Budd‐chiari syndrome causing acute liver failure: A multicenter case series

Abstract

Background Budd-Chiari syndrome (BCS) is a rare disease resulting from obstruction of the hepatic venous outflow tract that typically presents with abdominal pain, jaundice and ascites without frank liver failure. However, BCS may also evolve more rapidly to acute liver failure (ALF-BCS). Aims To describe the clinical features, treatment and outcomes of ALF due to BCS and compare our results with those in the published literature. Methods Twenty of the 2,300 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998, presented with a clinical diagnosis of BCS. An additional 19 cases of ALF-BCS in the English-language literature were reviewed and compared to the ALFSG cases. Results Most ALF-BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of cases. BCS was diagnosed by Doppler ultrasonography or abdominal CT in all cases. Liver biopsies (6) all had evidence of severe peri-central necrosis. Treatments used included most commonly anticoagulation (71%), but also transjugular intrahepatic portosystemic shunt (TIPS) (37%) and orthotopic liver transplantation (37%). In-hospital mortality was approximately 60%. Conclusions Budd-Chiari syndrome is a rare cause of ALF and mandates prompt diagnosis and management for successful outcomes. Once the diagnosis is confirmed, prompt anticoagulation is recommended in conjunction with evaluation for malignancy or thrombophilic disorder. Mortality may have improved in recent years with use of TIPS and/or OLT compared to prior published reports.

Review article: a multidisciplinary approach to the diagnosis and management of Budd‐Chiari syndrome

“…LT is the final therapeutic option in severe BCS unresponsive to hepatic venous interventions or TIPSS. LT can also be considered for first line therapy in patients that present with fulminant/acute liver failure . In patients with small vessel BCS, there is no role of percutaneous recanalisation/stenting as portal hypertension is all intrahepatic.…”

Budd‐chiari syndrome and acute liver failure: A complex condition requiring a rapid response

“…The combined experience was 38 cases, 19 from each source, and the rarity of the condition was confirmed by the fact that BCS accounted for <1% of cases of acute liver failure. (5) The outcome in these patients was relatively poor with a 42% survival rate, although the outcome was better in more recent cases.…”

“…This topic is addressed in an article in this edition of Liver Transplantation from the US Acute Liver Failure Group combining its own data together with cases identified through a review of the literature. The combined experience was 38 cases, 19 from each source, and the rarity of the condition was confirmed by the fact that BCS accounted for <1% of cases of acute liver failure . The outcome in these patients was relatively poor with a 42% survival rate, although the outcome was better in more recent cases.…”

Acute Liver Failure

“…6 BCS-related ALF dictates prompt diagnosis and management with anticoagulation in conjunction with cancer or thrombophilic disorder evaluation; mortality might improve by transjugular intrahepatic portosystemic shunt and/or OLT use. 50 As far as viral hepatitis is concerned, hepatitis A and B viruses rank as the most common viral causes whereas the remaining pathogens such as hepatitis E, herpes simplex, or even Epstein-Barr viruses are more likely to be found in special categories of patients such as those who are immunocompromised or during gestation. In HBV-ACLF patients, some data indicate that human mesenchymal stem cell transplantation might significantly decrease the mortality rate, without raising the incidence of severe complications.…”

“…[80][81][82] Once BCS diagnosis is established, the timely initiation of anticoagulation in therapeutic doses and-when available -angioplasty can improve the patient's prognosis. 50 Finally, in mushroom-induced ALF, it is crucial to apply all dispensable measures, including prompt administration of silibinin (the major active constituent of silymarin), gastric lavage and activated charcoal, and enough diuresis on top of proper hydration. Nevertheless, it has to be mentioned that gastric lavage as fundamental supportive therapy for mushroom poisoning has been questioned by some authors.…”