Diagnostic Radiology
Venous Calcifications Associated with Cavernous Transformation of the Portal Vein: Computed Tomographic and Angiographic Correlations 1 Jonathan Adler, M.D.2 A case of rare venous calcifications involving cavernous transformation of the portal vein and spontaneous splenocaval shunt is presented. Findings were obtained with plain abdominal radiography, computed tomography, and angiography. Inflammation of the portal vein with subsequent occlusion could account for splenomegaly and hypertrophy of the splenic artery and vein with atheromatous calcifications due to turbulence and sclerosis. Angiography is essential prior to surgical intervention in order to select the appropriate shunt. IND EX TERMS : Abdom en. calcification' Computed tomography. abdomen' Portal vein. Veins, splenic . (GI system. calcification. 7[0).810)' (GI system. vascular calcification. including aneurysm. 7[OJ.813). (Portal vein. splenic vein . aneurysm. 9 [57J. 730) • (Portal vein . splenic vein, cavernous transformation . 9[57) .752)
Radiology 132:27-28. July 1979
URVILINEAR venous calcifications within the abdomen are rare. In the case that follows. these calcifications were documented with plain radiography of the abdomen , computed tomography, and angiography.
Fig. 1. Plain abdominal radiograph showing left upper quadrant calcifications crossing the midline.
C
CASE REPORT A 69-year-old man of Italian descent was admitted for evaluationof jaundice. At age 19, he was found to have an enlarged spleen. At ages 25 and 62, he had transient jaundice but refused treatment. On this admission, he was found to have splenomegaly extending to the iliac crest. A globular mass in the epigastrium was palpable with a loud systolic bruit audible over it. Bilirubin was 17.4 g/100 rnl, direct 11.4, alkaline phosphatase 294 Bodansky units, SOOT 105 U/ml, and SGPT 102/ml. Plain radiographic calcifications extended from the left upper quadrant, to the midline, to the right paravertebral area near the right heart border (Fig. 1). Computed tomography of the upper abdomen revealed many curvilinear calcifications of the epigastric region (Fig. 2, a and 3, a). An exploratory laparotomy revealed dense fibrous tissue around the gallbladder preventing good exposure of the porta hepatis. A decompression hepatogastrostomy was performed, as well as multiple liver biopsies near the gallbladder fossa. Huge pulsatile vessels in the epigastrium were visualized. Angiography was performed postoperatively. The aortogramshowed an enormoustortuous splenic artery. Selective catheterizationshowed most of the left upper quadrantcalcifications to be in the splenic artery (Fig. 2, b). The venous phase showed a huge spontaneous splenocaval shunt which was responsible for the remaining calcifications (Fig. 3, b).
The venous phase of the superior mesenteric artery injection demonstrated hepatofugal flow through omental veins to the splenic hilus . Sparse filling of intrahepatic portal vein tributaries and extrahepatic collaterals confirmed the diagnosis of portal vein obstruction with cavernornatous transformation. A liver biopsy disclosed portal fibrosis with cholestasis but no evidence of malignancy. The liver architecture was normalwith no cirrhotic changes.
DISCUSSION Portal and splenic vein calcifications are presumed to be related to pyelophlebitis and the associated sclerotic reaction of the vessel wall. The calcification is within the thickened intima and media of the vein and must be distinguished from calcification of a thrombus within the lumen of a vein (1). The causes of the portal vein thrombosis in 2 reported cases were Banti syndrome (2) and postnecrotic cirrhosis following hepatitis. Prior to antibiotics, infectious processes were the most common cause, with polycythemia or other causes of increased blood viscosity accounting for some cases . Cavernous transformation of the portal vein results from formation of periportal collateral veins bridging the mesenteric and intrahepatic portal veins when the portal vein has been occluded. Most patients present with Santi syndrome (anemia, splenomegaly, and gastrointestinal bleeding). This is due to portal hypertension and formation of portosystemic collaterals, usually with bleeding from esophageal varices resulting from hepatofugal flow (3-6). Splenocaval collaterals are rare compared with the well known coronary-esophageal varices, mesenteric-iliac ve in, splenorenal, splenoretroperitoneal, or gastroepiploic and pancreatico-duodenal collaterals. The et iology of cavernous transformation of the portal vein is often related to sepsis, fever , and dehydration (7 , 8). In the neonatal stage, an umbilical infection is implicated, but this is a rare complication of such infections or of umbilical vein catheterizations. In adults, the etiology is often unknown but may be related to intra-abdominal infections with spread to the portal vein, compression due to a neoplasm, or an abnormal clotting mechanism. No clinical signs of the acute illness may be evident, and a latent period exists before the onset of splenomegaly or gastrointestinal bleeding heralds the presence of portal hyper-
1 Fromthe Departmentof Radiology, New York MedicalCollege, Westchester CountyMedical Center,Valhalla,New York. ReceivedJuly 14, 1978; revision requested Dec. 22, 1978 and accepted Apr. 3, 1979. 2 Current address: Department of Radiology, Englewood Hospital, Englewood, New Jersey. as
28
JONATHAN ADLER
July 1979
2
3
Fig . 2. a. CT scan at the L2-L3 level showing an epigastric mass containing many curvilinear calcifications which represent a tortuous splenic artery. b. Selective splenic artery injection showing the left upper quadrant calcifications in tortuous branches of the arteries. Fig. 3. a. CT scan at the L 1 level showing a tubular midline calcification which subsequently proved to be a splenocaval collateral vein. b. Late venous phase showing the midline and right paraspinal calcifications within dilated tortuous veins draining from the splenic hilum to the right atrium.
tension. Angiography is essential prior to surgical intervention so that an appropriate shunt can be chosen, as the portal vein is not available for systemic anastomosis. Avoidance of surgical interruption of spontaneous portosystemic shunts may preclude serious error in some patients. REFERENCES 1. Magovern GJ, Muehsam GE: Calcification of portal and splenic veins. Am J Roentgenol 71:84-88, Jan 1954 2. Bleich AR, Kipen CS: Venous calcification in Banti's syndrome; report of a case. Radiology 50:657-660, May 1948 3. Rosch J, Dotter CT: Extrahepatic portal obstruction in childhood and its angiographic diagnosis. Am J Roentgenol 112:143-149, May 1971 4. Ruzicka FF Jr, Rossi P: Arterial portography: patterns of venous flow. Radiology 92:777-787, Mar 1969
5. Rosch J, Steckel RJ: Abdominal viscera . [In] Hanafee WN, ed: Selective Angiography. Baltimore, Williams & Wilkins, 1972, Chapt 2, pp 77-80 6. Bron KL: Arterial portography. [In] Abrams HL, ed: Angiogram . Boston, Little-Brown, 1971, Vol 2, Chapt 64, pp 1081-1'083 7. Thompson EN, Sherlock S: The aetiology of portal vein thrombosis with particular reference to the role of infection and exchange transfusion. Quart J Med 33:465-480, Oct 1964 8. Pinkerton JA, Holcomb GW Jr, Foster JH: Portal hypertension in childhood. Ann Surg 175:870-886, Jun 1972
Department of Radiology Englewood Hospital 350 Engle S1. Englewood, New Jersey 07631
Venous Calcifications Associated with Cavernous Transformation of the Portal Vein: Computed Tomographic and Angiographic Correlations 1 Jonathan Adler, M.D.2 A case of rare venous calcifications involving cavernous transformation of the portal vein and spontaneous splenocaval shunt is presented. Findings were obtained with plain abdominal radiography, computed tomography, and angiography. Inflammation of the portal vein with subsequent occlusion could account for splenomegaly and hypertrophy of the splenic artery and vein with atheromatous calcifications due to turbulence and sclerosis. Angiography is essential prior to surgical intervention in order to select the appropriate shunt. IND EX TERMS : Abdom en. calcification' Computed tomography. abdomen' Portal vein. Veins, splenic . (GI system. calcification. 7[0).810)' (GI system. vascular calcification. including aneurysm. 7[OJ.813). (Portal vein. splenic vein . aneurysm. 9 [57J. 730) • (Portal vein . splenic vein, cavernous transformation . 9[57) .752)
Radiology 132:27-28. July 1979
URVILINEAR venous calcifications within the abdomen are rare. In the case that follows. these calcifications were documented with plain radiography of the abdomen , computed tomography, and angiography.
Fig. 1. Plain abdominal radiograph showing left upper quadrant calcifications crossing the midline.
C
CASE REPORT A 69-year-old man of Italian descent was admitted for evaluationof jaundice. At age 19, he was found to have an enlarged spleen. At ages 25 and 62, he had transient jaundice but refused treatment. On this admission, he was found to have splenomegaly extending to the iliac crest. A globular mass in the epigastrium was palpable with a loud systolic bruit audible over it. Bilirubin was 17.4 g/100 rnl, direct 11.4, alkaline phosphatase 294 Bodansky units, SOOT 105 U/ml, and SGPT 102/ml. Plain radiographic calcifications extended from the left upper quadrant, to the midline, to the right paravertebral area near the right heart border (Fig. 1). Computed tomography of the upper abdomen revealed many curvilinear calcifications of the epigastric region (Fig. 2, a and 3, a). An exploratory laparotomy revealed dense fibrous tissue around the gallbladder preventing good exposure of the porta hepatis. A decompression hepatogastrostomy was performed, as well as multiple liver biopsies near the gallbladder fossa. Huge pulsatile vessels in the epigastrium were visualized. Angiography was performed postoperatively. The aortogramshowed an enormoustortuous splenic artery. Selective catheterizationshowed most of the left upper quadrantcalcifications to be in the splenic artery (Fig. 2, b). The venous phase showed a huge spontaneous splenocaval shunt which was responsible for the remaining calcifications (Fig. 3, b).
The venous phase of the superior mesenteric artery injection demonstrated hepatofugal flow through omental veins to the splenic hilus . Sparse filling of intrahepatic portal vein tributaries and extrahepatic collaterals confirmed the diagnosis of portal vein obstruction with cavernornatous transformation. A liver biopsy disclosed portal fibrosis with cholestasis but no evidence of malignancy. The liver architecture was normalwith no cirrhotic changes.
DISCUSSION Portal and splenic vein calcifications are presumed to be related to pyelophlebitis and the associated sclerotic reaction of the vessel wall. The calcification is within the thickened intima and media of the vein and must be distinguished from calcification of a thrombus within the lumen of a vein (1). The causes of the portal vein thrombosis in 2 reported cases were Banti syndrome (2) and postnecrotic cirrhosis following hepatitis. Prior to antibiotics, infectious processes were the most common cause, with polycythemia or other causes of increased blood viscosity accounting for some cases . Cavernous transformation of the portal vein results from formation of periportal collateral veins bridging the mesenteric and intrahepatic portal veins when the portal vein has been occluded. Most patients present with Santi syndrome (anemia, splenomegaly, and gastrointestinal bleeding). This is due to portal hypertension and formation of portosystemic collaterals, usually with bleeding from esophageal varices resulting from hepatofugal flow (3-6). Splenocaval collaterals are rare compared with the well known coronary-esophageal varices, mesenteric-iliac ve in, splenorenal, splenoretroperitoneal, or gastroepiploic and pancreatico-duodenal collaterals. The et iology of cavernous transformation of the portal vein is often related to sepsis, fever , and dehydration (7 , 8). In the neonatal stage, an umbilical infection is implicated, but this is a rare complication of such infections or of umbilical vein catheterizations. In adults, the etiology is often unknown but may be related to intra-abdominal infections with spread to the portal vein, compression due to a neoplasm, or an abnormal clotting mechanism. No clinical signs of the acute illness may be evident, and a latent period exists before the onset of splenomegaly or gastrointestinal bleeding heralds the presence of portal hyper-
1 Fromthe Departmentof Radiology, New York MedicalCollege, Westchester CountyMedical Center,Valhalla,New York. ReceivedJuly 14, 1978; revision requested Dec. 22, 1978 and accepted Apr. 3, 1979. 2 Current address: Department of Radiology, Englewood Hospital, Englewood, New Jersey. as
28
JONATHAN ADLER
July 1979
2
3
Fig . 2. a. CT scan at the L2-L3 level showing an epigastric mass containing many curvilinear calcifications which represent a tortuous splenic artery. b. Selective splenic artery injection showing the left upper quadrant calcifications in tortuous branches of the arteries. Fig. 3. a. CT scan at the L 1 level showing a tubular midline calcification which subsequently proved to be a splenocaval collateral vein. b. Late venous phase showing the midline and right paraspinal calcifications within dilated tortuous veins draining from the splenic hilum to the right atrium.
tension. Angiography is essential prior to surgical intervention so that an appropriate shunt can be chosen, as the portal vein is not available for systemic anastomosis. Avoidance of surgical interruption of spontaneous portosystemic shunts may preclude serious error in some patients. REFERENCES 1. Magovern GJ, Muehsam GE: Calcification of portal and splenic veins. Am J Roentgenol 71:84-88, Jan 1954 2. Bleich AR, Kipen CS: Venous calcification in Banti's syndrome; report of a case. Radiology 50:657-660, May 1948 3. Rosch J, Dotter CT: Extrahepatic portal obstruction in childhood and its angiographic diagnosis. Am J Roentgenol 112:143-149, May 1971 4. Ruzicka FF Jr, Rossi P: Arterial portography: patterns of venous flow. Radiology 92:777-787, Mar 1969
5. Rosch J, Steckel RJ: Abdominal viscera . [In] Hanafee WN, ed: Selective Angiography. Baltimore, Williams & Wilkins, 1972, Chapt 2, pp 77-80 6. Bron KL: Arterial portography. [In] Abrams HL, ed: Angiogram . Boston, Little-Brown, 1971, Vol 2, Chapt 64, pp 1081-1'083 7. Thompson EN, Sherlock S: The aetiology of portal vein thrombosis with particular reference to the role of infection and exchange transfusion. Quart J Med 33:465-480, Oct 1964 8. Pinkerton JA, Holcomb GW Jr, Foster JH: Portal hypertension in childhood. Ann Surg 175:870-886, Jun 1972
Department of Radiology Englewood Hospital 350 Engle S1. Englewood, New Jersey 07631
