THROMBOSIS AND HEPATIC INFARCT ELICITED IN HYPERLIPEMIC RATS BY BACTERIAL ENDOTOXIN.
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BACKGROUND: Massive thrombosis of hepatic veins is clinically the most serious type of Budd-Chiari syndrome (BCS). Ischemic impairment is the basic problem in case of acute or fulminate course of BCS. Restitution of blood drainage within the liver is a key therapeutic approach in such situation. In chronic course of the disease, symptoms of portal hypertension as ascites, G1 bleeding or hepatorenal syndrome are more common. The portosystemic shunt leads both to blood outflow restitution and to the decrease of portal hypertension. TIPS is a promising method due to minimal perioperative risk for the patient in critical situation and also due to its easiness of use. The aim of our study was to determine the clinical outcome in patients with BCS treated by TIPS in a retrospective analysis. METHODS AND RESULTS: During 12 years 23 patients with intraparenchymal thrombotic occlusion of hepatic veins were treated using TIPS, 17% were children, only 4 patients (17%) were men, the median age was 33.3 years (range 13 to 75 years). One third of the procedures was performed as urgent. In 2/3 of patients thrombosis developed in relation to myeloproliferative syndrome, in nearly 1/3 the origin of thrombosis was not detected. In 2 patients a defect of coagulation was revealed. In the first 11 patients the bare stent was used, the consecutive 12 patients received the ePTFE covered stent (stentgraft). Six patients died during follow-up: I due to fulminate liver failure, 2 due to liver failure caused by acute shunt occlusion, 1 due to the progression of the underlying hematooncological disease; the reason of death in 2 patients was not known. One patient was treated by OLTx during follow-up. The 17 surviving patients are in good condition with good shunt function although they need anticoagulant therapy and intermittent reinterventions. The average period between revisions was 2-3 years, 2 patients had no revision of TIPS for 4 years. The use of ePTFE covered stents had no effect on the number of early occlusions (approx. 18%), the occurrence of late stenoses and occlusions was substantially decreased (p=0.04, log-rank test). CONCLUSIONS: Standing on this experience we consider TIPS, in accordance with literature data, an advantageous therapeutic approach in Budd-Chiari syndrome caused by massive liver vein thrombosis. If the follow up treatment is rigorous, the TIPS usually ensures the necessary perfusion and the function of the liver So it may spare the patients of objectionable liver transplantation.
Forty-two cases of hepatic venous outflow obstruction with biopsied and autopsied specimens were studied. The morphological changes in the liver and the value of liver tissue in the diagnosis among the different subgroups of this conditions were evaluated. Seven cases in the group with veno-occlusive disease (VOD) showed prominent changes in the sublobular veins and terminal hepatic venules and were characterized by acute disruption of the vessel walls. Two cases showed thrombosis in which there were history of chemotherapy. In the group with Budd-Chiari's syndrome (BCS), in addition to the involvement of the large veins, thrombosis was observed in the small hepatic vein radicles in five out of six cases with myeloproliferative related disorders. Thrombosis was rarely present in cases associated with idiopathic and web lesions in BCS but frequent large vessel tumor thrombosis was noted in the group with malignant tumors. The hepatic parenchymal changes in the VOD group and the tumor related cases in BCS group were acute perivenular congestion, hemorrhage and coagulative necrosis, whereas the histological changes in other subgroups of BCS were perivenular scarring, dilatation of the sinusoids and rarely, reversed lobulation. Subtle differences were noted in the vascular system and the liver parenchyma between each subgroup. If good clinical information is available, it is possible to differentiate the various etiologies in hepatic venous outflow obstruction in the liver by morphology.
Hepatic arterial infusion chemotherapy has been often selected as a therapeutic option for advanced hepatocellular carcinoma with multiple intrahepatic metastases and/or portal vein thrombosis. We successfully treated and obtained CR in the 2 cases of far advanced hepatocellular carcinoma with intraarterial infusion chemotherapy (FAP). Case 1 was a 71-year-old male who had advanced hepatocellular carcinoma with intrahepatic metastasis (IM3) which was recurrent after two surgeries. He received hepatic arterial infusion chemotherapy (FAP: 5-fluorouracil 500 mg/day: continuous infusion, day 1-5, adriamycin 10 mg/day, day 1, CDDP 10 mg/day, day 1). After 10 courses, abdominal CT revealed that the viable lesions had completely disappeared (CR). This patient is still alive with no recurrence after 21 months from the beginning of this treatment. Case 2 was a 74-year-old male who had advanced hepatocellular carcinoma with portal vein thrombi (Vp4) and intrahepatic metastasis (IM3). He received FAP arterial infusion chemotherapy with the same protocol as case 1. After 8 courses of this therapy, CT revealed that these lesions had disappeared (CR). This patient is still alive with no recurrence after 9 months from the beginning of this treatment. For 15 patients with advanced hepatocellular carcinoma using a same protocol, the response rate of this therapy was 33.3% (CR & PR). These findings suggested that combined arterial infusion chemotherapy of FAP may be feasible and a promising modality for the advanced HCC with intrahepatic metastases and/or portal vein thrombosis.
UNLABELLED: Abnormal splanchnic circulation (ASC) is often detected too late, when hepatic circulation is already irreversibly compromised. If we could detect surgical or metabolic problems early after graft reperfusion, we might be able to correct them immediately before the damage becomes irreversible. The aim of this study was to determine if ASC can be predicted early after liver transplantation (LT) using portal vein pressure measurements and graft oxygen consumption monitoring. PATIENTS AND METHODS: Twenty-patients (13 men, 7 women of mean age 46 years) undergoing LT with the piggyback technique for hepatitis C virus (HCV)/hepatitis B virus (HBV)-related cirrhosis were retrospectively divided in two groups. Group A (16 patients), in which LT was successful, and group B (4 patients) in which LT was unsuccessful because of primary nonfunction (2 patients), infrahepatic portal vein thrombosis (1 patient), or hepatic vein kinking (1 patient). We then compared the portal blood pressure values and the prehepatic and posthepatic oxygen content difference (p-pDO(2)) before portal clamping; at the end of anhepatic phase; 5, 15, and 25 minutes after portal vein (PV) reperfusion; and 5, 20, 40, and 100 minutes after hepatic artery anastomosis. RESULTS: Early after graft reperfusion; portal pressure decreased to levels lower than that at baseline in group A, but remained high until the end of surgery in group B. At the end of surgery, p-pDO(2) increased more among group B than group A. CONCLUSION: ASC, specifically an increased PV resistance, can be predicted early after LT by portal vein pressure measurements and graft oxygen consumption monitoring.
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The antiphospholipid antibody syndrome is characterized by arterial and venous thrombosis including hepatic veins. Although transjugular intrahepatic portosystemic shunt or liver transplantation have been considered for Budd-Chiari syndrome, treatment options for patients with complete obstruction of three hepatic veins including the junction with the inferior vena cava are limited. We describe a 27-year-old female, who suffered thrombotic obliteration of hepatic veins including the portion confluent with the inferior vena cava (Budd-Chiari syndrome) associated with marked ascites and liver dysfunction. Transjugular intrahepatic portosystemic shunt using a Wall-stent (10 mm in diameter) between inferior vena cava and intrahepatic portal vein was performed. Intrastent coagulation and recurrence of thrombosis were prevented by combination therapy with warfarin potassium and ticlopidine hydrochloride. These treatments induced loss of ascites and improvement of liver function, and she has been able to resume daily life. The portosystemic shunt described above in addition to combination therapy with warfarin potassium and ticlopidine hydrochloride appeared to be one of the options for treating Budd-Chiari syndrome associated with antiphospholipid antibody syndrome.
We retrospectively evaluated 21 patients with Budd-Chiari syndrome who underwent liver transplant. The pathological findings were correlated with imaging studies that included computed tomography (CT) in all cases, sonography in 20, and magnetic resonance (MR) in 15. Pathological features of Budd-Chiari syndrome in subacute or chronic form, such as parenchymal fibrosis, hemorrhage, and congestion, were found in all resected livers. These occurred usually in conjunction with restricted hepatic veins due to thrombosis or fibrosis with partially recanalized lumen. The status of hepatic veins was correctly assessed and correlated with pathology in 13 of 20 patients who had sonograms, in 12 of 15 patients who had MR, and in nine of 18 patients with contrast-enhanced CT scans. Patency of the inferior vena cava was well seen by all three modalities; parenchymal abnormalities were best visible on CT (19 of 21), while ascites, caudate lobe enlargement and collateral vessels were best detected with MR or CT. We conclude that each imaging modality offers certain values and limitations in the assessment of vascular or parenchymal findings in Budd-Chiari syndrome.
Orthotopic hepatic transplantation has become a well-established treatment modality for end-stage liver disease, and research in this field is constantly evolving. Of the 34 canine liver transplants performed in this study, 17 (50%) survived more than 3 days (mean survival time 15 days). Causes of perioperative death included hemorrhage (4), anesthetic complications (3), systemic anaphylaxis (3), portal vein thrombosis (3), hepatic venous outflow block (2), and hepatic artery thrombosis (2). Gentle handling with minimal dissection of the donor liver in situ resulted in a decreased incidence of hepatic venous outflow block. The incidence of biliary leak was similar irrespective of the method of biliary reconstruction, although the incidence of acute cholangitis was 56% in the cholecystoduodenostomy group compared with 0% in the choledochocholedochostomy cohort. Using celiac to common hepatic end-to-side arterial anastomosis with preservation of the gastroduodenal artery, thrombosis of the hepatic artery was encountered in four instances, an incidence similar to previously reported studies where end-to-end hepaticohepatic arterial anastomosis or donor aortic conduit was utilized. The incidence of postoperative intestinal intussusception was reduced from 40 to 0% in those who underwent transmesenteric intestinal plication following implantation of the liver. Among short-term survivors, sepsis was the most frequent noted complication (10), followed by intestinal intussusception (6), rejection (6), and gastrointestinal bleeding (1). Among recipient dogs that survived more than 3 days, rejection was the most common cause of graft loss (5), followed by biliary leak (4) and hepatic artery thrombosis (2).
We report a case of Budd-Chiari syndrome following repair of a giant omphalocele. Thrombosis of hepatic veins and of retrohepatic inferior vena cava may result from direct pressure on the hepatic venous outlet after visceral reduction and final abdominal wall closure.
The occurrence of a portal vein thrombosis in a haemophilia A patient is reported. The patient, a 53 year old male, had been followed by us for the past 20 years in our out-patient Clinic. He was hospitalized recently for a suspected hepatic cirrhosis. Severe ascites, hepato-splenomegaly together with weight loss and mild fever were present. During the hospitalization, an ultrasound and CT scan of the liver confirmed the cirrhotic pattern and showed the presence of a portal vein thrombosis. There were no changes in the underlying coagulation defect, in fact, the patient had recurrent haemarthrosis. Furthermore, with the ultrasound examination, some focal hepatic lesions--probably due to a hepatocellular carcinoma--were also observed. The patient died because of massive haematemesis due to rupture of oesophageal varices.
AIM: To establish a new pig model for auxiliary partial orthotopic liver transplantation (APOLT). METHODS: The liver of the donor was removed from its body. The left lobe of the liver was resected in vivo and the right lobe was used as a graft. After the left lateral lobe of the recipient was resected, end-to-side anastomoses of suprahepatic inferior vena cava and portal vein were performed between the donor and recipient livers, respectively. End-to-end anastomoses were made between hepatic artery of graft and splenic artery of the host. Outside drainage was placed in donor common bile duct. RESULTS: Models of APOLT were established in 5 pigs with a success rate of 80%. Color ultrasound examination showed an increase of blood flow of graft on 5(th) d compared to the first day after operation. When animals were killed on the 5(th) d after operation, thrombosis of hepatic vein (HV) and portal vein (PV) were not found. Histopathological examination of liver samples revealed evidence of damage with mild steatosis and sporadic necrotic hepatocytes and focal hepatic lobules structure disorganized in graft. Infiltration of inflammatory cells was mild in portal or central vein area. Hematologic laboratory values and blood chemical findings revealed that compared with group A (before transplantation), mean arterial pressure (MAP), central venous pressure (CVP), buffer base (BB), standard bicarbonate (SB) and K(+) in group B (after portal vein was clamped) decreased (P<0.01). After reperfusion of the graft, MAP, CVP and K(+) restored gradually. CONCLUSION: Significant decrease of congestion in portal vein and shortened blocking time were obtained because of the application of in vitro veno-venous bypass during complete vascular clamping. This new procedure, with such advantages as simple vessel processing, quality anastomosis, less postoperative hemorrhage and higher success rate, effectively prevents ischemia reperfusion injury of the host liver and deserves to be spread.
We reviewed the CT findings in 17 patients with angiographically proved Budd-Chiari syndrome to determine the ability of CT to show acute thrombosis of the inferior vena cava (IVC) and hepatic veins. In eight patients with membranes (web or band) in the IVC, no thrombus was detected with CT or angiography. In the other nine patients, thrombi in the IVC and/or hepatic veins were seen as intraluminal filling defects that did not change in appearance on precontrast and postcontrast CT scans. Attenuation values of intraluminal filling defects of the IVC ranged from 38 to 42 H in four patients. High-attenuation intraluminal filling defects (60-70 H) of the IVC (five patients) and hepatic veins (one of five patients) were detected. Of these five patients, four had acute symptoms and one had chronic vague symptoms. The underlying disease was a web or band in the IVC and hepatic veins in three patients, invasive hepatocellular carcinoma in one, and injury to the IVC wall during hepatectomy in one. Inferior venacavography showed occlusion of the hepatic segment of the IVC in all five patients. Additional angiograms obtained by injection of contrast medium after a catheter tip was placed in the occluded hepatic IVC showed numerous filling defects suggestive of thrombi of recent onset, which correlated with the high-attenuation thrombi seen on CT scans in two patients. In the remaining three patients, high-attenuation areas in the IVC and hepatic veins also were considered to represent thrombi of recent onset because the attenuation values later decreased to 33-42 H. Spontaneous reduction in diameter of the thrombosed segment of the IVC was observed in four of the five patients. Knowledge of the CT features of acute thrombosis of the IVC and hepatic veins is useful in the early diagnosis of Budd-Chiari syndrome.
A methodology to evaluate routine ultrasonography performed in liver pathology is described. The results of 62 autopsies, undoubtedly the most accurate anatomical reference, were compared to those of sonographic examination of the liver performed two months before death at the most. Discordance was found in 23 cases. False negative results in the detection of metastasis and thrombosis of hepatic veins or inferior vena cava were the major pitfalls. The reasons and the consequences of each error were determined for each case.
BACKGROUND/PURPOSE: Extrahepatic portal vein thrombosis (EPVT) in children can lead to severe bleeding from gastrointestinal varices, ascites, thrombocytopenia from hypersplenism, and other coagulation disorders. The authors have used the superior mesenteric vein to intrahepatic left portal vein (Rex) shunt in 5 children with symptomatic EPVT and report their results with this novel technique. METHODS: Children with symptomatic portal hypertension were screened for the underlying cause. All children with essentially normal livers and obstruction of the extrahepatic portal vein were considered for the Rex shunt. Evaluation included liver function tests, liver biopsy, and radiological evaluation of the intrahepatic vascular anatomy. RESULTS: Five patients between the ages of 2.8 and 10.5 years underwent evaluation for portal hypertension secondary to extrahepatic portal vein obstruction. Three patients had idiopathic extra hepatic portal vein thrombosis with cavernous transformation, 1 had thrombosis after a living-related liver transplant, and 1 had compression and obstruction of the main portal vein from enlarged lymph nodes after treatment of systemic histoplasmosis. All patients were symptomatic. Three patients had intermittent bleeding from esophageal and gastric varices, and all 5 had relative degrees of hypersplenism with enlarged spleens and thrombocytopenia (11,000 to 77,000). Three patients had significant leukopenia. Results of imaging studies suggested that 3 patients had inadequate intrahepatic portal veins for shunting, but all patients at exploration underwent successful shunting. There were no serious intraoperative complications. Postoperative complications included ascites in 2 patients that resolved within 1 month. There were no early shunt thromboses. The median postoperative length of stay was 7 days. Clinical follow-up ranged from 7 to 21 months. Gastrointestinal bleeding did not recur in any patient, and ascites resolved in all. Spleen size decreased significantly (P < .01) from 9.4 +/- 4.0 cm to 5.0 +/- 3.7 cm below the left costal margin. Mean platelet count and white blood cell count rose after shunting from 79 +/- 42 to 176 +/- 73 (P < .02) and 5.4 +/- 2.3 to 7.5 +/- 3.9 (P = .06), respectively. All shunts were studied at 1 and 7 days, and 3 and 6 months after the procedure. Shunt patency was documented in all cases. Subsequently, shunt blockage occurred in 2 patients. CONCLUSIONS: The Rex shunt has proven to be an effective method of resolving portal hypertension caused by EPVT including thrombosis after living donor transplantation. This shunt is preferable to other surgical procedures because it eliminates portal hypertension and its sequelae by restoring normal portal flow to the liver.
We have reported the cases of two patients who had acute pylephlebitis associated with portal vein thrombosis and septic hepatic emboli as a result of right colonic diverticulitis. Although rare, pylephlebitis is a treatable but often lethal complication of intra-abdominal sepsis. Several bacterial pathogens, especially Escherichia coli are associated with pylephlebitis. Early suspicion and prompt antibiotic therapy can lead to resolution of portal vein thrombosis and hepatic abscess formation, resulting in full recovery for the patient. Surgery may not be required. Our two patients received ampicillin--the best first-line drug--until specific antibiotic therapy could be given. Early administration of a broad spectrum antibiotic is essential.
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