FROM THE DEPARTMENTS OF SURGERY (DIRECTOR: PROF. S. BORGSTROM) AND DIAGNOSTIC RADIOLOGY (DIRECTOR: PROF. G.-F. SALTZMAN), UNIVERSITY OF UMEA, S-901 85 UMEA, SWEDEN.
ANGIOGRAPHY OF CYSTIC LIVER DISEASE A report of three cases L. DOMELLOF, G.-F. SALTZMAN and O. SUNNEGARDH
Nonparasitic liver cysts are not uncommon. As they relatively seldom give rise to symptoms most of them are detected in connection with laparotomy or autopsy. Radiologic procedures have not hitherto been of any particular significance for the diagnosis. Calcium deposits in the cyst wall may sometimes be demonstrated, but they are much rarer in these cysts than in parasitic liver cysts (SVOBODA 1957). Angiography, in the form of abdominal aortography (CAPLAN & SIMON 1966, PELTOKALLIO 1970), selective angiography (PELTOKALLIO, COUTSOFTIDES & HERMANN 1974), or splenoportal phlebography (KUMMERLE & EHLERT 1967, PELTOKALLIO), has in some cases been successful in demonstrating liver cysts as poorly vascularized expanding lesions. Animal experiments (EKELUND et coIl. 1974) have also indicated that vascular displacement around avascular expansivities is the most common angiographic finding in liver cysts. NEIMANN & GOLDSTEIN (1975) recently pointed out that in addition to stretching and displacement of hepatic artery branches cysts may appear as sharply circumscribed defects in the hepatographic phase. The cysts may be surrounded by a hypervascular rim representing the cyst wall or adjacent compressed parenchyma. It has been considered of interest to present a further three cases of nonparasitic cystic liver disease in which angiography was performed. Another reason for reporting them is that the findings in two of the cases support the observations made by the last-mentioned authors. Submitted for publication 20 May 1977. Acta Radiologica Diagnosis 18 (1977) Fasc, 6 November
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AN~IOGRAPHY OF CYSTIC LIVER DISEASE
Fig. 1
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Fig. 2
Fig. I. Case 1. Capillary phase. Strands of liver parenchyma with normal uptake of contrast medium between round avascular areas. Fig. 2. Case 2. Arterial phase. The intrahepatic arteries stretched and partly curved around expanding lesions.
Case reports Case 1. Female aged 57. At cholecystectomy for a solitary calculus in the gallbladder, performed 2 years before the present examination, the liver was regarded as normal. Following that operation, the patient had complained of increasing fatigue, intermittent pain in the right hypochondrium, and an epigastric sensation of fullness. Cholegraphy, urography, and barium enema revealed nothing abnormal. The liver gradually became palpable under the right costal margin. Isotope scanning demonstrated hepatomegaly, and a well-demarcated expanding lesion with a decreased uptake in the upper lateral part of the right liver lobe. Routine hematologic and liver tests were normal. Angiography of the hepatic artery (Fig. 1). The liver appeared enlarged. The intrahepatic vessels were stretched, in places displaced around poorly vascularized expanding areas of varying sizes. Between these areas, strands of liver parenchyma displaying uptake of contrast medium were observed in the capillary phase. Numerous cysts of varying sizes were found at laparotomy. The largest of these was evacuated, yielding 2 000 ml of thick brownish fluid, and was drained over a period of 2 weeks. Biopsy of the liver revealed that the liver parenchyma between the cysts was slightly
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654
L. DOMELLOF, G.-F. SALTZMAN AND O. SUNNEGARDH
Fig. 3. Case. 3.a) Arterial phase, b) capillaryphase.The arteriesare stretchedand to someextent displaced in the enlarged liver. Numerous avascular areas surrounded by liver parenchyma with normal uptake of contrast medium.
fibrotic and contained inflammatory cells but was otherwise normal. Nothing suggested malignancy. After the operation the patient was free from symptoms. Case 2. Male aged 42. Massive heredity for renal cystic disease on the mother's side. Hypertonia and polyuria were diagnosed 6 years before the present consultation, and urography revealed polycystic renal disease. On the same occasion, considerable hepatic enlargement was also disclosed on palpation. Isotope scanning demonstrated numerous large uptake defects in both liver lobes. Six years later, the size of the liver had further increased. Laparotomy revealed closely packed cysts with no signs of ordinary liver parenchyma. The cysts were punctured and yielded 1 500 ml of fluid. Samples from the liver displayed little evidence of abnormality. A thymol test gave a slightly raised value and a bromsulphalein test was moderately pathologic. Renal function was only slightly impaired. Angiography of the coeliac artery (Fig. 2) was performed 6 months after the laparotomy, and revealed displacement of the blood vessels in an enlarged liver. No abnormal vessels were seen, and there was no uptake of contrast medium in the renal parenchyma. The patient has been under observation for a further 4 years and the clinical picture has remained unchanged.
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ANGIOGRAPHY OF CYSTIC LIVER DISEASE
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Case 3. Female aged 40. Hematuria had been diagnosed 5 years before. Urography and nephroangiography were carried out and demonstrated the characteristic appearances of polycystic renal disease. Renal function tests in the following 5 years were normal. In connection with recent symptoms suggestive of gallstone, no contrast filling of the gallbladder was obtained at a peroral cholecystography. Liver function tests of various types all gave normal results. Scintigraphy revealed an enlarged liver with multiple uptake defects. Angiography of the coeliac and superior mesenteric arteries (Fig. 3). The hepatic artery arose as a branch from the superior mesenteric artery, and the intrahepatic arterial branches were in some areas displaced. In the capillary phase, multiple round defects were evident between normally contrast-filled liver parenchyma. At laparotomy numerous liver cysts of varying size were found in the enlarged liver. The liver capsule was slightly fibrotic. The parenchyma between the cysts was macroscopically normal.
Discussion
Nonparasitic cystic liver disease has previously been diagnosed mostly at laparotomy. Angiography has been considered of some value for diagnosis due to the fact that the vessels have been displaced around the larger cysts. As the liver function, in patients with this disease, is usually only slightly, or not at all impaired, a large part of the liver parenchyma remains intact. The functioning parenchyma is demonstrated at radiography through the uptake of contrast medium (Cases I, 3). This is achieved by high quality films produced by applying the highest possible selectivity in the angiographic technique. The subtraction method would probably be of value in some cases. The fact that nonparasitic polycystic liver disease is often combined with polycystic renal disease, as was the case both in these three patients and in those previously reported, brings up the question of whether the liver should not be examined in all patients in whom polycystic renal disease has been diagnosed. The question deserves all the more consideration, since it seems likely that angiography could in the future be substituted by computer tomography or ultrasonography, which involve less discomfort for the patient.
SUMMARY Three patients with nonparasitic polycystic liver disease have been examined by angiography. The value and technical aspects of the examination are discussed in the light of the observations in these cases as well as in previously reported cases.
ZUSAMMENFASSUNG Drei Patienten mit nichtparasitischer polyzystischer Lebererkrankung wurden angiographisch untersucht. Der Wert und die technischen Aspekte dieser Untersuchung werden im Lichte von den Beobachtungen bei diesen Fallen so wie bei frUher beschriebenen Fallen diskutiert.
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656
L. DOMELLOF, G.-F. SALTZMAN AND O. SUNNEGARDH
RESUME Trois malades atteints de maladie polykystique du foie non parasitaire ont ete examines par angiographie. Les auteurs examinent la valeur et les aspects techniques de cet examen, a la lumiere des observations faites dans ces cas ainsi que d'apres la litterature.
REFERENCES CAPLAN L. H. and SIMON M.: Nonparasitic cysts of the liver. Amer. J. Roentgenol. 96 (1966), 421. COUTSOFTIDES T. and HERMANN R. E.: Nonparasitic cysts of the liver. Surg. Gynec. Obstet. 138 (1974), 906. EKELUND L., HENRIKSSON H., OLIN T. and SJOGREN H. 0.: Angiography in hepatic cysts and tumours in the rat. Invest. Radiol. 9 (1974), 396. KOMMERLE F. und EHLERT C. P.: Zur Chirurgie der nichtparasitaren Leberzysten. Med. Klin. 62 (1967), 471. NEIMANN H. L. and GOLDSTEIN H. M.: Angiography of benign and malignant hepatic masses. Sem. Radiol. 10 (1975), 197. PELTOKALLIO V.: Non-parasitic cysts of the liver. Ann. Chir. Gynaec. Fenn. 59 (1970), Suppl. No. 174. SVOBODA M.: Zystenleber, im Rontgenbild einen Magenkrebs vortauschend. Zbl. Chir. 11 (1957), 448.
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ANGIOGRAPHY OF CYSTIC LIVER DISEASE A report of three cases L. DOMELLOF, G.-F. SALTZMAN and O. SUNNEGARDH
Nonparasitic liver cysts are not uncommon. As they relatively seldom give rise to symptoms most of them are detected in connection with laparotomy or autopsy. Radiologic procedures have not hitherto been of any particular significance for the diagnosis. Calcium deposits in the cyst wall may sometimes be demonstrated, but they are much rarer in these cysts than in parasitic liver cysts (SVOBODA 1957). Angiography, in the form of abdominal aortography (CAPLAN & SIMON 1966, PELTOKALLIO 1970), selective angiography (PELTOKALLIO, COUTSOFTIDES & HERMANN 1974), or splenoportal phlebography (KUMMERLE & EHLERT 1967, PELTOKALLIO), has in some cases been successful in demonstrating liver cysts as poorly vascularized expanding lesions. Animal experiments (EKELUND et coIl. 1974) have also indicated that vascular displacement around avascular expansivities is the most common angiographic finding in liver cysts. NEIMANN & GOLDSTEIN (1975) recently pointed out that in addition to stretching and displacement of hepatic artery branches cysts may appear as sharply circumscribed defects in the hepatographic phase. The cysts may be surrounded by a hypervascular rim representing the cyst wall or adjacent compressed parenchyma. It has been considered of interest to present a further three cases of nonparasitic cystic liver disease in which angiography was performed. Another reason for reporting them is that the findings in two of the cases support the observations made by the last-mentioned authors. Submitted for publication 20 May 1977. Acta Radiologica Diagnosis 18 (1977) Fasc, 6 November
652
Downloaded from acr.sagepub.com at MCMASTER UNIV LIBRARY on September 16, 2015
AN~IOGRAPHY OF CYSTIC LIVER DISEASE
Fig. 1
653
Fig. 2
Fig. I. Case 1. Capillary phase. Strands of liver parenchyma with normal uptake of contrast medium between round avascular areas. Fig. 2. Case 2. Arterial phase. The intrahepatic arteries stretched and partly curved around expanding lesions.
Case reports Case 1. Female aged 57. At cholecystectomy for a solitary calculus in the gallbladder, performed 2 years before the present examination, the liver was regarded as normal. Following that operation, the patient had complained of increasing fatigue, intermittent pain in the right hypochondrium, and an epigastric sensation of fullness. Cholegraphy, urography, and barium enema revealed nothing abnormal. The liver gradually became palpable under the right costal margin. Isotope scanning demonstrated hepatomegaly, and a well-demarcated expanding lesion with a decreased uptake in the upper lateral part of the right liver lobe. Routine hematologic and liver tests were normal. Angiography of the hepatic artery (Fig. 1). The liver appeared enlarged. The intrahepatic vessels were stretched, in places displaced around poorly vascularized expanding areas of varying sizes. Between these areas, strands of liver parenchyma displaying uptake of contrast medium were observed in the capillary phase. Numerous cysts of varying sizes were found at laparotomy. The largest of these was evacuated, yielding 2 000 ml of thick brownish fluid, and was drained over a period of 2 weeks. Biopsy of the liver revealed that the liver parenchyma between the cysts was slightly
Downloaded from acr.sagepub.com at MCMASTER UNIV LIBRARY on September 16, 2015
654
L. DOMELLOF, G.-F. SALTZMAN AND O. SUNNEGARDH
Fig. 3. Case. 3.a) Arterial phase, b) capillaryphase.The arteriesare stretchedand to someextent displaced in the enlarged liver. Numerous avascular areas surrounded by liver parenchyma with normal uptake of contrast medium.
fibrotic and contained inflammatory cells but was otherwise normal. Nothing suggested malignancy. After the operation the patient was free from symptoms. Case 2. Male aged 42. Massive heredity for renal cystic disease on the mother's side. Hypertonia and polyuria were diagnosed 6 years before the present consultation, and urography revealed polycystic renal disease. On the same occasion, considerable hepatic enlargement was also disclosed on palpation. Isotope scanning demonstrated numerous large uptake defects in both liver lobes. Six years later, the size of the liver had further increased. Laparotomy revealed closely packed cysts with no signs of ordinary liver parenchyma. The cysts were punctured and yielded 1 500 ml of fluid. Samples from the liver displayed little evidence of abnormality. A thymol test gave a slightly raised value and a bromsulphalein test was moderately pathologic. Renal function was only slightly impaired. Angiography of the coeliac artery (Fig. 2) was performed 6 months after the laparotomy, and revealed displacement of the blood vessels in an enlarged liver. No abnormal vessels were seen, and there was no uptake of contrast medium in the renal parenchyma. The patient has been under observation for a further 4 years and the clinical picture has remained unchanged.
Downloaded from acr.sagepub.com at MCMASTER UNIV LIBRARY on September 16, 2015
ANGIOGRAPHY OF CYSTIC LIVER DISEASE
655
Case 3. Female aged 40. Hematuria had been diagnosed 5 years before. Urography and nephroangiography were carried out and demonstrated the characteristic appearances of polycystic renal disease. Renal function tests in the following 5 years were normal. In connection with recent symptoms suggestive of gallstone, no contrast filling of the gallbladder was obtained at a peroral cholecystography. Liver function tests of various types all gave normal results. Scintigraphy revealed an enlarged liver with multiple uptake defects. Angiography of the coeliac and superior mesenteric arteries (Fig. 3). The hepatic artery arose as a branch from the superior mesenteric artery, and the intrahepatic arterial branches were in some areas displaced. In the capillary phase, multiple round defects were evident between normally contrast-filled liver parenchyma. At laparotomy numerous liver cysts of varying size were found in the enlarged liver. The liver capsule was slightly fibrotic. The parenchyma between the cysts was macroscopically normal.
Discussion
Nonparasitic cystic liver disease has previously been diagnosed mostly at laparotomy. Angiography has been considered of some value for diagnosis due to the fact that the vessels have been displaced around the larger cysts. As the liver function, in patients with this disease, is usually only slightly, or not at all impaired, a large part of the liver parenchyma remains intact. The functioning parenchyma is demonstrated at radiography through the uptake of contrast medium (Cases I, 3). This is achieved by high quality films produced by applying the highest possible selectivity in the angiographic technique. The subtraction method would probably be of value in some cases. The fact that nonparasitic polycystic liver disease is often combined with polycystic renal disease, as was the case both in these three patients and in those previously reported, brings up the question of whether the liver should not be examined in all patients in whom polycystic renal disease has been diagnosed. The question deserves all the more consideration, since it seems likely that angiography could in the future be substituted by computer tomography or ultrasonography, which involve less discomfort for the patient.
SUMMARY Three patients with nonparasitic polycystic liver disease have been examined by angiography. The value and technical aspects of the examination are discussed in the light of the observations in these cases as well as in previously reported cases.
ZUSAMMENFASSUNG Drei Patienten mit nichtparasitischer polyzystischer Lebererkrankung wurden angiographisch untersucht. Der Wert und die technischen Aspekte dieser Untersuchung werden im Lichte von den Beobachtungen bei diesen Fallen so wie bei frUher beschriebenen Fallen diskutiert.
Downloaded from acr.sagepub.com at MCMASTER UNIV LIBRARY on September 16, 2015
656
L. DOMELLOF, G.-F. SALTZMAN AND O. SUNNEGARDH
RESUME Trois malades atteints de maladie polykystique du foie non parasitaire ont ete examines par angiographie. Les auteurs examinent la valeur et les aspects techniques de cet examen, a la lumiere des observations faites dans ces cas ainsi que d'apres la litterature.
REFERENCES CAPLAN L. H. and SIMON M.: Nonparasitic cysts of the liver. Amer. J. Roentgenol. 96 (1966), 421. COUTSOFTIDES T. and HERMANN R. E.: Nonparasitic cysts of the liver. Surg. Gynec. Obstet. 138 (1974), 906. EKELUND L., HENRIKSSON H., OLIN T. and SJOGREN H. 0.: Angiography in hepatic cysts and tumours in the rat. Invest. Radiol. 9 (1974), 396. KOMMERLE F. und EHLERT C. P.: Zur Chirurgie der nichtparasitaren Leberzysten. Med. Klin. 62 (1967), 471. NEIMANN H. L. and GOLDSTEIN H. M.: Angiography of benign and malignant hepatic masses. Sem. Radiol. 10 (1975), 197. PELTOKALLIO V.: Non-parasitic cysts of the liver. Ann. Chir. Gynaec. Fenn. 59 (1970), Suppl. No. 174. SVOBODA M.: Zystenleber, im Rontgenbild einen Magenkrebs vortauschend. Zbl. Chir. 11 (1957), 448.
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