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Neoplasms containing normal hepatic vessels: imaging features.

BACKGROUND: We investigated the incidence and imaging features of hepatic neoplasms containing normal hepatic vessels. METHODS: Among 3183 patients with various hepatic neoplasms, we found nine patients with normal hepatic vessels traversing hepatic neoplasms. The presence of mass effect on the vessel traversing hepatic neoplasms was evaluated. Other suggestive findings of neoplasms such as altered hepatic contour, portal vein thrombosis, mass effect on extratumoral vessel, and bile duct dilatation were analyzed. RESULTS: Thirteen hepatic vessels (nine hepatic veins and four portal veins) extended through hepatic neoplasms in nine patients. Undisturbed hepatic vessels within the neoplasms were found in five patients with either primary or metastatic hepatic neoplasm. In one patient with undisturbed hepatic vessels within the neoplasm, there were no associated abnormal findings such as biliary dilatation, change of hepatic contour, or any changes involving the vessels external to neoplasms. CONCLUSION: Although rare, various primary and secondary hepatic neoplasms can have normal hepatic vessels passing through them without mass effect. However, correct diagnosis in most neoplasms would be possible with careful examination of associated findings.

Adolescent↗

Fulminate intracardiac thrombosis associated with Budd-Chiari-syndrome and inferior vena cava thrombosis.

The most common cause of edema of the legs and dyspnea is congestive heart failure. Further differential diagnosis such as renal or hepatic failure have to be considered. We report the case of a previous healthy 65-year-old woman who developed dyspnea and massive edema of the legs followed by acute hepatic and renal failure. Imaging studies showed a thrombosis of the inferior vena cava (IVC) caused by a tumor between the right kidney and the IVC. Histological examination revealed a leiomyosarcoma of the IVC. Hepatic failure due to venous outflow obstruction (Budd-Chiari syndrome, BCS) was diagnosed. Coagulation profile showed a complex disorder due to acute hepatic failure. Factor V Leiden and prothrombin gene mutation G20210A could be excluded. The thrombosis extended from the femoral veins up to the right atrium. After 11 days of anticoagulation with heparin platelet counts decreased by more than 50%. Suspecting a heparin-induced thrombocytopenia the patient was placed on recombinant hirudin (lepirudin) for anticoagulation. Hepatic venogram showed a thrombosis of the hepatic vein orifices but not of the hepatic veins. The tumor and the thrombi were removed surgically. When the cardiopulmonary bypass was terminated new intracardiac thrombi occurred. Despite immediate surgical intervention the patient finally died due to right ventricular failure caused by the fulminate intracardiac thrombosis. In conclusion, thrombosis of the IVC may mimic congestive heart failure and may cause BCS. Neoplasms and coagulation disorders may cause thrombosis of the IVC.

Aged↗

Budd-Chiari syndrome: early intervention with angioplasty and thrombolytic therapy.

We present a complex case of the Budd-Chiari syndrome due to thrombosis of the hepatic veins in the presence of stenosis of the left hepatic vein and membranous obstruction of the inferior vena cava. The acute thrombosis occurred after laparoscopic surgical removal of the gallbladder. Because we strongly suspected the Budd-Chiari syndrome, hepatic venography was performed. The hepatic venous outflow obstruction was relieved by angioplasty and thrombolytic therapy with use of local infusions of urokinase into the clot. We propose that angiography be performed in patients in whom the Budd-Chiari syndrome is suspected and that angioplasty and thrombolytic therapy be initiated early.

Adult↗

Absence of the common bile duct and junction of the cystic duct with the left hepatic duct in a patient with chronic portal vein thrombosis.

A50-year-old male patient was admitted to the hospital because of fatigue and a palpable abdominal mass. The diagnoses of chronic renal failure and portal vein thrombosis were established by specific investigations. Incidentally, junction of the cystic duct with the left hepatic duct and absence of the common bile duct were found during endoscopic retrograde cholangiopancreatography study. To the authors' knowledge, this is the first reported case of an absence of the common bile duct and junction of the cystic duct with the left hepatic duct in a patient with chronic portal vein thrombosis. Such lesions may cause unexpected complications during biliary surgery.

Chronic Disease↗

Meso-portal bypass in children with portal vein thrombosis: rapid increase of the intrahepatic portal venous flow after direct portal hepatic reperfusion.

BACKGROUND/PURPOSE: In children with portal vein (PV) thrombosis, hepatopetal portal flow can be restored by an innovative surgical procedure, the meso-portal-bypass (MPB), if the umbilical portion of the intrahepatic left PV and the superior mesenteric vein are patent. This is associated with resolution of symptoms related to extrahepatic portal hypertension (EHPH). However, no data are available yet on intrahepatic hemodynamic changes after MPB. The aim of this study was to evaluate morphologic adaptation and flow characteristics of the intrahepatic PV branches (ihPVb) after MPB. METHODS: Prospective follow-up Doppler scans of the ihPVb were performed at 0.5 to 1, 3 to 6 and 12 months after MPB in 13 consecutive patients (2000-2002) and compared with preoperative findings. RESULTS: Only small ihPVb were detected preoperatively on Doppler in 8 of 13 cases. Postoperatively (median follow up 12; range, 6 to 24 months), all 13 patients had patent MPB with hepatopetal flow, and ihPVb were easy to detect with satisfactory vessel diameters and flow velocities. CONCLUSIONS: The preoperatively small ihPVb increase rapidly in diameter and hepatopetal flow velocity in patients benefiting from MPB. This correlates well with progressive resolution of their symptoms related to EHPH and reflects rapid adaptation of ihPVb.

Adolescent↗

Pediatric liver transplantation: fourteen years of experience at the children institute in São Paulo, Brazil.

This study reports the 14-year experience of a single center on 206 liver transplantations from living and cadaveric donors performed in 179 pediatric patients. Biliary atresia (57.2%) and fulminant hepatitis (9.8%) were the most frequent indications. The mean age of the recipients was 3 years, 7 months (9 months to 18 years) and mean weight was 14 kg (7 to 57 kg). The allografts were distributed as 82 (39.8%) whole cadaveric, 76 (36.9%) reduced-size cadaveric, 46 (22.3%) living donor liver transplants, and 2 (0.9%) ex situ split livers. The waiting periods were 25 days for living donors and 2.5 years for cadaveric donors (P <.001). Twenty-seven children were retransplanted with hepatic artery thrombosis the most frequent indication. The postoperative complications were: primary nonfunction (12.2%), biliary stenosis (28.8%), hepatic artery thrombosis (12.2%), portal vein stenosis (4.9%), hepatic vein stenosis (6.9%), and lymphoproliferative disorder (5.9%). The diagnosis of biliary stenosis was obtained by liver biopsy and transhepatic cholangiography and treated by balloon dilatation, although four children (3.9%) required a redo hepaticojejunostomy. The venous stenoses were percutaneously dilated with five-children (4.9%) requiring venous stents. The incidence of hepatic vein stenosis was 15.6% among living donor and 2.5% in cadaveric liver transplantation (P <.05). The overall 5-year patient and graft survivals were 70.2% and 65.1%. Liver transplantation provides excellent long-term survival. The use of grafts from living donors decreases the waiting periods but increases the incidence of hepatic vein stenosis.

Brazil↗

Side-to-side portacaval shunt in the treatment of Budd-Chiari syndrome.

This is a case of a 22-year-old female patient with idiopathic thrombosis of the hepatic veins in whom side-to-side portacaval shunt was followed by the disappearance of a hitherto intractable ascites and a marked improvement in her general condition. The patient remained well for 1 1/2 years after surgery; however, liver cirrhosis, which was present before shunt, persisted. From this case and from the few published cases, the authors concluded that side-to-side portacaval shunt might be beneficial to patients with thrombosis of the hepatic veins.

Adult↗

Anticardiolipin antibodies in chronic hepatitis B and chronic hepatitis D infection, and hepatitis B-related hepatocellular carcinoma. Relationship with portal vein thrombosis.

OBJECTIVE: To assess the presence of anticardiolipin antibodies (ACAs) in patients with chronic hepatitis B virus (HBV) infection, chronic hepatitis D virus (HDV) infection and HBV-related hepatocellular carcinoma (HCC) and to associate this with the incidence of portal vein thrombosis (PVT) in HCC patients. PATIENTS AND METHODS: Sixty-five cirrhotic patients with HBV-related HCC, 28 naive patients with chronic HBV infection and 14 naive patients with chronic HDV infection were enrolled prospectively in the study. Thirty-two healthy blood donors were used as controls. The ACAs (immunoglobulin G and immunoglobulin M) were measured using an enzyme-linked immunosorbent assay system. Statistical analysis used non-parametric methodology (chi-squared test, Student t-test and Fisher exact test, P value<0.05). RESULTS: Eleven of the 65 patients with HCC (16.9%) showed a positive ACA titre and 22 of the patients (34%) had PVT. Of these patients, eight (36%) had a positive ACA titre. In contrast, from the 43 patients without PVT, only three (11%) showed a positive titre. From the 28 HBV patients, six (21.5%) had a positive ACA titre, and six out of 14 (42.8%) HDV patients also showed a positive ACA titre. Three of the six ACA positive HBV patients presented an extrahepatic manifestation of the disease. One out of 32 control patients (3%) had positive ACAs. CONCLUSION: Both chronic HBV and chronic HDV infections are potent stimulants for the production of ACAs. The presence of ACAs in a great proportion of HBV-cirrhosis-related HCC patients with PVT suggests their possible participation in thrombotic mechanisms and in the hypercoagulable state that occurs in advanced liver disease and HCC.

Adult↗

CO(2) wedged hepatic venography in the evaluation of portal hypertension.

BACKGROUND/AIMS/METHODS: During hepatic vein catheterisation, in addition to measurement of hepatic venous pressure gradient (HVPG), iodine wedged retrograde portography can be easily obtained. However, it rarely allows correct visualisation of the portal vein. Recently, CO(2) has been suggested to allow better angiographic demonstration of the portal vein than iodine. In this study we investigated the efficacy of CO(2) compared with iodinated contrast medium for portal vein imaging and its role in the evaluation of portal hypertension in a series of 100 patients undergoing hepatic vein catheterisation, 71 of whom had liver cirrhosis. RESULTS: In the overall series, CO(2) venography was markedly superior to iodine, allowing correct visualisation of the different segments of the portal venous system. In addition, CO(2), but not iodine, visualised portal-systemic collaterals in 34 patients. In cirrhosis, non-visualisation of the portal vein on CO(2) venography occurred in 11 cases; four had portal vein thrombosis and five had communications between different hepatic veins. Among non-cirrhotics, lack of portal vein visualisation had a 90% sensitivity, 88% specificity, 94% negative predictive value, and 83% positive predictive value in the diagnosis of pre-sinusoidal portal hypertension. CONCLUSIONS: Visualisation of the venous portal system by CO(2) venography is markedly superior to iodine. The use of CO(2) wedged portography is a useful and safe complementary procedure during hepatic vein catheterisation which may help to detect portal thrombosis. Also, lack of demonstration of the portal vein in non-cirrhotic patients strongly suggests the presence of pre-sinusoidal portal hypertension.

Carbon Dioxide↗

Transformation of inferior vena caval thrombosis to membranous obstruction in a patient with the lupus anticoagulant.

A 24-yr-old woman with hemolytic anemia developed multiple thrombosis of the hepatic vein and inferior vena cava. She was found to have circulating lupus anticoagulant that could have been causally related to the thrombosis and hence the Budd-Chiari syndrome. On her first admission to the hospital vena cava and hepatic vein catheterizations revealed partial thrombotic occlusion of the cava at the level of the diaphragm, which was subsequently transformed into complete membranous obstruction. The right hepatic vein, which was patent on the first admission, was also completely occluded. These observations support the theory that membranous obstruction of the inferior vena cava is a sequela to inferior vena caval thrombosis rather than a congenital anomaly.

Adult↗

Outcome of extra-anatomic vascular reconstruction in orthotopic liver transplantation.

BACKGROUND: Portal venous and hepatic arterial reconstruction are critical to successful outcomes in orthotopic liver transplantation (OLT). With portal vein thrombosis or inadequate hepatic arterial inflow, extra-anatomic vascular reconstruction is required. However, the clinical outcomes following extra-anatomic vascular reconstruction are largely unknown. METHODS: To determine the outcomes associated with extra-anatomic vascular reconstruction, we performed a retrospective review of 205 OLT recipients transplanted between 1995 and 2000. RESULTS: Extra-anatomic portal venous inflow was based upon the recipient superior mesenteric vein using donor iliac vein graft in a retrogastric position (n = 12). Extra-anatomic arterial inflow was based on recipient infrarenal aorta using donor iliac artery graft through the transverse mesocolon (n = 25). OLT with routine anatomic vascular construction served as control (n = 168). Extra-anatomic vascular reconstruction was not associated with increased morbidity, mortality, operating room time, length of stay, or thrombosis. CONCLUSION: We conclude that extra-anatomic vascular conduits are associated with excellent long-term outcomes and provide acceptable alternatives for vascular reconstruction in OLT.

Female↗

Acute Budd-Chiari syndrome, portal and splenic vein thrombosis in a patient with ulcerative colitis associated with antiphospholipid antibodies and protein C deficiency.

We report the case of a female patient who had severe thrombotic complications in peripheral (V. jugularis, subclavia, brachialis, poplitea) and visceral (portal and splenic) veins 4 years after the first diagnosis of severe ulcerative pancolitis. A thrombolysis therapy for subclavian and jugular vein thrombosis was performed without complication, but she soon developed acute thrombosis of the hepatic veins (acute Budd-Chiari syndrome). She quickly recovered after liver transplantation and now - 6 years later - she lives a normal life with continuous anticoagulation and medical therapy of the colitis.3 possible causes for the severe coagulation defect in this patient can be supposed: Thrombocytosis, protein C deficiency and an antiphospholipid antibody syndrome.

Adolescent↗

A rare case of complex vascular reconstruction in liver transplantation.

Abnormalities of recipient or donor vascular structures are associated with reconstructive difficulties in liver transplantation. A patient with thrombosis of the right hepatic vein and associated stricture of the inferior vena cava (IVC), portal vein thrombosis and multiple aberrant arteries underwent orthotopic liver transplantation. The donor's suprahepatic IVC was anastomosed to the recipient's intrathoracic IVC. The portal vein flow was restored by venous graft interposition, while the arterial flow was ensured by interposing an iliac arterial graft anastomosed to the infrarenal aorta. In conclusion, graft function remains excellent more than 5 years postoperatively.

Anastomosis, Surgical↗

Hepatic arterial blood flow in large hepatocellular carcinoma with or without portal vein thrombosis: assessment by transcutaneous duplex Doppler sonography.

BACKGROUND: As liver cirrhosis progresses, the portal venous blood (PVBF) flow decreases, accompanied by an increase in hepatic arterial blood flow. Large hepatocellular carcinoma is a hypervascular tumour with a rapid growth, which seems to require an increase of the tumoral arterial blood flow. Furthermore, hepatocellular carcinoma is frequently associated with portal vein thrombosis, which subsequently impedes portal blood supply. METHODS: The purpose of our study was to estimate alterations in the hepatic arterial blood flow in large hepatocellular carcinomas occurring in liver cirrhosis, in comparison with liver cirrhosis and controls. Liver blood flow measurements were determined by duplex Doppler sonography in 47 patients with large hepatocellular carcinomas (13 with portal vein thrombosis and 34 without this thrombosis), 42 liver cirrhosis patients and 30 controls. The Doppler perfusion index was calculated as the ratio of hepatic arterial blood flow to total hepatic blood flow. RESULTS: The patients with liver cirrhosis had a significant increase of hepatic arterial blood flow as compared to controls (P < 0.001), accompanied by a significant reduction in PVBF (P < 0.005). As a result, the Doppler perfusion index was increased in patients with liver cirrhosis as compared to controls (P < 0.001). The hepatic arterial blood flow was increased in patients with hepatocellular carcinoma but without portal vein thrombosis as compared to the cirrhotic patients (P < 0.001), with a significant reduction of PVBF (P < 0.001). Hepatic arterial blood flow was also increased in patients with both hepatocellular carcinoma and portal vein thrombosis as compared to the patients without this thrombosis (P < 0.001). CONCLUSION: These results suggest that in large hepatocellular carcinomas there is a decreased PVBF, accompanied by an increased hepatic arterial blood flow. The hepatic arterial buffer response seems to be active in hepatocellular carcinomas and maintains liver perfusion to adequate levels.

Adult↗

[Preliminary experimental study on treatment of portal hypertension with auxiliary partial orthotopic liver transplantation].

OBJECTIVE: To evaluate the therapeutic efficacy of auxiliary partial orthotopic liver transplantation (APOLT) on portal hypertension in liver cirrhosis with amelioration of portal vein congestion, changes in portal vein pressure and status of the graft. METHODS: The recipients were porcine model of biliary cirrhosis reproduced by ligation of the common bile duct. During transplantation, arterial blood pressure, central venous pressure were recorded. Buffer base, standard bicarbonate and pH of arterial blood samples were determined and analyzed in order to assess the impact of operation on the animals. The hemodynamics were also monitored. Color Doppler ultrasonographic examination was performed on recipients before operation, intra-operation and 7 days after operation, respectively. Portal vein pressure, blood bilirubin and variables of liver function were measured by using an autoanalyzer. Wedge biopsy specimens of each pig were obtained, stained with hematoxylin-eosin, and examined. Analysis of variance was performed. Otherwise non-parametric tests were used. RESULTS: Eight weeks after ligation of the common bile duct, biliary cirrhosis was reproduced in all the pigs, and histopathological examination of the liver specimen showed a large number of pseudo-lobules. In 6 pigs with hepatic cirrhosis liver transplantation was done. Five of the 6 (83.3%) animals survived for 7 days. One recipient died because of unsuccessful operation, the others showed stable hemodynamics during the operation. Seven days after transplantation, the blood flow of the two liver portal veins was observed by the use of color Doppler ultrasonography. It was found that the blood flow in the donor portal vein was much richer than that of native portal vein. The venous outflow of the graft was ample and smooth, and no thrombosis of the hepatic vein or portal vein was found. The variables, including alanine aminotransferase, aspartate aminotransferase, bilirubin and total bilirubin, were significantly improved 7 days after operation compared with pre-operative data. Portal pressure was found to be (20.76+/-2.42)cm H(2)O(1 cm H(2)O=0.098 kPa), (17.62+/-2.33)cm H(2)O and (14.72+/-2.25)cm H(2)O before the operation, during the operation, and 7 days after operation, respectively, and the difference was statistically significant(P<0.01). On the 7 days after transplantation, histopathological examination revealed evidence of damage with mild steatosis and sporadic necrotic hepatocytes and focal hepatic lobular degeneration in the graft, especially in the area around the central vein. CONCLUSION: APOLT is a hopeful option for the treatment of portal hypertension. This procedure can provide not only some improvement of the liver function but also decrease the pressure of portal vein.

Animals↗

Hemorrhage from ruptured hepatic artery pseudoaneurysm co-occurring with pancreatic head tumor and portal vein thrombosis: surgical and endovascular treatment.

We report a case of 50-year-old man with a hepatic artery pseudoaneurysm, pancreatic head tumor, and portal vein thrombosis. The patient was operated on due to acute bleeding into duodenum. The pancreatic tumor was unresectable, which is why the ruptured pseudoaneurysm was only sutured. The second therapeutic step was to protect the hepatic artery against rupture. Therefore, a stent graft and then a wall graft endoprosthesis were placed across the pseudoaneurysm neck. The final angiogram demonstrated total exclusion of the pseudoaneurysm with preservation of the arterial lumen.

Aneurysm, False↗