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A five-year-old girl with portal vein thrombosis and severe gastrointestinal hemorrhage recurring after repeated endoscopic sclerotherapy was successfully embolized via an ultrasonically guided transsplenic catheterization of the splenic vein.
Hepatic transplantation was performed in 46 patients over a 2-year period at the University of Minnesota. Thirty-six of these patients subsequently underwent 166 sonographic examinations of the upper abdomen. Forty-three examinations were performed within 2 weeks of transplantation, 47 between 2 and 8 weeks after transplantation, and 76 more than 8 weeks after transplantation. The sonograms were reviewed retrospectively and correlated with the available clinical and histopathologic data. Diffuse, nonspecific parenchymal abnormalities were seen in 27 patients. Focal regions of parenchymal abnormality seen in four patients were associated with abscess and infarction. Moderate to severe biliary dilatation was seen in seven patients with biliary obstruction diagnosed by percutaneous or T-tube cholangiography. Obstruction was seen most commonly in children who had undergone cholecystojejunostomy biliary reconstruction, and it required radiologic or surgical intervention. Routine sonographic assessment of the upper abdominal vasculature showed thrombosis of the portal vein in two cases, thrombosis of the inferior vena cava in one case, thrombosis of the donor aorta in three cases. Focal intraabdominal fluid collections were identified in 14 patients and represented hematomas in all but one. Free peritoneal fluid was detected in 19 patients. Sonography was used to guide percutaneous aspiration of abdominal fluid and to guide fine-needle puncture for percutaneous transhepatic cholangiography. Upper abdominal sonography provides a noninvasive means of evaluating and confirming a wide spectrum of complications that can follow hepatic transplantation. Because clinical and laboratory findings often are nonspecific in these patients, sonographic detection of focal parenchymal abnormalities, biliary dilatation, and vascular compromise is especially valuable.
The suprarenal segment of the inferior cave can be exposed by sterno-laparotomy. By reclining the left and caudate lobes of the liver to the right, the left border of the retro-hepatic segment of the inferior vena cava is displayed. Duodeno-pancreatic separation gives access to its intra-hepatic segment. The entire suprarenal segment of the vein can thus be exposed, its lower portion only being barred by the hepatic vessels. Such wide exposure makes complete hepatic and caval vascular exclusion possible, as well as elective repair surgery of all lesions involving this segment.
Pulmonary hypertension accompanying portal hypertension is a rare cause of plexogenic pulmonary arteriopathy, which etiopathogenesis is still unknown. The case report presented is that of a young man who underwent surgery at age 8 for ruptured esophageal varices associated with portal thrombosis, and who later developed pulmonary hypertension which led to death at age 21. Hepatic filtration shunt and similarity of this syndrome to pulmonary hypertension subsequent to ingestion of the appetite depressant Aminorex suggest the hypothesis that pressor substances, not detoxified by the liver, are released into the pulmonary circulation.
A preliminary report is presented of a posterior exposure of the splenic vein allowing H-graft splenorenal shunt to be carried out without removing the spleen. The operation was successfully performed in 14 patients with portal hypertension due to schistosomal hepatic fibrosis, and this was associated with post-hepatitic cirrhosis in 5 of them. Dacron grafts were inserted in 8 patients and autologous internal jugular vein grafts in 6 patients. The long term follow-up of the patients (maximum 45 months) showed that recurrence of bleeding developed in 2 patients due to shunt thrombosis and one died. Complications occurred in 3 patients, including one patient who developed first grade encephalopathy. Shunt patency was demonstrated in 12 patients, including all the vein grafts.
The transjugular intrahepatic portosystemic shunt (TIPS) is an interventional treatment resulting in decompression of the portal system by creation of a side-to-side portosystemic anastomosis. Since its introduction 16 years ago, more than 1,000 publications have appeared demonstrating broad acceptance and increasing clinical use. This review summarizes our present knowledge about technical aspects and complications, follow-up of patients and indications. A technical success rate near 100% and a low occurrence of complications clearly depend on the skills of the operator. The follow-up of the TIPS patient has to assess shunt patency, liver function, hepatic encephalopathy and the possible development of hepatocellular carcinoma. Shunt patency can best be monitored by duplex sonography and can avoid routine radiological revision. Short-term patency may be improved by anticoagulation, while such a treatment does not influence long-term patency. Stent grafts covered with expanded polytetrafluoroethylene show promising long-term patency comparable with that of surgical shunts. With respect to the indications of TIPS, much is known about treatment of variceal bleeding and refractory ascites. The thirteen randomized studies that are available to date show that survival is comparable in patients receiving TIPS or endoscopic treatment for acute or recurrent variceal bleeding. Another group comprises patients with refractory ascites and related complications, such as hepatorenal syndrome and hepatic hydrothorax. It has been demonstrated that TIPS improves these complications. Five randomized studies comparing TIPS with paracentesis and one study comparing TIPS with the peritoneo-venous shunt showed good response of ascites but controversial results on survival. In addition, TIPS has been successfully applied to patients with Budd-Chiari syndrome, portal vein thrombosis, before liver transplantation, and for the treatment of ectopic variceal bleeding.
PURPOSE: Randomized studies on tamoxifen treatment of hepatocellular carcinoma (HCC) produced conflicting results. The aim of this study was to assess the efficacy of tamoxifen administration in improving overall survival of patients with advanced HCC. PATIENTS AND METHODS: A total of 420 patients with HCC who were not suitable for surgery or local treatment were randomly assigned between April 1995 and May 2000: 210 in the control group and 210 in the tamoxifen group (20 mg/d orally). Patients with WHO performance status greater than 2, belonging to Child-Pugh class C, or with serum creatinine greater than 130 mumol/L were not eligible. RESULTS: Tolerance was good and the main reported adverse effects were thrombophlebitis (three patients), nausea (two patients), and hot flushes (three patients). Outcome did not differ between the two treatment arms: estimated median survival was 4.8 and 4.0 months in the tamoxifen and in the control groups, respectively (P = .25). Univariate analysis showed significant association of survival with age, Okuda stage, WHO performance status, Child-Pugh class, intrahepatic tumor stage, alpha-fetoprotein serum concentration, and presence of extrahepatic spread, portal vein thrombosis, hepatomegaly, or hepatalgia. In a Cox proportional hazards model we found a significant beneficial effect of tamoxifen on survival in patients belonging to Okuda I or II stages. CONCLUSION: In this large study, tamoxifen did not improve the survival of patients with advanced HCC, but there is a suggestion that patients without major hepatic insufficiency seem to have some survival benefit. New trials involving this specific population are warranted.
Pancreatic pseudocyst is a common complication of acute and chronic pancreatitis. Extrapancreatic locations of pancreatic pseudocyst in the liver, pleura, mediastinum, or pelvis have been described. However, a pancreatic pseudocyst located in the liver is an infrequent condition. We present the case of a 46-year-old man with pancreatic pseudocyst located in the liver secondary to chronic alcoholic pancreatitis. During admission, the patient underwent an abdominal CT scan that showed a mass located in the head and body of the pancreas, as well as a thrombosis of the splenic vein. A percutaneous needle aspiration biopsy of the pancreas was obtained under CT guidance, which showed no tumoral involvement. Fourty-eight hours after the procedure the patient developed abdominal pain and elevated serum amylase levels. A pancreatic MRI exam showed two pancreatic pseudocysts, one of them located in the left hepatic lobe, the other in the pancreatic tail. Chronic pancreatitis signs also were found. Enteral nutrition via a nasojejunal tube was administered for two weeks. The disappearance of the pancreatic pseudocyst located in the pancreatic tail, and a subtotal resolution of the pancreatic pseudocyst located in the liver were observed. To date twenty-seven cases of pancreatic pseudocyst located in the liver have been published, most of them managed with percutaneous or surgical drainage.
We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.
We reported a 60-year-old male patient with hepatocellular carcinoma (HCC) of 5cm in diameter with advanced tumor thrombosis in the left main trunk of portal vein and bile duct. He was treated with multimodal treatments resulting in a long-term survival of more than 4 years. At first, he was treated with transcatheter arterial chemoembolization (TACE) in April 1999, but the therapeutic effect was insufficient. Therefore, we performed an extended left hepatic lobectomy in July. Since six HCCs appeared in a posterior segment in January 2000, we achieved microwave coagulation therapy under laparotomy. Because of diffuse relapse of HCCs in the same segment of the liver, we performed hepatic arterial chemotherapy (HAC) using low-dose CDDP and 5-FU. As a result, complete disappearance of the tumors was observed. A new lesion appeared in S7 in January 2001. We performed TACE, but relapsed in June, so we selected percutaneous radio-frequency ablation under CO2 angiography. Since a recurrent tumor was detected at the same therapeutic site with invasion to the diaphragm in September 2002, we performed a partial liver resection with synchronous excision of the diaphragm. We continued TACE and systemic chemotherapy for relapses in and out of the liver. Accordingly, he lived for over four years. We conclude that a long-term survival in this patient can be attributable to appropriate treatment selections and timing, such as hepatic resection, TACE, HAC and ablation therapies based on changes in diagnostic imaging and tumor markers. In addition, we have to pay attention to keep good hepatic reserve in order to continue treatment for recurrences of HCC.
AIM: Clinical application and potential complication of percutaneous transsplenic varices embolization (PTSVE) of esophageal or gastrio-fundal varices in patients with hepatocellular carcinoma (HCC) complicated with portal vein cancerous thrombosis (PVCT). METHODS: 18 patients with HCC complicated with PVCT and esophageal or gastrio-fundal varices who underwent PTSVE were collected. The rate of success, complication, mortality of the procedure and postoperative complication were recorded and analyzed. RESULTS: PTSVE were successfully performed in 16 of 18 cases, and the rate of success was 89%. After therapy erythrocyte counts decreased in all of the natunts. 5 of patients needed blood transfusion, 2 patients required surgical intervention because of and 11 patients with ascites were alleviated by diuresis. Among these 18 patients, the procedure-related mortality was 11% (2/18), one died of acute hepatic failure on the forth day after procedure, another died of acute renal failure on the fifth day. The patients were follow up for 1-12 mon exceptone. 13 of them died of their tumors but none of them experienced variceal bleeding. CONCLUSION: PTSVE is a relatively safe and effective method to treat esophageal or gastrio-fundal varices in HCC patients with PVCT when percutaneous transhepatic varices embolization (PTHVE) of varices is impossible.
Orthotopic end-to-end cavostomy following liver transplantation was without any complications in 63 cases as compared to latero-lateral cavostomy (14.2%) and the piggy-back technique of partial liver grafts (14.2%). Transjugular catheterangioplasty was a practical and successful treatment for the latter, while reoperation for thrombosis in the latero-lateral anastomosis resulted in lethal pulmonary embolism.
A new operative approach to resecting tumor thrombus originating from a right renal cell carcinoma extending into the suprahepatic inferior vena cava (IVC) is reported. Complete local control of the IVC must be obtained above and below the tumor thrombus to remove it under direct vision. The caudate lobe of the liver was resected to expose the retrohepatic IVC and open the lesser omentum. The subhepatic IVC was encircled just below the confluence of the hepatic veins. Caval tumor thrombus was removed en bloc, including the right kidney, by use of the total hepatic vascular exclusion technique (THVE) and IVC exclusion. The retrohepatic IVC was clamped just below the confluence of the hepatic veins once the thrombus was removed, and the suprahepatic IVC clamp was then released and the THVE was terminated. The sequential clamping from the suprahepatic IVC to the retrohepatic IVC below the confluence of the hepatic veins shortened the THVE time.
PURPOSE: To evaluate benefit, feasibility, and frequency of complications with transjugular liver biopsy using a semi-automatic Tru-cut system. MATERIALS AND METHODS: Eighty-five consecutive patients (57 males, 28 females) with various liver disorders (cirrhosis [30], hepatitis [12], acute hepatopathy [34], orthotopic liver transplantation [8], hepatocellular carcinoma [1]), coagulopathies (n=71) and/or ascites (n = 46) were referred to our department for a transjugular liver biopsy. Mean age was 48 +/- 16 years (range 17 to 75 years). Success and complications were retrospectively evaluated from the radiology reports, pathology reports, and patient files. Success was defined as procuring a tissue specimen that enabled a definite histological diagnosis. The complications included thrombosis at the puncture site, hematoma, cardiac arrhythmia, capsular perforation, hemorrhage, and cardiac damage. Mortality included all deaths within 30 days after the procedure. Procedure-related mortality included all deaths related to the procedure. RESULTS: The procedure was technically successful in 80 patients (94 %) and unsuccessful in 5 patients (6 %) due to a failed hepatic vein cannulation (1 patient with Budd Chiari syndrome and total liver vein occlusion, 4 patients with unsuitable anatomy). One biopsy pass was made in 22 patients, and two passes were made in 45 and three or more passes in 14 patients, all in a single session. The sample quality was judged by the pathologist as good in 71 of 80 patients (89 %) and poor in 8 patients (10 %). A diagnosis was not possible in 1 patient. Eight procedure-related complications occurred, which were classified according to the criteria of the Society of Interventional Radiology (SIR) as minor in 5 (3 type A, 2 type B) and major in 3 (1 pneumothorax, type C, 1 nonfatal bleeding, type D, and 1 fatal bleeding, type F). Procedure-related mortality was 1 %, overall mortality 15 % (mostly due to progressive liver failure). CONCLUSION: In patients with coagulopathies, transjugular liver biopsy is a viable alternative for hepatic tissue evaluation with a good sample quality. In a small number of patients severe complications may occur.
The diagnosis of acute rejection in liver transplantation usually needs hepatic biopsy, but hepatic biopsy sometimes involves severe complications. We analyzed biliary bilirubin fraction after living related liver transplantation by using high performance liquid chromatography (HPLC) and investigated availability for the early diagnosis of acute rejection retrospectively. Nine children with liver cirrhosis due to biliary atresia were included in this study, who underwent living related liver transplantation at The Second Department of Surgery, Tohoku University School of Medicine. Bile was collected daily from a biliary canulae inserted into the hepatic duct of the graft under aseptic and without exposure to the light. We measured the proportion of bilirubin diglucuronide (BDG), bilirubin monoglucuronide (BMG) and unconjugated bilirubin (UCB) of bile pigments in the bile by HPLC. In three of four patients with acute rejection, BDG + BMG (= Bc) was above 85% and BDG/Bc ratio was below 0.6 at the time of hepatic biopsy. After rejection therapy, BDG/Bc ratio increased in their bile. The remaining one case with acute rejection as well as bile duct injury due to arterial thrombosis of S2, Bc was below 85%, and BDG/Bc ratio was below 0.6. In four of the other five patients who had several severe complications, i.e., arterial or portal vein thrombosis, bile stasis due to cholangitis and sepsis due to necrotizing myofascitis, Bc was below 85% and BDG/Bc ratio was below 0.6. We concluded that analysis of biliary bilirubin fraction after liver transplantation could be reliable as a noninvasive maker and valuable for the early diagnosis of acute rejection.
In a 66-year-old woman with pruritus, jaundice, dark-brown urine and light-colored faeces obstructive jaundice was diagnosed. Despite extensive investigations, it was not possible to clearly distinguish if varicosis or cholangiocarcinoma was the cause of the obstruction. During laparotomy the right lobe of the liver was seen to be greatly underdeveloped. The portal system showed a varicose deformation with compression of the bile ducts and portal hypertension. The right lobe of the liver was removed and the portal hypertension was treated by creating a shunt between the hepatic portal vein and the right ovarian vein. The jaundice disappeared and the patient recovered. Histological investigation showed atrophy, secondary biliary fibrosis, cirrhosis and a biliary cystadenoma. There were no signs of malignancy. The varicose deformation can be considered to be a result of the portal hypertension caused by fibrosis and cirrhosis with possibly a history of thrombosis and insufficient recanalization.
We previously reported the beneficial effects of combination therapy of interferon (IFN)-alpha/5-fluorouracil (FU) for advanced hepatocellular carcinoma (HCC) with tumour thrombi in the major portal branches. This report describes the results of longer follow-up and includes more than double the number of patients relative to the original report, and evaluates the role of IFN-alpha/type 2 interferon receptor (IFNAR2) expression on the response to the combination therapy. The study subjects were 55 patients with advanced HCC and tumour thrombi in the major branches of the portal vein (Vp3 or 4). They were treated with at least two courses of IFN-alpha/5-FU without major complication. In the 55 patients, 24 (43.6%) showed objective response (eight (14.5%) showed complete response, 16 (29.1%) partial response), four (7.3%) showed no response, and 27 (49.1%) showed progressive disease. Immunohistochemically, IFNAR2 expression was detected in nine out of 13 (69.2%) patients. There was significant difference in the time-to-progression survival (P = 0.0002) and the overall survival (P < 0.0001) between IFNAR2-positive and -negative cases. There was a significant correlation between IFNAR2 expression and response to IFN-alpha/5-FU combination therapy in univariate analysis (P = 0.0070). IFN-alpha/5-FU combination therapy is a promising modality for advanced HCC with tumour thrombi in the major portal branches and could significantly depend on IFNAR2 expression.
Portal cavernoma is a network of veins whose caliber, initially millimetric or microscopic, is increased and which contain hepatopedal portal blood. It results from occlusion, thrombotic and always chronic, of the extra-hepatic portal system. Diagnosis is mainly done by imaging. Clinical signs of portal cavernoma are usually related to extra-hepatic portal hypertension (hematemesis due to rupture of oeso-gastric varices, splenomegaly, rectal bleeding from ano-rectal varices, growth retardation in children) and sometimes to the cause of portal hypertension (abdominal pain, venous bowel infarction). Occurrence of portal thrombosis is often the conjunction of a local cause and a prothrombotic disorder which must be systematically detected. Biliary consequences of cavernoma are related to compression of common bile duct and are usually asymptomatic. In case of jaundice or cholangitis, portal decompression by portosystemic shunt can be performed to treat both biliary symptoms and portal hypertension.