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

the Acute Liver Failure Study Group

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

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). In this study, we describe the clinical features, treatment, and outcomes of ALF due to BCS and compare our results with those in the published literature. Twenty of the 2344 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998 presented with a clinical diagnosis of BCS. An additional 19 patients of ALF-BCS in the English language literature were reviewed and compared with the ALFSG cases. Most ALF-BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of patients. BCS was diagnosed by Doppler ultrasonography or abdominal computed tomography in all patients. Liver biopsies (n = 6) all had evidence of severe pericentral 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%. In conclusion, BCS 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 orthotopic liver transplantation compared with prior published reports. Liver Transplantation 23 135–142 2017 AASLD.

Original languageEnglish (US)
Pages (from-to)135-142
Number of pages8
JournalLiver Transplantation
Volume23
Issue number2
DOIs
StatePublished - Feb 1 2017

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Budd-Chiari Syndrome
Acute Liver Failure
Liver Transplantation
Transjugular Intrahepatic Portasystemic Shunt
Doppler Ultrasonography
Liver Failure
Rare Diseases
Hospital Mortality
Jaundice
Ascites
Abdominal Pain
Registries
Necrosis
Language
Tomography
Biopsy

ASJC Scopus subject areas

  • Surgery
  • Hepatology
  • Transplantation

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Budd-chiari syndrome causing acute liver failure : A multicenter case series. / the Acute Liver Failure Study Group.

In: Liver Transplantation, Vol. 23, No. 2, 01.02.2017, p. 135-142.

Research output: Contribution to journalArticle

the Acute Liver Failure Study Group. / Budd-chiari syndrome causing acute liver failure : A multicenter case series. In: Liver Transplantation. 2017 ; Vol. 23, No. 2. pp. 135-142.
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abstract = "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). In this study, we describe the clinical features, treatment, and outcomes of ALF due to BCS and compare our results with those in the published literature. Twenty of the 2344 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998 presented with a clinical diagnosis of BCS. An additional 19 patients of ALF-BCS in the English language literature were reviewed and compared with the ALFSG cases. Most ALF-BCS patients were white (84{\%}) and female (84{\%}) in their fourth decade. A hypercoagulable state was noted in 63{\%} of patients. BCS was diagnosed by Doppler ultrasonography or abdominal computed tomography in all patients. Liver biopsies (n = 6) all had evidence of severe pericentral 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{\%}. In conclusion, BCS 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 orthotopic liver transplantation compared with prior published reports. Liver Transplantation 23 135–142 2017 AASLD.",
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