Clinicopathological forms and prognostic index in Budd-Chiari syndrome

Langlet, Philippe; Escolano, Sylvie; Valla, Dominique; Coste-Zeitoun, Delphine; Denié, C.; Mallet, Alain; Lévy, V. G.; Franco, Dominique; Vinel, Jean–Pierre; Belghiti, Jacques; Lebrec, Didier; Hay, Jean‐Marie; Zeitoun, Guy

doi:10.1016/s0168-8278(03)00323-4

Cited by 162 publications

(117 citation statements)

References 29 publications

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“…In the second study, these results were evaluated in an independent sample of 69 patients. 25 The original index was extended with an additional factor, representing acute, chronic, or acute-on-chronic BCS. However, a recent review of an expert panel on BCS has stated that at the present time, no consensus has been reached on the classification into acute and chronic disease, because scientific arguments for this classification are still lacking.…”

Section: Discussionmentioning

confidence: 99%

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

et al. 2004

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Budd-Chiari syndrome (BCS) is a rare disorder that is characterized by hepatic venous outflow obstruction. The aim of this study was to assess determinants of survival and to evaluate the effect of portosystemic shunting. In this international multicenter study, 237 patients with BCS, diagnosed between 1984 and 2001, were investigated. Univariate, multivariate, and time-dependent Cox regression analyses were performed. Overall survival at 1, 5, and 10 years was 82% (95% CI, 77%-87%), 69% (95% CI, 62%-76%), and 62% (95% CI, 54%-70%), respectively. Encephalopathy, ascites, prothrombin time, and bilirubin were independent determinants of survival. A prognostic classification combining these factors could identify three classes of patients (classes I-III). The 5-year survival rate was 89% (95% CI, 79%-99%) for class I, 74% (95% CI, 65%-83%) for class II, and 42% (95% CI, 28%-56%) for class III. Anticoagulants were administered to 72%; only for patients in class I was this associated with a trend toward improved survival (relative risk [

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Section: Discussionmentioning

confidence: 99%

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

et al. 2004

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“…Poor results of surgery in patients with liver dysfunction; anatomical difficulties causing success rates as low as 30%; higher post-operative complications and mortality rates (more than 20%); high re-stenosis rates (around 30%) requiring surgical revisions in a minority (around 10%); and poor survival rates as low as 57% at five years have been described [1,3,4,70,73,74] . Surgical treatment has also failed to show a favorable effect in two multivariate analyses [68,75] , but was successful in one other study [76] . In most Indian reports, while surgery for IVC involvement has shown fair results [34,36] ; surgical treatments for HV occlusion have shown poor results [12,38] .…”

Section: Discussionmentioning

confidence: 99%

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Amarapurkar¹,

Punamiya²,

Patel³

2008

WJG

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AIM:To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India. METHODS:Forty-nine consecutive cases of BuddChiari syndrome (BCS) were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava (IVC) and/or hepatic vein (HV) obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation. RESULTS:Mean duration of symptoms was 41.5 ± 11.2 (range = 1-240) mo. HV thrombosis (HVT) was present in 29 (59.1%), IVC thrombosis in eight (16.3%), membranous obstruction of IVC in two (4%) and both IVC-HV thrombosis in 10 (20.4%) cases. Of 35 cases tested for hypercoagulability, 27 (77.1%) were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 (97.3%): IVC stenting in seven (18.9%), IVC balloon angioplasty in two (5.4%), combined IVC-HV stenting in two (5.4%), HV stenting in 11 (29.7%), transjugular intrahepatic portosystemic shunt (TIPS) in 13 (35.1%) and combined TIPS-IVC stenting in two (5.4%). Complications encountered in follow-up: death in five, re-stenosis of the stent in five (17.1%), hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT. CONCLUSION:In our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability, etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases.

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“…Clinical: type I -acute, type II -chronic, type III -acute or chronic lesion with the worst prognosis (Langlet et al, 2003).…”

Section: Classificationmentioning

confidence: 99%

Budd-Chiari Syndrome

et al. 2017

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Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment. Without treatment, 90% of patients die within 3 years, mostly due to complications of liver cirrhosis. BCS can be classified according to etiology (primary, secondary), clinical course (acute, chronic, acute or chronic lesion), and morphology (truncal, radicular, and venooclusive type). The diagnosis is established by demonstrating obstruction of the venous outflow and structural changes of the liver, portal venous system, or a secondary pathology by ultrasound, computed tomography, or magnetic resonance. Laboratory and hematological tests are an integral part

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Clinicopathological forms and prognostic index in Budd-Chiari syndrome

Cited by 162 publications

References 29 publications

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Budd-Chiari Syndrome

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