Obstruction of the portal or hepatic veins and primary myeloproliferative disorders.
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Hepatic Veins↗
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Results for “HEPATIC VEIN THROMBOSIS”
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[Orthotopic liver transplantation at the Island Hospital. Initial experiences 1985-1990].
Between 1985 and 1990 22 orthotopic liver transplantations (OLT) were realized in 19 patients. Active infection and diffuse splanchnic venous thrombosis were the only contra-indications to the intervention. Sixteen patients were transplanted electively; three had to be retransplanted urgently. Three patients had an urgent primary transplant. The incidence of surgical complications related to liver implantation was fair. One patient (5%) developed a late portal vein thrombosis; another patient (5%) had to be retransplanted because of hepatic artery thrombosis. All patients presented one or more major postoperative complications. All, but one, patients had a rejection of the allograft; five of them needed treatment with mono- or polyclonal antilymphocytic sera to reverse the rejection. One patient was retransplanted because of a hyperacute rejection. The six-month survival in this series is 68.5% (13 of 18 patients); one patient died 7 months post-OLT due to a neurological complication of her Wilson disease. Quality of life (from 6 to 64 months post-OLT) is excellent in the 12 long-term survivors. This small experience of the Bernese transplantation program shows that liver transplantation is a safe surgical procedure allowing excellent quality of life in a majority of patients.
Adult↗
[Clinical features of Budd-Chiari syndrome].
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Adolescent↗
Retrohepatic cavoatrial bypass for coarctation of inferior vena cava with a polytetrafluoroethylene graft.
Eight patients with chronic Budd-Chiari syndrome resulting from coarctation of the inferior vena cava underwent operation. Transatrial dilatation was of no avail in the first case. The obstructed segment was directly visualized in the subsequent seven cases by a transthoracic, transdiaphragmatic, retroperitoneal approach. In these latter seven cases, severe hourglass constriction of the inferior vena cava was observed just above the right hepatic vein. There was no evidence of inflammation, extrinsic compression, or thrombosis. Retrohepatic cavoatrial bypass with an antibiotic-sterilized aortic homograft was unsuccessful in three patients. Five patients including one with homograft failure underwent successful retrohepatic cavoatrial bypass with a polytetrafluoroethylene graft (20 mm plain in four cases and 16 mm ringed graft in one case). These patients have been followed up for 21 months to 6 years with no recurrence of symptoms. The term coarctation of the inferior vena cava appears more appropriate than membranous obstruction of the inferior vena cava because of the operative findings in the present series.
Adult↗
Unusual outcome of percutaneous transhepatic cholangiography.
In a 72-year old male with fever, mild jaundice and moderate enlargement of both liver and spleen, percutaneous transhepatic cholangiography resulted in direct filling of the portal vein system. Both trunc and left and right hepatic lobe branches showed a typical pattern of incomplete portal vein thrombosis, with mural irregularities and radiolucencies within the lumen. On subsequent operative cholangiography the biliary tract was shown to be normal. At autopsy multiple intrahepatic abscesses of pylephlebitic origin were found. The significance of visualization of the portal vein system during PTC is briefly discussed in comparison with other angiographic technics.
Aged↗
Mesoatrial shunt: a prosthesis modification.
The mesoatrial shunt is the indicated treatment in the Budd-Chiari syndrome when the inferior vena cava is occluded and not usable for the standard portasystemic shunts. As the mesoatrial shunt courses from the abdomen into the right side of the chest, it passes posterior to the xiphoid and sternum and frequently is compressed at this point. A prosthesis modification is reported whereby an exterior silicone rubber cuff is bonded to the Dacron or Gortex graft at the point at which it passes posterior to the sternum. This obviates the possibility of graft compression and should increase long-term patency.
Adult↗
Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound.
Computed tomography and real-time ultrasonography may not be conclusive for the diagnosis of the Budd-Chiari syndrome; in many cases more information may be needed, especially on vascular alterations. Doppler ultrasonography provides qualitative data on flow direction and pattern, thereby contributing significantly to diagnosis. Eight cases in which hepatic vein patency was unclear and presence of intrahepatic vessels resembling hepatic veins raised problems of interpretation in real-time ultrasonography are described. In some cases, patency or occlusion of the upper portion of the inferior vena cava were difficult to identify with real-time ultrasonography. Doppler ultrasonographic investigation showed flow in the hepatic veins to be completely absent in two cases and reversed in another two. In the remaining four cases, a flat waveform was evident. Flow in the inferior vena cava was reversed in four cases and showed a flat waveform in three other cases. Portal vein thrombosis was detected in only one case, whereas the remaining seven patients showed slow hepatopetal flow. These findings demonstrate that absent or reversed flow in the hepatic veins and/or flat flow in the hepatic veins associated with reversed flow in the inferior vena cava may be considered diagnostic for the Budd-Chiari syndrome. For this series the sensitivity of Doppler ultrasonography was 87.5%.
Adult↗
Sinistral (left-sided) portal hypertension.
Between 1953 and 1988, 21 patients with splenic vein thrombosis (SVT), 12 of whom had sinistral portal hypertension (SPH) were treated at our institution. SVT was identified at autopsy in nine patients. Twelve additional patients presented with SPH: bleeding esophageal varices, SVT and normal hepatic function. SVT was caused by pancreatic neoplasm (5), chronic pancreatitis (5), and pancreatic pseudocyst (2). SVT was diagnosed by splanchnic angiography, splenoportography, computerized tomography, and ultrasonography. Gastric varices were diagnosed by endoscopy (10) and barium swallow (2). Splenectomy was performed as primary therapy in 10 patients. Three of these 10 had en block distal pancreatectomy. Two high-risk patients had splenic artery embolization, one as a prelude to splenectomy performed 48 hours later and the other as definitive therapy. One splenectomized patient continued to bleed. No further bleeding occurred in 10 splenectomized patients in follow-up from 1 week to 14 years. Sinistral portal hypertension is a clinical syndrome of splenic vein thrombosis caused by pancreatic pathology and manifests as bleeding gastric varices in patients with a patent portal vein and normal hepatic function. Splanchnic arteriography is necessary for accurate diagnosis. Splenectomy is the effective treatment of choice.
Adult↗
Prognosis of hepatocellular carcinoma: comparison of 7 staging systems in an American cohort.
Currently there is no consensus as to which staging system is best in predicting the survival of patients with hepatocellular carcinoma (HCC). The aims of this study were to identify independent predictors of survival and to compare 7 available prognostic staging systems in patients with HCC. A total of 239 consecutive patients with cirrhosis and HCC seen between January 1, 2000, and December 31, 2003, were included. Demographic, laboratory, and tumor characteristics and performance status were determined at diagnosis and before therapy. Predictors of survival were identified using the Kaplan-Meir test and the Cox model. Sixty-two percent of patients had hepatitis C, 56% had more than 1 tumor nodule, 24% had portal vein thrombosis, and 29% did not receive any cancer treatment. At the time of censorship, 153 (63%) patients had died. The 1- and 3-year survival of the entire cohort was 58% and 29%, respectively. The independent predictors of survival were performance status (P < .0001), MELD score greater than 10 (P = .001), portal vein thrombosis (P = .0001), and tumor diameter greater than 4 cm (P = .001). Treatment of HCC was related to overall survival. The Barcelona Clinic Liver Cancer (BCLC) staging system had the best independent predictive power for survival when compared with the other 6 prognostic systems. In conclusion, performance status, tumor extent, liver function, and treatment were independent predictors of survival mostly in patients with cirrhosis and HCC. The BCLC staging system includes aspects of all of these elements and provided the best prognostic stratification for our cohort of patients with HCC.
Carcinoma, Hepatocellular↗
Portal and mesenteric vein thrombosis in a patient heterozygous for a mutation (Arg506-->Gln) in the factor V gen (factor V Leiden).
In 30-50% of patients with portal thrombosis, no underlying etiology is found. The recent reports of new hereditary clotting defects are contributing to the understanding of this problem, but they only justify a small number of idiopathic cases. Instead, anticoagulant protein C resistance, caused by a mutation in the V factor gene, appears to be at least 10 times more common than any of the other known inherited deficiencies of anticoagulant proteins. In spite of that, extensive thrombosis of portomesenteric or hepatic venous circulation has been rarely described in this hereditary clotting defect. We report a typical case of familial and recidivant deep vein thrombosis in a young man heterozygous for the factor V Leiden mutation (Arg506-Gln), who developed an acute portal and mesenteric vein thrombosis. The patient was discharged with an oral anticoagulant treatment and remains asymptomatic 2 years later. In conclusion, the high prevalence of the factor V Leiden in young and aged patients with idiopathic vein thrombosis and the case here described makes it obligatory to consider this disorder in patients with portal and/or mesenteric vein thrombosis, especially in those without evident etiology.
Adult↗
Risk factors for intraoperative portal vein thrombosis in pediatric living donor liver transplantation.
Pathologic changes of the recipient native portal venous system may cause thrombosis of the portal vein, especially in pediatric living donor liver transplantation (LDLT). This study assessed the utility of Doppler ultrasound (US) for the detection of intraoperative portal vein occlusion and identification of predisposing risk factors in the recipients. Seventy-three pediatric recipients who underwent LDLT at Chang Gung Memorial Hospital, Taiwan, from 1994 to 2002 were included. Preoperative and intraoperative Doppler US evaluation of the portal vein was performed. Age, body weight, native liver disease, type of graft, graft recipient weight ratio (GRWR), type of portal anastomosis, portal velocity, portal venous size and presence of portosystemic shunt were analyzed for statistical significance of predisposing risk factors. Eight episodes of intraoperative portal vein thrombosis, with typical findings of absent Doppler flow in portal vein and prominent hepatic artery with a resistant index lower than 0.5 (p < 0.001), were detected during transplantation, which was then corrected by thrombectomy and re-anastomosis. Children age < or =1 yr (p = 0.025), weight < or =10 kg (p = 0.024), low portal flow < or =7 cm/s (p = 0.021), portal venous size < or =4 mm (p = 0.001), and GRWR >3 (p < 0.017) were all risk factors for intraoperative portal vein thrombosis. Doppler US is essential in the preoperative evaluation, early detection and monitoring of outcome of the portal vein in liver transplant.
Adolescent↗
[Hepatic encephalopathy following portal thrombosis associated with type B liver cirrhosis].
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Female↗
Diagnosis of hepatocellular carcinoma by fine needle biopsy of portal vein thrombosis.
The authors describe a case of HBsAg positive liver cirrhosis and elevated alpha-fetoprotein value (AFP) where imaging techniques failed to detect focal liver changes. Diagnosis of hepatocellular carcinoma was reached by ultrasound-guided fine needle aspiration biopsy of portal vein thrombosis.
Biopsy, Needle↗
Radiological diagnosis of bleeding esophageal varices.
With bleeding esophageal varices radiological investigation of the underlying cause, mostly portal hypertension, is necessary. The cross-sectional imaging methods are suitable for non-invasive differentiation of the various types of underlying obstruction. Angiography may be necessary to verify the pathological findings (i.e. the extent of thrombosis), for complete demonstration of the topographical anatomy and assessment of hemodynamic parameters, and is thus an integral part of the diagnostic work-up before shunt surgery.
Angiography↗
Percutaneous stent placement as treatment of renal vein obstruction due to inferior vena caval thrombosis.
A patient who had undergone his third orthotopic liver transplantation nearly 9 years prior to presentation developed worsening hepatic and renal function, as well as severe bilateral lower extremity edema. Magnetic resonance imaging demonstrated vena caval thrombosis from the suprahepatic venous anastomosis to the infrarenal inferior vena cava, obstructing the renal veins. This was treated by percutaneous placement of metallic stents from the renal veins to the right atrium. At 16 months clinical follow-up, the patient continues to do well.
Adult↗
Liver transplant in Hawaii: the survival of a small centre.
Although many report the importance of case volume in complex cases, liver transplantation (LT) can be carried out successfully in a small centre. During a 11.5-yr period, 88 patients underwent LT in a single transplant centre in Hawaii. Indications for LT were primarily hepatitis C (n = 49) and hepatitis B (n = 13) and 22 patients (25%) had hepatocellular cancer (HCC) on explanted liver. There was no primary graft nonfunction, one retransplant for recurrent hepatitis C and two late hepatic artery thromboses, which did not require a retransplant. One patient developed partial portal vein thrombosis related to a hypercoagulable state and was rescued with anticoagulation. Of the 22 patients with HCC, 18 are alive, two died from recurrent disease (253 and 1428 d post-LT, respectively), one died because of a ruptured hepatic artery aneurysm (151 d) and one from complications caused by noncompliance (723 d). One-, 3- and 5-yr survival rates were 89%, 82% and 71%, respectively. Mean survival was 3034.9 d. During this time period, 142 liver resections, 77 pancreatic resections and 43 splenorenal shunts were performed by this group of surgeons. Because of the recent explosion of information on case volumes and centres of excellence, LT can be performed successfully at a small centre. Other major hepatobiliary/transplant procedures can help the surgeons maintain their operative skills. A smaller LT program may require a longer period of evolution, but it can provide a service for a geographically isolated population that would otherwise have limited opportunity for LT.
Adolescent↗
Tumor involvement in hepatic veins: comparison of MR imaging and US for preoperative assessment.
PURPOSE: To compare use of magnetic resonance (MR) imaging and ultrasonography (US) for diagnosis of vascular involvement by tumor at the hepatic vein confluence. MATERIALS AND METHODS: Thirty-seven consecutive patients with tumors at the hepatic vein confluence were prospectively evaluated with spin-echo and gradient-echo MR imaging and gray-scale and Doppler US. Encasement, thrombosis, occlusion, and nonvisualization were considered to be evidence of vascular involvement. Imaging results were compared with surgical and pathologic examination findings in 27 patients who underwent resection. RESULTS: Sixteen hepatic veins (nine right, four middle, three left) were seen to be involved at surgery. Twelve of 16 involved veins were identified at MR imaging (75% sensitivity, 98% specificity, 92% positive predictive value, 94% negative predictive value). Thirteen of 16 involved veins were detected at US (81% sensitivity, 97% specificity, and 87% positive and 95% negative predictive values). There was one false-positive diagnosis of inferior vena cava involvement at both MR imaging and US. Ten patients had unresectable disease. One patient had motion artifact on MR images; in the remaining nine patients, MR imaging and US yielded identical findings at 26 of 27 hepatic vein sites. CONCLUSION: MR imaging and US provide comparable results for assessment of hepatic vein involvement by tumor.
Bile Duct Neoplasms↗
Isolated pancreatectomy for pancreatic head carcinoma using catheter bypass of the portal vein.
We investigated perioperative complications and survival rate in 114 patients with pancreatic cancer who underwent surgical resection between July, 1981 and March, 1992 with an antithrombogenic bypass catheter of the portal vein which we have developed. With this catheter, mesenteric venous blood can be bypassed to the systemic circulation or intrahepatic portal vein to prevent portal congestion or hepatic ischemia during portal obstruction or simultaneous obstruction of the hepatic artery. All the arteries supplying, and veins draining, the pancreas are dissected prior to its manipulation, following which isolated pancreatectomy accompanied by portal vein resection can be performed. Eighty patients (70%) also underwent isolated pancreatectomy. The perioperative death rate was 9.6%, and the main factor contributing to mortality with the bypass method was not portal vein resection, but various postoperative complications, such as insufficiency of the pancreatojejunostomy and thrombosis of the hepatic artery or superior mesenteric artery after resection and reconstruction. We conclude that aggressive surgery with the bypass method increases the operability of pancreatic cancer, although auxiliary therapies combined with radical surgery are necessary to improve the therapeutic outcome for this cancer.
Catheterization↗