal contours are lobulated.
CASE REPORTS CASE I: A 40-year-old man presented with a two-year history of recurrent right-upper-quadrant pain and low-grade fever. One year prior to admission, an oral cholecystogram and intravenous cholangiogram were normal, although the intrahepatic biliary radicles were not demonstrated. His history was otherwise unremarkable. Except for an elevated alkaline phosphatase, liver function tests were normal. A radionuclide liver scan showed a 4-cm filling defect in the right lobe, and a CT scan (Fig. 1)demonstrated extensive, branching, low-density areas throughout both lobes of the liver, consistent with dilated biliary ducts. Irregular cystic areas appeared attached to these structures, and one such large area was demonstrated in the right lobe, corresponding to the defect in the radionuclide scan. Within this area, punctate regions of higher density were demonstrated, which were believed to represent solid material, either noncalcified stones or debris. Stones were present in the distal common duct. Multiple small cortical and medullary cysts were seen in the kidneys (Fig. 1c). An excretory urogram (Fig. 2) was consistent with renal tubular ectasia, and a percutaneous transhepatic cholangiogram (Fig. 3) confirmed the diagnosis of Caroli disease. The patient is now being followed medically.
Fig. 3. CASE I. Percutaneous transhepatic cholangiogram demonstrates dilated intrahepatic biliary radicles as well as a large cystic area in the right lobe (black arrow) with filling defects. Radiolucent stones are present in the distal common duct (curved arrow). The gallbladder is also filled (arrowhead).
CASE II: This 27-year-old woman had been followed for 10 years with a history of mild chronic renal failure and hepatosplenomegaly of unknown etiology. She was thought to have "polycystic kidney disease" on the basis of retrograde pyelography and urography performed at the time of first presentation. Her history was negative for hepatitis or exposure to hepatotoxic agents or anesthetics. The present admission was for recurrent hematemesis secondary to esophageal varices. A CT scan (Fig. 4) demonstrated a normal liver, but revealed ascites and splenomegaly with enlargement of the portal vein. The kidneys were not enlarged, and multiple small cysts were present at the corticomedullary junctions. An excretory urogram demonstrated findings consistent with medullary sponge kidney (Fig. 5). A mesocaval shunt and
DIAGNOSIS OF CAROL! DISEASE BY CT
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Body Computed Tomography
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Fig. 4. CASE II. a. CT scan following intravenous administration of contrast material demonstrates a normal appearing liver. The spleen (8) is markedly enlarged, and the portal vein (arrow) is very prominent and probably dilated. Ascites can be seen around the liver and spleen. b. CT scan through the kidneys following intravenous administration of contrast material demonstrates multiple small, punctate, low-density areas at the corticomedullary junctions. The kidneys are small with irregular cortical margins. Ascites and splenomegaly are again demonstrated. splenectomy were performed, and a wedge biopsy of the liver demonstrated portal fibrosis with marked proliferation of moderately dilated small bile canaliculi in the portal areas. The hepatic parenchymal cells were normal. The pathologic diagnosis was congenital hepatic fibrosis.
as in CASE I, the clinical manifestations are due to biliary stone formation, cholangitis, and occasionally liver abscess. The usual presenting symptoms are repeated ept-
DISCUSSION
Caroli disease comprises a spectrum of abnormalities
(4-6) of which the 2 cases presented represent the extremes. CASE I demonstrates the pure form of the disease characterized by: (a) saccular dilatation of the intrahepatic bile ducts; (b) high incidence of stone formation and cholangitis; and (c) absence of cirrhosis and portal hypertension. CASE II fulfills the clinical and histological criteria of the opposite end of the spectrum which is characterized by: (a) proliferation of small dilated terminal intrahepatic canaliculi with minimal if any dilatation of the large intrahepatic ducts; (b) cirrhosis and portal hypertension; and (c) absence of stone formation and cholangitis. Some patients demonstrate an intermediate form of the disease. Renal tubular abnormalities are associated with all forms (2, 4, 7, 8). The extent of involvement may range from benign tubular ectasia where a small percentage of tubules are affected, to severe and generalized tubular ectasia resulting in so-called medullary sponge kidney. Associated cysts of variable size are sometimes present in the medulla and corticomedullary junction as well as in the cortex. As has been previously noted, this abnormality is thought to be inherited as an autosomal recessive trait, as opposed to polycystic liver and kidney disease which is inherited as an autosomal dominant (4, 6). The clinical manifestations of Caroli disease are most commonly recognized in children or young adults, but are occasionally seen as late as the fifth decade. The specific presentation and SUbsequent manifestations depend on whether the predominant lesion is a saccular dilatation of the intrahepatic ducts or hepatic fibrosis. If it is the former,
Fig. 5. CASE II. View of the left kidney from an excretory urogram demonstrates deformed calyces with irregular collections of contrast material in the renal pyramids consistent with medullary sponge kidney.
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sodes of crampy upper abdominal pain with occasional fever and jaundice. If the predominant lesion is hepatic fibrosis, as in CASE II, the manifestations are those of portal hypertension, and the usual presenting symptom is hematemesls. The associated renal abnormalities are usually asymptomatic. Radiologic evaluation has usually been of little help in establishing the diagnosis. In those cases in which intrahepatic biliary duct dilatation was the predominant manifestation, intravenous cholangiography has on rare occasions been helpful by the demonstration of faint, irregular, patchy collections of contrast material throughout the liver (5, 9). In general, radionuclide liver scanning is not helpful but may be abnormal in some cases where intrahepatic masses or patchy uptake can be identified. The 131 1rose bengal liver scan has been reported to be helpful in that it may demonstrate a decreased rate of bile drainage (5). Percutaneous transhepatic cholangiography is very specific in demonstrating pathological changes but is, of course, invasive. Selective hepatic angiography may demonstrate multiple avascular masses and is therefore of little value in the differential diagnosis. Excretory urography frequently demonstrates cortical cysts or renal tubular ectasia which may suggest the diagnosis; however, these renal lesions are usually found as isolated abnormalities unrelated to Caroli disease. CT scanning is an ideal technique for evaluating and establishing the diagnosis of Caroli disease. In fact, in his most recent review article, Caroli himself suggested that this would seem to be the best method, although no cases studied by CT had yet been reported (6).CT scanning is especially valuable because it may be used to assess both organ systems involved with a single noninvasive examination. When dilatation of the intrahepatic biliary ducts is the predominant feature, CT scanning can make the diagnosis with essentially 100% certainty. The findings are easily distinguished from polycystic or multiple cystic involvement of the liver because of the clear demonstration of the irregular cystic lesions communicating with the dilated biliary ducts, In polycystic liver disease the cysts are
September 1979
round and smooth and may deform but do not communicate with the biliary ducts. In simple biliary obstruction, the most dilated ducts are central, with tapering to the periphery; they do not show focal ectatic areas. When congenital hepatic fibrosis is the dominant manifestation, the diagnosis may be suggested by demonstration of a normal or slightly enlarged liver of normal density and splenomegaly with dilated splenic and portal veins in association with renal cystic disease. ACKNOWLEDGMENTS: We would like to thank Dr. Arthur Zacks for providingthe percutaneous transhepaticcholangiogramon CASE I. Also, we are indebtedto Mrs. OdetteBrinton and Mrs. Valerie Nelson for their invaluable technical assistance.
Department of Radiology Ross General Hospital 1150 Sir Francis Drake Blvd. Ross, CA 94957
REFERENCES 1. Caroli J. Soupault R. Kossakowski J, et al: La dilatation polykystique conqenltate des voles biliaires intra-hepatiques: essal de classification. Sem Hop Paris 34: 128-135. Feb 1958 2. Martin EE. Corcos V, Albano 0: La dilatation conqenltale des voles biliaires lntra-hepatiques segmentaires. Presse Med 73: 2565-2570, Oct 1965 3. Mujahed Z. Glenn F. Evans JA: Communicating cavernous ectasia of the intrahepatic ducts (Caroli's disease). Am J Roentgenol 113:21-26. Sep 1971 4. Foulk WT: Congenital malformations of the intrahepatic biliary tree in the adult. Editorial. Gastroenterology 58:253-256. Feb
1970 5. Mall JC. Ghahremani 00. Boyer JL: Caroli's disease associated with congenital hepatic fibrosis and renal tubular ectasia. A case report. Gastroenterology 66:1029-1035. May 1974 6. Caroli J: La maladie de Caroli. Med et Hyg 36:29-35. Jan
1978 7. Reilly BJ. Neuhauser EBD: Renal tubular ectasia in cystic disease of the kidneys and liver. Am J Roentgenol 84:546-554. Sep
1960 8. Six R. Oliphant M. Grossman H: A spectrum of renal tubular ectasia and hepatic fibrosis. Radiology 117:117-122. Oct 1975 9. Caroli J. Corcos V: La dilatation congllOitale des voies biliaires intrahepatiques. Rev Medicochir Mal Foie 39:1-70. Feb 1964
