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[Portal hypertension of extrahepatic origin].

Portal hypertension may be due to a suprahepatic or infrahepatic block. Suprahepatic blocks result in Budd-Chiari syndrome. Their principal cause is myeloproliferative syndromes and invasion of the hepatic veins by a tumour. Their main manifestations are ascites and liver enlargement. Treatment consists of re-establishing the hepatofugal drainage. Infrahepatic blocks are due to obstruction of the portal vein, either by thrombosis facilitated by thrombogenic diseases, or by a tumour. Their main consequences are gastrointestinal hemorrhages. Treatment consists of preventing the rupture of oesophageal varices.

Budd-Chiari Syndrome↗

[Budd-Chiari syndrome secondary to thrombosis of the inferior vena cava in myeloproliferative disease].

A case with abdominal pain, hepatosplenomegaly and ascites is reported. Ultrasonography and computed tomography showed a thrombus localized in the inferior vena cava at the confluence of the hepatic veins. The diagnosis of Budd-Chiari syndrome was made two years after clinical and laboratory findings appeared. The patient was affected by a Philadelphia-positive myeloproliferative disease that was the predisposing condition to the inferior vena cava thrombosis. We suggest that the diagnosis of Budd-Chiari syndrome must be considered in patients presenting conditions associated with thrombotic diathesis: so a suitable therapy can be started before an irreversible hepatic damage.

Aged↗

Duplex ultrasonographic evaluation of liver transplants.

Two hundred and twenty-two duplex ultrasonographic examinations were performed on 36 liver transplants in 30 patients over a period of 5 years and 9 months. Positive sonographic findings were correlated with 13 angiographic examinations. Arterial complications included 4 thromboses, 5 stenoses, and one mycotic aneurysm. A false-negative result was obtained in 2 cases of stenosis and a false-positive result in one case of thrombosis. One portal vein thrombosis, 2 stenoses, and 2 cases of portal hypertension were diagnosed correctly. Sensitivity was 87%, specificity 95%, and accuracy 93%. Duplex Doppler was least effective in the diagnosis of hepatic artery stenosis. Five cases showed biliary complications of ischemic origin. Angiography is indicated when duplex Doppler is positive or doubtful, but also when the clinical condition is unclear.

Adult↗

The use of a pig liver xenograft for temporary support of a patient with fulminant hepatic failure.

A 26-year-old female patient with fulminant hepatic failure and a history of autoimmune hepatitis was heterotopically transplanted with a pig hepatic xenograft to provide temporary metabolic support prior to transplantation with a human donor organ. Circulating natural antipig antibodies were removed prior to transplantation by plasmapheresis and ex vivo en bloc perfusion of the donor pig kidneys. The liver xenograft functioned after transplantation as measured by active bile production, stabilization of prothrombin levels, and reduction in the circulating levels of lactic acid and the enzymes AST and ALT. Despite the removal of greater than 90% of the recipient's natural xenoantibodies prior to transplantation, the levels of antibody rapidly returned and were associated with antibody and complement-mediated rejection of the donor graft. Immunohistochemical evidence of graft rejection could be detected by the deposition of antibody, complement components including properdin, and endothelial swelling as early as 3 hr posttransplantation. These lesions progressed in severity and were accompanied by evidence of thrombosis and ischemic necrosis of the liver xenograft by 34 hrs posttransplantation. The main portal vein, hepatic artery, and vena cava were patent. The placement of the liver graft did not result in any improvement in the neurological status of the patient and she died 34 hr after xenografting due to irreversible brain damage. The information derived from this case has renewed interest in the clinical use of bioartificial devices and whole organ perfusion using xenogeneic tissue for temporary bridging of patients prior to allografting.

Adult↗

Randomized controlled trial for hepatocellular carcinoma with portal vein thrombosis: intra-arterial iodine-131-iodized oil versus medical support.

UNLABELLED: Portal vein thrombosis is a poor prognostic factor in patients with hepatocellular carcinoma (HCC) and a contraindication for chemoembolization. Intra-arterial injection of 131I-iodized oil which does not modify arterial flow, is feasible in this condition. The aim of this prospective randomized controlled trial was to compare the efficacy of treatment with radiolabeled oil (treated group) versus medical support (control group) in patients with stage I or II HCC (classification of Okuda) with portal vein thrombosis. METHODS: Twenty-seven HCC patients (26 males, 1 female), aged 53-79 yr, with portal vein thrombosis were randomly assigned to Lipiocis group (n = 14) or Control group (n = 13). Additional injections of radiolabeled oil were given 2, 5, 8 and 12 mo after initial therapy. Medical support treatment consisted of: tamoxifen (n = 5), 5 FU intravenously (n = 1), NSAIDs or corticosteroids (n = 5). Efficacy was evaluated according to survival rate (Kaplan-Meier method; log rank test), AFP serum values (measured at 2, 5, 8 and 12 mo) and angiography. RESULTS: The two groups were comparable (Child's classification, Okuda's classification, liver function tests, location of the thrombus). Tolerance was excellent in the Treated group. The actuarial survival curves were significantly different (p < 0.01) between the two groups, the survival rates (Cl 95%) at 3, 6 and 9 mo being 71% (48%-95%), 48% (12%-55%), 7% (1%-31%) for the Treated group; and 10% (1%-33%), 0% and 0% for the Control group. CONCLUSION: Intra-arterial hepatic injection of 131I-labeled iodized oil is a safe and effective palliative treatment of HCC with portal vein thrombosis.

Adrenal Cortex Hormones↗

Acute portal vein thrombosis.

The findings in acute portal vein thrombosis in a patient with chronic calcific pancreatitis and two episodes of pancreatic surgery are described. The diagnosis was made by ultrasound, which showed a dilated portal vein filled with low-level echoes, surrounding hepatic oedema, hypertrophy of the hepatic artery, splenomegaly, collateral vessels and ascites. This was confirmed by computed tomography. The ultrasonic differences in appearance between acute and chronic portal vein thrombosis are discussed, in the context of portal hypertension. The diagnosis of acute portal vein thrombosis should be considered in patients in the appropriate situation who suffer a sudden clinical deterioration with right upper quadrant or abdominal pain. Ultrasound is recommended as the imaging modality of first choice because of the flexibility of its scanning plane and its real time and Doppler capabilities. Computed tomography is valuable in patients with an ileus or heavy pancreatic calcification and for its ability to demonstrate patent vessels on intravenous injection of contrast medium.

Acute Disease↗

[Off-label use in long-term anticoagulation after liver transplantation due to phenprocoumon-induced hepatic failure].

BACKGROUND: Drug therapy should be individualized according to criteria of efficacy, adverse effects, and treatment adherence. This is particularly important at the interface of inpatient and ambulatory care. Aspects of drug approval (labeling) and individual refunding by health care insurance should also be taken into account. CASE REPORT: A patient (male, 61 years, painter) showed elevated transaminases after treatment with phenprocoumon because of a deep vein thrombosis in 1999. Transaminases normalized completely after discontinuation of phenprocoumon. Other reasons for the elevated transaminases could be excluded. After a recurrent thrombosis in 2003 phenprocoumon was prescribed again followed by recurrent elevation of transaminases and subsequent cholestatic hepatitis progressing to fulminant hepatic failure that required liver transplantation. After transplantation the patient's general state of health was good and liver function nearly normal. Anticoagulation was indicated beyond the postoperative phase because of recurrent deep vein thrombosis and atrial fibrillation. A low-molecular-weight heparin was chosen for long-term treatment. CONCLUSION: A low-molecular-weight heparin appears to be the most appropriate way to maintain effective and safe anticoagulation in this patient. Coumarins carry a residual risk of an extrahepatic, immunologically mediated cross-sensitization. Long-term use of ximelagatran may also cause liver damage. For heparinoids, hirudins, and other drugs affecting coagulation like platelet aggregation inhibitors, therapeutic evidence is not sufficient. Though subcutaneous application of heparin is a disadvantage for the patient, therapeutic alternatives do not have better documented efficacy or less hepatotoxic potential. The low-molecular-weight heparin fulfills the criteria for refunding set by federal jurisdiction.

Anticoagulants↗

Thrombosis of the portal vein in a miniature schnauzer.

A miniature schnauzer with a history of apathy, anorexia and jaundice was presented to the Utrecht University Clinic for Companion Animals. Abnormal laboratory findings included highly increased levels of total bile acids and alkaline phosphatase, and hyponatraemia. Abdominal ultrasonography revealed that the right side of the liver was enlarged and the left side was small, together with a thrombus in the portal vein. Biopsies from the right side of the liver demonstrated subacute to chronic active hepatitis, for which the dog was treated with prednisolone (1 mg/kg/day for four weeks). No improvement was observed and the owner requested euthanasia. At necropsy the left lobes of the liver were found to be small and firm, while the right lobes were large and soft. There were two thrombi in the portal vein. Microscopic examination revealed chronic active hepatitis and cirrhosis.

Animals↗

Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography.

OBJECTIVE: Cavernous transformation of the portal vein is defined as the formation of venous channels within or around a previously thrombosed portal vein. The purpose of this work was to study the hemodynamic consequences of cavernous transformation of the portal vein in a group of afflicted patients by use of Doppler sonography. We wished to study the evolution from portal vein thrombosis to the formation of cavernous transformation, the extent of resulting extrahepatic collateral channels, and the patterns of splanchnic collateral circulation. MATERIALS AND METHODS: Seventy-five patients (48 adults and 27 children) with cavernous transformation of the portal vein were studied with color and/or pulsed Doppler sonography. Blood flow in the extrahepatic portal vein, in its segmental branches, in the hepatic veins and artery, and in the splanchnic veins was examined. Collateral pathways were sought. For nine patients with acute thrombosis of the portal vein, serial examinations were performed during the formation of cavernous transformation. RESULTS: In nine patients, a fresh thrombus filled and distended the portal vein and became recanalized within a few days. Tortuous vessels appeared at the porta hepatis. These were characterized as veins or arteries with Doppler sonography. Soon the portal vein could no longer be identified within the mass of tortuous vessels. The cavernous transformation developed within 6-20 days of the acute thrombosis. A spongelike mass of collateral vessels around the main portal vein was seen in all but two patients. Intrahepatic extension of the cavernous transformation was seen in 57 patients (76%) and involved one or more intrahepatic portal veins. Two types of collateral circulation were observed: portosystemic, mainly through the left gastric and the perisplenic veins (the caput medusae, i.e., the paraumbilical-to-abdominal venous route, was never seen); and portoportal, from the periportal or pericholecystic venous channels to the intrahepatic portal veins. In nine patients, flow within unaffected intrahepatic branches of the portal vein was reversed as directed toward the cavernous transformation surrounding other, thrombosed intrahepatic segments of the portal vein. CONCLUSION: After thrombosis of the portal vein, portoportal venous channels may form not only at the porta hepatis but also within the liver. Intrahepatic blood may be shunted from one segmental portal vein to another. In addition, portosystemic collateral channels are formed, suggesting that, despite extensive hemodynamic adaptations, portal hypertension ensues.

Acute Disease↗

Hepatic and pulmonary pathology of experimental Fascioloides magna infection in guinea pigs.

The guinea pig was used to study the pathology of Fascioloides magna, an important pathogen for sheep. Although flukes migrated freely through various tissues in infected guinea pigs, the most serious lesions occurred in the liver and lungs. The sequential development of lesions indicated that flukes first invaded the quadrate lobe of the liver and subsequently migrated to other liver lobes and tissues. Six weeks post-infection, there was a marked drop in the recovery of flukes from the liver along with a dramatic increase in pulmonary involvement. Much of the hepatic and pulmonary pathology in infected animals was secondary to extensive vascular lesions caused by migrating flukes. In the liver, vascular lesions predominantly involved the portal and hepatic veins. Thrombophlebitis and locally extensive necrosis, resembling infarction, were observed. Vascular lesions in the lungs occurred in the pulmonary arteries leading to thrombosis and haemorrhagic infarction. Discovery of a fluke in a pulmonary artery, along with the pattern of hepatic and pulmonary lesions, suggested that flukes probably used the cardiovascular system as a pathway for dissemination. Death in fluke-infested guinea pigs was most often associated with severe pulmonary lesions. The nature and distribution of fluke-induced lesions observed in this study demonstrate that the guinea pig is a suitable animal model for Fascioloides magna infection in sheep.

Animals↗

Tacrolimus (FK 506) induced thrombotic thrombocytopenic purpura after ABO mismatched second liver transplantation: salvage with plasmapheresis and prostacyclin.

We report the course of thrombotic thrombocytopenic purpura (TTP) in a patient receiving tacrolimus (FK506) immunosuppression for an ABO mismatched second liver graft. A Chinese woman with fulminant hepatitis-B reactivation failed a living-related orthotopic liver transplantation (OLT) due to portal vein thrombosis. An ABO mismatched cadaveric OLT (group A to O) was performed, with peri-operative plasmapheresis to reduce anti-A hemagglutinin titers. On day 30, she developed fever, hemolysis, thrombocytopenia and neurologic dulling. Prominent microangiopathic features in peripheral blood film, and characteristic brain lesions on magnetic resonance imaging confirmed TTP. She responded initially to intensive plasmapheresis with cryosupernatant replacement, and withdrawal of FK506. An attempted reintroduction of FK506 for threatened rejection led to TTP exacerbation. This was controlled with prolonged plasmapheresis and a ten-day infusion of prostacyclin. Immunosuppression was changed to mycophenolate mofetil. By day 53, the peripheral film and lactate dehydrogenase level had returned to baseline and plasmapheresis was stopped.

ABO Blood-Group System↗

Paraneoplastic syndromes in patients with hepatocellular carcinoma in Taiwan.

BACKGROUND: Hepatocellular carcinoma (HCC) is the most common malignancy in Taiwan. Some patients may manifest paraneoplastic syndromes during the clinical course of the disease. In this study, the authors evaluated the clinical significance of these paraneoplastic syndromes, compared the prevalence of these syndromes between cases of hepatitis B virus (HBV)-related and hepatitis C virus (HCV)-related HCC, and estimated significant predictors associated with the syndromes. METHODS: Clinical data on 1197 HCC patients, including age, gender, Child-Pugh score, survival time, laboratory data (including liver biochemistry, hepatitis markers, and serum alpha-fetoprotein [AFP]), and tumor features (including tumor size, portal vein thrombosis, and histologic pictures), were retrospectively reviewed. RESULTS: A total of 232 of 1197 patients (19.4%) had paraneoplastic syndromes. HCC patients with paraneoplastic syndromes had significantly higher serum AFP; higher rates of initial main portal vein thrombosis, metastasis, and bilobal tumor involvement; larger tumor volume; and shorter survival than those without these syndromes. Patients with HBV-related HCC had a significantly higher prevalence of paraneoplastic syndromes than patients with HCV-related HCC (20.1% vs. 11.2%, P = 0.005). In a stepwise multivariate logistic regression analysis, AFP >50,000 ng/mL and tumor volume >30% were significant predictive variables associated with the presence of paraneoplastic syndromes in HCC patients. CONCLUSIONS: HCC patients with paraneoplastic syndromes usually had higher levels of serum AFP and larger tumor volumes than those without. Patients with HBV-related HCC had a significantly higher prevalence of paraneoplastic syndromes than those with HCV-related HCC.

Aged↗

Displacement of preexisting thrombus by umbilical vein catheterization.

Balloon atrial septostomy is recommended as a standard palliative procedure in neonates with congenital heart defects who are dependent on intracardiac shunting. We describe an unusual and unreported complication associated with "bedside" balloon atrial septostomy. The umbilical venous catheterization resulted in displacement of a thrombus from the ductus venosus or the hepatic vein. We recommend careful two-dimensional echocardiographic monitoring during such procedures.

Catheterization↗

Subclavian vein obstruction with collateral flow through the umbilical and hepatic veins.

Upper venous return to the heart can be obstructed by any number of pathologies and often leads to formation of collateral pathways around the obstruction. This case report describes a thrombotic obstruction of the left subclavian and brachiocephalic veins and a collateral pathway involving recanalization of the umbilical vein. Even more rare and unique is the anastomosis of the recanalized umbilical vein to the middle and left hepatic vein rather than the portal vein. The anatomy of the expected collateral pathways and the anastomotic vascular anomaly is described in detail, including a discussion of the embryological development of the system.

Brachiocephalic Veins↗

Major upper gastrointestinal haemorrhage associated with hepatic arterial chemoperfusion.

BACKGROUND: The present study reviews the nature of upper gastrointestinal complications of hepatic arterial chemoperfusion at a tertiary referral centre for the treatment of hepatic malignancy. METHODS: The patients involved in the present study all had major upper gastrointestinal (GI) haemorrhage and were undergoing hepatic arterial chemoperfusion. RESULTS: Eight patients had major upper GI haemorrhage. Three of these patients were not referred for surgical management, and all three patients died. The five patients who were admitted or transferred to our unit and who underwent surgery all survived. CONCLUSIONS: These complications are probably caused by extravasation of 5-fluorouracil (5-FU) following thrombosis of the gastroduodenal artery. The resulting cavity may perforate into the hepatic artery, portal vein, duodenum or biliary tree. Surgeons and oncologists should be aware of these complications. If upper abdominal pain occurs, chemoperfusion should cease immediately and an urgent investigation, which may include catheter angiography, gastroscopy and computed tomography (CT) scanning, should be carried out to exclude an hepatic artery pseudo-aneurysm.

Aged↗

[Abdominal ultrasound diagnosis].

The doppler sonography is an important tool in the diagnosis of liver diseases, even by using new techniques like the power doppler. There are a lot of indications for the evaluation of the hepatic vessels. Apart from the diagnosis of portal hypertension in liver cirrhosis a portal vein thrombosis should be detected or excluded in cases of new or refractory ascites. The Budd-Chiari syndrome is another important indication. Focal liver lesions can have characteristic findings in doppler sonography, but an exact differentiation of liver tumors is not possible. But doppler sonography even using the power doppler technique is be used more and more in hepatocellular carcinoma to evaluate the response to treatment with chemoembolization, radiofrequency thermoablation or percutaneous ethanol injection. The accuracy is compatible to angiography and computer-tomography.

Carcinoma, Hepatocellular↗