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PubMed1980

[Liver vein obstruction. Clinical aspects and diagnosis of the Budd-Chiari syndrome].

In recent years we have observed 9 cases of Budd-Chiari syndrome. The diagnostic, etiological and therapeutic aspects of this rare hepatic vein disease are discussed. 3 patients had a neoplasm, and 1 female had polycythemia; she had been on oral contraceptives. 1 patient had osteomyelosclerosis. 4 females exhibited thrombosis of the hepatic veins; 2 of them had been on oral contraceptives. Liver scan has proven to be an adequate method for follow-up studies in demonstrating the hypertrophied caudate lobe. Very rarely, the small and medium-sized hepatic veins are involved, most often after ingestion of certain plant toxins. This syndrome is termed the Stuart-Bras syndrome or "veno-occlusive disease".

Adult↗
PubMedDate not supplied

Contrast-enhanced three-dimensional MR portography.

Three-dimensional (3D) magnetic resonance (MR) portography with contrast material enhancement is a fast means of evaluating the portal venous system that has some advantages over currently used modalities, such as digital subtraction angiography, helical computed tomography, ultrasonography, and nonenhanced MR angiography with time-of-flight and phase-contrast techniques. With contrast-enhanced 3D MR portography, a first-pass study of the mesenteric vasculature is performed after rapid bolus injection of gadopentetate dimeglumine; a 3D fast field echo sequence is used, which can demonstrate the intrahepatic and extrahepatic portal venous system clearly. Repeated sequences after administration of gadopentetate dimeglumine allow separate demonstration of the splanchnic arteries and portomesenteric veins. The images are reconstructed by means of maximum-intensity projection postprocessing, and a subtraction technique can be used to eliminate arterial enhancement and demonstrate portosystemic shunts. The coronal source images simultaneously demonstrate parenchymal lesions of the liver, pancreas, biliary tract, and spleen. This technique is clinically indicated in portosystemic shunt, portal vein thrombosis, hepatocellular carcinoma, pancreatobiliary tumor, hepatic vein obstruction, differentiation of splanchnic arterial from portal venous disease, and gastrointestinal hemorrhage. Its limitations include allergic reactions to contrast media, inappropriate positioning of the 3D acquisition slab, respiratory motion artifacts, and pseudodissection.

Contrast Media↗
PubMed1993

[Thrombolytic treatment of Budd-Chiari syndrome with portal venous thrombosis].

Own material of 7 patients is presented pathology: venous thrombosis involving both hepatic veins and portal system, resulting in severe impairment of liver function and portal circulation, leading up to a fatal end (if not treated). Neither surgical decompression nor liver transplantation is feasible from the technical point of view. Patients were treated with activators of fibrinolysis. In four cases the treatment was effective and resulted in recanalisation of the previously occluded veins. Early re-thrombosis was the reason of death in one case. One patient died 4 years later because of cerebral vascular thrombosis. Two others live over 5 years, symptom-free. The authors consider thrombolytic treatment as a life saving procedure in cases of hepatic and portal venous thrombosis.

Acenocoumarol↗
PubMed2005

[Efficacy of repeated hepatic arterial infusion chemotherapy in advanced hepatocellular carcinoma with portal vein tumor thrombosis].

BACKGROUND/AIMS: The aim of this study is to elucidate the efficacy of repeated hepatic arterial infusion chemotherapy (HAIC) and different chemotherapeutic regimens for treating patients having advanced hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT). METHODS: From Jan. 1999 and Dec. 2003, a total of 103 patients diagnosed as having HCC with PVTT, but without extrahepatic spreading, were enrolled in this study. They were stratified into two groups. Group I (67 patients) received intraarterial cisplatin (CDDP, 80 mg/m2 for 2 hours on Day 1), Group II (36 patients) received intraarterial CDDP (60 mg/m2 for 2 hours on Day 2) and 5-fluorouracil (5-FU, 500 mg/m2 for 5 hours on Day 1-3). They were scheduled to receive at least three consecutive courses of the HAIC at 1 month intervals. RESULTS: Among the 66 patients who completed the protocol, one (2.5%) and seven (17.5%) patients of group I, and one (3.8%) and four (15.4%) of group II, exhibited complete and partial responses, respectively. The median survival period of all the patients was 6 months. Group II showed a tendency to improve the median survival compared to group I (8.5 vs 5.0 months, respectively, P=0.45). The most common adverse reaction was nausea (58.2%). However, an elevation of the total bilirubin level was more frequent in Group I than in Group II (61.3% vs 20.7%, respectively, P<0.05). CONCLUSIONS: Repeated HAIC using CDDP achieved favorable results in a few patients with HCC with PVTT, and additional 5-FU may be useful.

Adult↗
PubMed1985

Pediatric liver transplantation. Part II. Diagnostic imaging in postoperative management.

The postoperative diagnostic imaging examinations of 44 children who underwent 59 orthotopic liver transplantations were reviewed. The imaging modalities used for the evaluation of suspected complications include plain roentgenography, ultrasonography (US), computed tomography (CT), nuclear scintigraphy, arteriography, percutaneous and operative cholangiography, and endoscopic retrograde cholangiopancreatography. The main postoperative complications included ischemia, thrombosis (hepatic artery and portal vein), infarction, obstruction or leakage of the biliary anastomosis, hepatic and perihepatic infection, and allograft rejection. US, the most frequently used abdominal imaging modality, was best suited for detection of biliary duct dilatation, fluid collections in or around the transplanted liver, and hepatic arterial, inferior vena caval, and portal vein thrombosis. CT was especially helpful in corroborating findings of infection and in locating abscesses. Technetium 99m sulfur colloid (early- and late-phase imaging) provided a sensitive, although nonspecific, means of assessing allograft vascularization and morphology. Angiography showed vascularity most clearly, and cholangiography was the most useful in the assessment of bile duct patency. A diagnostic imaging algorithm is proposed for evaluation of suspected complications.

Adolescent↗
PubMed2002

Budd-Chiari syndrome due to prothrombotic disorder: mid-term patency and efficacy of endovascular stents.

Our objective was to evaluate efficacy and patency of metallic stent placement for symptomatic Budd-Chiari syndrome (BCS) due to prothrombotic disorders. Eleven patients with proved BCS due to prothrombotic disorders were referred for endovascular treatment because of refractory ascites (n=9), abdominal pain (n=8), jaundice (n=6), and/or gastrointestinal bleeding (n=4). Stents were inserted for stenosed hepatic vein (n=7), inferior vena cava (n=2), or mesenterico-caval shunt (n=2). Clinical efficacy and stent patency was evaluated by clinical and Doppler follow-up. After a mean follow-up of 21 months, 6 patients had fully patent stents without reintervention (primary stent patency: 55%). Two patients with hepatic vein stenosis had stent thrombosis and died 4 months after procedure. Restenosis occurred in 3 cases (2 hepatic vein and 1 mesenterico-caval shunt stenosis) and were successfully treated by balloon angioplasty (n=2) and addition of new stents (n=1) leading to a 82% secondary stent patency. Of 9 patients with patent stent, 7 were asymptomatic (77%) at the end of the study. Stent placement is a safe and effective procedure to control of symptomatic BCS. Prothrombotic disorder does not seem to jeopardize patency in anticoagulated patients.

Adult↗
PubMed2006

Favorable resolution of hepatic infarctions in transplanted liver after portal vein thrombosis treated by surgical thrombectomy: a case report.

Portal vein thrombosis (PVT) after orthotopic liver transplantation (OLT) is a life-threatening complication associated with a high rate of graft loss and patient death, with reported incidence of 1% to 2% in adults. We report a case of an early PVT after OLT complicated by hepatic infarctions in the liver graft. After surgical thrombectomy and restoration of the portal inflow, hepatic infarctions resolved spontaneously within 6 months, which was confirmed by computed tomography.

Humans↗
PubMed2005

Donation after cardiac death: the University of Wisconsin experience with liver transplantation.

OBJECTIVE: To determine whether the outcomes of liver transplantation (LTx) from donation after cardiac death (DCD) donors are equivalent to those from donation after brain death (DBD) donors. SUMMARY BACKGROUND DATA: Because of the significant donor organ shortage, more transplant centers are using livers recovered from DCD donors. However, long-term, single-center outcomes of liver transplantation from DCD donors are limited. METHODS: From January 1, 1993, to July 31, 2002, 553 liver transplants were performed from DBD donors and 36 were performed from DCD donors. Differences in event rates between the groups were compared with Kaplan-Meier estimates and the log-rank test. Differences in proportion and differences of means between the groups were compared with Fisher exact test and the Wilcoxon rank sum test, respectively. RESULTS: Mean warm ischemic time at recovery in the DCD group was 17.8 +/- 10.6 minutes. The overall rate of biliary strictures was greater in the DCD group at 1 year (33% versus 10%) and 3 years (37% versus 12%; P = 0.0001). The incidence of hepatic artery thrombosis, portal vein stenosis/thrombosis, ischemic-type biliary stricture (ITBS), and primary nonfunction were similar between groups. However, the incidence of both hepatic artery stenosis (16.6% versus 5.4%; P = 0.001) and hepatic abscess and biloma formation (16.7% versus 8.3%; P = 0.04) were greater in the DCD group. Trends toward worse patient and graft survival and increased incidence of ITBS were seen in DCD donors greater than 40 years compared with DCD donors less than 40 years. Overall patient survival at 1 year (DCD, 80%; versus DBD, 91%) and 3 years (DCD, 68%; versus DBD, 84%) was significantly less in the DCD group (P = 0.002). Similarly, graft survival at 1 year (DCD, 67%; versus DBD, 86%) and 3 years (DCD, 56%; versus DBD, 80%) were significantly less in the DCD group (P = 0.0001). CONCLUSIONS: Despite similar rates of primary nonfunction, LTx after controlled DCD resulted in worse patient and graft survival compared with LTx after DBD and increased incidence of biliary complications and hepatic artery stenosis. However, overall results of LTx after controlled DCD are encouraging; and with careful donor and recipient selection, LTx after DCD may successfully increase the donor liver pool.

Adult↗
PubMed1992

Long-term results of treatment of Budd-Chiari syndrome with portal decompression.

Thirty-three patients with Budd-Chiari syndrome were studied for 1 to 19 years following portal decompression. All had ascites, hepatomegaly, abnormal liver function, angiographic demonstration of inferior vena cava and/or hepatic vein occlusion, and biopsy specimens showing intense hepatic congestion and necrosis. When thrombosis was confined to hepatic veins (20 patients), side-to-side portacaval shunt resulted in 95% operative survival, 90% prolonged survival, permanent shunt patency, relief of ascites, reversal of liver dysfunction, and reversal or improvement of hepatic lesions. When thrombosis involved the inferior vena cava, mesoatrial shunt (eight patients) was unsatisfactory because of a 63% mortality rate from liver failure due to shunt thrombosis. In contrast, a new procedure consisting of combined portacaval and caval-atrial shunts (five patients) has been highly successful, with 100% survival, shunt patency, relief of ascites, and reversal of pathologic abnormalities.

Adult↗
PubMed1998

Bright basal ganglia in T1-weighted magnetic resonance images are frequent in patients with portal vein thrombosis without liver cirrhosis and not suggestive of hepatic encephalopathy.

BACKGROUND/AIMS: Deposition of paramagnetic substances in basal ganglia, resulting in increased signals in T1-weighted magnetic resonance images (bright basal ganglia), is frequently seen in liver cirrhosis. The present study describes the prevalence of bright basal ganglia and its clinical significance in patients with long-standing portal vein thrombosis in the absence of liver cirrhosis. METHODS: Six patients with angiographically proven complete portal vein thrombosis and cavernomatous transformation without signs of acute or chronic liver disease were studied by magnetic resonance imaging of the brain, neuropsychiatric evaluation, psychometric tests, electroencephalography, and determination of arterial ammonia levels and of serum manganese concentrations from peripheral venous blood. RESULTS: Five out of six patients demonstrated increased signal intensity in the basal ganglia. Overt portal-systemic encephalopathy was not noted prior to or at the time of evaluation. Normal EEG results were recorded in all patients. Only one of the six patients had pathological results in at least two out of four psychometric tests. This latter patient had had a large right-sided brain infarction. Arterial ammonia concentrations were normal in four of the six patients; one patient with increased ammonia levels had concomitant renal insufficiency with azotemia. The other four patients had no relevant concomitant diseases. Serum manganese levels were non-significantly increased compared with a control group (p=0.06), but they were significantly correlated to basal ganglia signal intensity (R=0.88; p=0.02). CONCLUSIONS: Our results demonstrate that bright basal ganglia primarily represent shunt-induced alterations. They are not directly associated with disturbed liver function nor with portal-systemic encephalopathy.

Adult↗
PubMed1997

Natural anticoagulants and the liver.

The regulation of blood coagulation is dependent on a complex interplay between procoagulant, anticoagulant and fibrinolytic proteins. Most of these proteins are synthesised in the liver and their levels are altered in patients with liver disease. The liver also plays an important role in the regulation of haemostasis throughout the clearance of activated clotting factors. It is therefore not surprising that the critically balanced coagulation system is dysregulated in patients with liver disease. In moderate liver failure bleeding disorders predominate, whereas in more advanced liver disease intravascular coagulation is commonly observed and contributes to the overall dysregulation of blood coagulation. In some patients, liver disease can be primarily caused by an abnormality of the coagulation system. These patients usually have a hypercoagulable state caused by a deficiency of a component of the natural anticoagulant system. These include protein C, protein S and antithrombin III. More recently, activated protein C resistance caused by a point mutation in the Factor V gene has been identified as an important risk factor for thrombosis. In these patients the abnormal Factor V is resistant to cleavage by activated protein C resulting in ongoing uncontrolled procoagulant drive. Both hepatic and portal vein thrombosis have been reported in these patients. Appropriate management of these patients should include a thorough assessment of their natural anticoagulant proteins and exclusion of activated protein C resistance as the cause of their thrombotic disorder.

Antithrombin III↗
PubMed2003

Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients.

Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out in all patients. Macroaggregated albumin lung perfusion scans were performed in patients with positive contrast echocardiogram. Hepatopulmonary syndrome was detected in 5 (10.8%) cirrhotic and 3 (9.7%) noncirrhotic portal hypertensive patients (2 idiopathic portal hypertension, 1 portal vein thrombosis). All patients with hepatopulmonary syndrome had an increased shunt fraction (13-62%) and a decreased diffusion capacity of carbon monoxide (40-79%), and 7 of them were hypoxemic (PaO2, 31.6-69.8 mm Hg). These findings show that hepatopulmonary syndrome may occur in both liver cirrhosis and noncirrhotic portal hypertension and that portal hypertension is the predominant etiopathogenic factor related to hepatopulmonary syndrome.

Adolescent↗
PubMed1989

Intensive care for children after orthotopic liver transplantation.

We report our experience in the management of children after orthotopic liver transplantation (OLT). From 03/84 to 04/87 50 patients (pts) were transplanted. Mean age was 4 3/12 years (8/12 to 13 2/12) and mean body weight 14.7 kg (5.8 to 40). Hospital mortality was 14%. Problems related to the surgery included: Abdominal complications: bleeding (8 pts), infection (18 pts), ascites and fistula (1 pt), need for secondary abdominal surgery (10 patients). Respiratory problems: lobar atelectasis (11 pts), right diaphragmatic paralysis (2 pts) and right pleural effusion (11 pts). Problems related to immunosuppression included: Bacterial infection (29 pts) fungal infection (5 pts), one patient died of disseminated cytomegalovirus infection. Side effect of cyclosporin A (CsA) were systemic blood hypertension (S.B.H.) (47 pts), sinusal bradycardia (37 pts), associated to SBH (24 pts), hypertensive encephalopathy (2 pts). Generalized seizures (2 pts in the absence of SBH). Renal side effects of CsA were hypercreatininemia, decreased sodium bicarbonate and hyperkaliemia. The nephrotoxicity of CsA was favoured by the use of other nephrotoxic drugs such as aminoglycosides, amphotericin B. Edematous pancreatitis was observed in 3 patients and related to the use of large doses of steroids. Problems related to the functioning of the graft included: Primary non-function of the graft (4 pts), hepatic artery thrombosis (8 pts) and severe acute rejection unresponsive to therapy (1 pt); these situations needed to be recognised early in order to organize a second OLT. Other causes of hepatic dysfunction were: portal vein thrombosis (1 pt), biliary tract obstruction (2 pts), angiocholitis (3 pts), right hepatic lobe necrosis (2 pts). Acute hepatic insufficiency in 7 children.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1997

Portosystemic shunting for paediatric portal hypertension.

The records of 22 patients who received portosystemic shunting for portal hypertension from 1985 to 1995 inclusive at the Royal Alexandra Hospital for Children (RAHC) were retrospectively reviewed. There were 11 girls and 11 boys. The average age at operation was 8 years, 3 months (range, 2 years, 3 months to 16 years, 7 months). The aetiology was idiopathic portal cavernomatous transformation (n = 9), billiary atresia (n = 4), cystic fibrosis (n = 3), documented neonatal portal vein thrombosis (n = 3), congenital hepatic fibrosis (n = 2), and portal vein obstruction after liver transplant (n = 1). The major presenting problem was upper gastrointestinal haemorrhage. Two patients had recurrent melaena from Roux-en-Y jejunal loop and caecal varices, respectively. Before receiving shunts, 12 patients had endoscopic sclerotherapy, 1 had gastric transection, and 2 had gastric varices oversewn. Portal pressure at preoperative splenoportogram averaged 28 mm Hg (range, 20 to 41). Urgent shunts were performed on 13 patients. Two disadvantaged patients had prophylactic shunts for severe hypersplenism. The types of shunts used were reversed splenorenal (n = 13), splenoadrenal (n = 6), inferior mesenteric renal (n = 1), portocaval (n = 1), inferior mesenteric caval (n = 1), and superior and inferior mesenteric caval (n = 1). In all, 22 patients had 23 shunts. The patency rate was 96% on 6 months to 10 years follow-up (average, 5.8 years). No spleen was lost. There were 2 late deaths. Two cystic fibrosis patients and one child with extrahepatic portal hypertension experienced post-shunt encephalopathy. Three patients rebled in the early postoperative period despite a patent shunt. Two patients subsequently received liver transplantation without any additional difficulties. Thus, portosystemic shunting using a method appropriate for the patient is a reliable option for treating children with portal hypertension in whom variceal sclerotherapy is inappropriate or has failed.

Adolescent↗
PubMed1990

[Complications in 52 liver transplantations excluding graft rejection].

We analyzed the postoperative complications excluding graft rejection in 52 consecutive orthotopic liver transplantations performed from March 1986 to November 1988 in 48 patients. Thirteen patients died: one intraoperatively, seven during the first 2 months, and five between 5 and 28 months. Complications were predominant during the first 3 months; infection was the most common complication. The main cause was viral agents. Cytomegalovirus was responsible for infection in 62 percent of cases, but was symptomatic in only 37 percent of patients and always had a favorable outcome. Six cases of disseminated candidiasis were observed with fatal outcome in 3 cases. Ten patients had septicemia due to Gram positive germs with a favorable course in all cases. Two patients required retransplantation on the 2nd postoperative day because of primary graft failure. Three patients had hepatic infarction which was fatal in one case. Technical complications were represented by intra-abdominal bleeding in 3 cases, perihepatic hematoma in 10 cases and stenosis of the biliary anastomosis in 8 cases; in one patient, partial portal vein thrombosis occurred; no hepatic arterial thrombosis occurred during the first postoperative days but this complication was diagnosed later in 3 instances by arteriography. Five out of 7 patients transplanted for malignant liver disease experienced recurrence which cause death in 4 cases. In 3 out of the 5 patients transplanted for postviral B cirrhosis, chronic active hepatitis occurred 6 months after transplantation and one of these patients had to be retransplanted at 13 months for recurrence of cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2005

Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt.

BACKGROUND: The long-term outcome of Budd-Chiari syndrome (BCS) with transjugular intrahepatic portosystemic shunts (TIPS) is not well studied. To address this, the records of 47 consecutive patients with BCS evaluated in one center from January 1989 to April 2004, were analyzed. RESULTS: Seven patients with liver tumors were excluded from analyses. Eleven patients had Bechet's disease, 14 had thrombophiliac disorders, four had myeloproliferative diseases and 11 patients had other or unknown causes. The site of block was hepatic vein in 16 patients, in the suprhepatic inferior vena cava in 19 and not known in five. The majority of patients (21/40; 52.5%) presented with subacute disease with massive ascites and abdominal pain as the dominant manifestations. Eight patients with membranes or segemental block were treated with transluminal angiopalsty, and six were treated with clinical and biochemical recovery. The TIPS was placed through a transcaval puncture in eight patients with progressive liver disease who were on medical therapy and had thrombosis limited to hepatic veins. One patient bled from portal vein puncture, which was managed by placing stent across the punctured site. The TIPS was very effective in decreasing portal pressure gradient, improving synthetic functions, reducing transaminase levels and controlling ascites. Five patients had TIPS dysfunction needing revision. In two patients it was difficult to maintain TIPS patency due to repeated TIPS dysfunction. However, both these patients were asymptomatic with normal liver function tests. Long-term follow up revealed that patients with TIPS had significantly better survival than those treated with medical therapy alone (log-rank test, P = 0.04). In a multivariate Cox-model analysis four variables, namely, more florid presentation, male sex, no treatment with TIPS and increasing Child-Pugh-Turcotte score, adversely affected the survival. CONCLUSIONS: Budd-Chiari syndrome needs an individualized multidisciplinary approach and TIPS is indicated in a subgroup of patients with progressive liver disease. It is safe, feasible and improves survival.

Adolescent↗