Budd-Chiari Syndrome

Urgently assess for acute liver failure, acute-on-chronic liver failure, rapidly worsening synthetic dysfunction, encephalopathy, tense or recurrent ascites, renal dysfunction, and gastrointestinal bleeding. Budd-Chiari syndrome can present as fulminant liver failure; hepatic venous outflow obstruction causes congestion, hepatic dysfunction, and occasionally liver failure. Wolters Kluwer+1Wolters KluwerBudd-Chiari Syndrome: Presentation, Management, and... : Official journal of the American College of Gastroenterology | ACGASHChapter 16: Atypical-site thrombosis - ASH Publications

Admit patients with hepatic failure, progressive encephalopathy, worsening ascites, or declining liver function to a center with hepatology, interventional radiology, and transplant capability. Failure of anticoagulation-based management marked by worsening liver function, ascites, or encephalopathy should trigger consideration of endovascular therapy, TIPS, and/or transplant evaluation rather than continued medical observation. aasldaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Treat portal-hypertension complications concurrently while defining venous anatomy. Ascites and encephalopathy are clinically consequential manifestations of portal hypertension and advanced liver disease; recurrent or refractory complications favor expedited decompressive intervention rather than delayed etiologic testing alone. ScienceDirect+1ScienceDirectManagement of portal hypertension, Budd– Chiari syndrome and portal vein thrombosis - ScienceDirectaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Diagnose Budd-Chiari syndrome when imaging demonstrates obstruction of hepatic venous outflow from the hepatic venules through the hepatic-vein/IVC junction, without an intracardiac or pericardial cause. Obstruction may involve small hepatic veins, major hepatic veins, or the hepatic portion of the IVC. Wolters Kluwer+2Wolters KluwerBudd-Chiari Syndrome: Presentation, Management, and... : Official journal of the American College of Gastroenterology | ACGScienceDirectManagement of portal hypertension, Budd– Chiari syndrome and portal vein thrombosis - ScienceDirectPubMedProposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion - PubMed

Use Doppler ultrasound as the first vascular test. Findings supporting Budd-Chiari syndrome include poor visualization or nonvisualization of hepatic veins, abnormal hepatic-vein flow, collapsed suprahepatic veins, and loss of visualization at the hepatic vein-IVC junction. If ultrasound is equivocal or intervention is contemplated, obtain contrast-enhanced CT or MR venography to define thrombosis or stenosis, IVC involvement, collateral vessels, caudate hypertrophy, and liver enhancement pattern. PubMedPubMedBudd-Chiari syndrome in a 33-year-old woman with hypercoagulable state: A case report

Use CT or MR venography to distinguish a focal hepatic-vein or IVC lesion potentially amenable to recanalization from diffuse hepatic-vein occlusion more likely to require TIPS. CT and MRI also identify portal-, splenic-, or superior mesenteric-vein thrombosis, which can coexist with Budd-Chiari syndrome and materially affects procedural planning. ScienceDirect+3ScienceDirectPortal Vein Thrombosis and Budd–Chiari Syndrome - ScienceDirectWolters KluwerRecanalization and Reconstruction of a... : Vascular and Endovascular SurgeryWolters KluwerBudd-chiari syndrome management: Timing of... : HepatologyPubMedBudd-Chiari syndrome in a 33-year-old woman with hypercoagulable state: A case report

Do not rely on liver biopsy to establish the vascular diagnosis when imaging is diagnostic. Biopsy may support assessment of liver injury or alternative pathology, but histology is not pathognomonic and varies across patients. Reserve it for unresolved diagnostic questions or when tissue will change management. ScienceDirect+1ScienceDirectBudd–Chiari syndrome: a review by an expert panel - ScienceDirectPubMedBudd-Chiari syndrome in a 33-year-old woman with hypercoagulable state: A case report

Classify disease as primary when hepatic venous outflow obstruction is thrombotic and as secondary when obstruction results from malignant invasion, post-transplant or post-resection hepatic-vein injury, extrinsic compression from hydatid cysts or polycystic liver disease, or rarely sarcoidosis. This distinction directs treatment toward systemic thrombosis prevention versus correction or treatment of the local cause. Wolters Kluwer+1Wolters KluwerBudd-Chiari Syndrome: Presentation, Management, and... : Official journal of the American College of Gastroenterology | ACGScienceDirectManagement of portal hypertension, Budd– Chiari syndrome and portal vein thrombosis - ScienceDirect

Evaluate every patient for an underlying hypercoagulable condition. Myeloproliferative disorders are prominent acquired causes, and thrombocytosis, erythrocytosis, or leukocytosis should heighten suspicion; the AASLD clinical example specifically links this phenotype to hypercoagulable evaluation. BMJ+1BMJa rare and life-threatening complication of Crohn's diseaseaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Obtain a thrombophilia-focused evaluation that includes assessment for myeloproliferative neoplasm, antiphospholipid antibodies, factor V Leiden, prothrombin G20210A, antithrombin deficiency, protein C deficiency, and protein S deficiency. Evaluate acquired exposures and conditions including pregnancy, oral contraceptive use, inflammatory bowel disease, tumor, and bacterial or fungal infection. Multiple prothrombotic factors may coexist, particularly when other splanchnic veins are thrombosed. BMJ+1BMJa rare and life-threatening complication of Crohn's diseaseScienceDirectPortal Vein Thrombosis and Budd–Chiari Syndrome - ScienceDirect

For suspected myeloproliferative neoplasm, test for JAK2 V617F and coordinate hematology assessment even when hepatic congestion or portal hypertension complicates interpretation of blood counts. JAK2 V617F has a recognized association with Budd-Chiari syndrome, and chronic myeloproliferative disorders are established acquired thrombotic risk factors. BMJ+2BMJa rare and life-threatening complication of Crohn's diseaseScienceDirectCurrent knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis - ScienceDirectaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Initiate therapeutic anticoagulation in Budd-Chiari syndrome while evaluating anatomy and underlying thrombophilia, unless a patient-specific contraindication requires modification. Therapeutic anticoagulation is described as first-line treatment for Budd-Chiari syndrome, and anticoagulation should begin while other therapeutic options are explored. ScienceDirect+1ScienceDirectManagement of portal hypertension, Budd– Chiari syndrome and portal vein thrombosis - ScienceDirectaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Do not use clinical stability after anticoagulation as the only endpoint. Reassess liver function, ascites, and encephalopathy; clinical deterioration despite anticoagulation indicates failure of medical management and should prompt endovascular and transplant discussion. aasldaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

When a thrombosed or stenotic hepatic vein has a treatable focal lesion, pursue restoration of patency with endovascular recanalization, angioplasty, and, when appropriate, stenting. In contrast, if hepatic-vein recanalization fails, TIPS can provide an alternative route for hepatic venous outflow. Wolters Kluwer+1Wolters KluwerRecanalization and Reconstruction of a... : Vascular and Endovascular SurgeryaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Use TIPS for patients who require decompression because of persistent portal-hypertension complications, worsening liver function, or anatomy unsuitable for recanalization. TIPS is commonly used in Budd-Chiari syndrome and remains effective even when thrombosis extends into the portal venous tree, although complex thrombosis requires experienced procedural planning. Wolters Kluwer+1Wolters KluwerBudd-chiari syndrome management: Timing of... : HepatologyaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLD

Refer for liver transplantation when fulminant liver failure, acute-on-chronic liver failure, failure of medical therapy and portosystemic shunting, or hepatocellular carcinoma complicates chronic Budd-Chiari syndrome. Long-term anticoagulation is required in most transplanted patients because thrombosis can recur. Wolters KluwerWolters KluwerBudd-Chiari Syndrome: Presentation, Management, and... : Official journal of the American College of Gastroenterology | ACG

After anticoagulation, recanalization, or TIPS, follow clinical congestion and hepatic function with serial assessment of ascites, encephalopathy, bilirubin, INR, creatinine, and blood counts. Recurrent ascites or worsening encephalopathy should prompt reassessment for persistent obstruction, TIPS dysfunction, progressive liver disease, or recurrent thrombosis. Wolters Kluwer+2Wolters KluwerBudd-Chiari Syndrome: Presentation, Management, and... : Official journal of the American College of Gastroenterology | ACGaasldAn 'Ascite-ing' Case of Abdominal Distention | AASLDpubs rsnaLong-term Outcome and Analysis of Dysfunction ...

Maintain surveillance for thrombosis recurrence in patients with underlying hypercoagulability and after TIPS. Hypercoagulable patients with primary Budd-Chiari syndrome are predisposed to new thrombosis in the TIPS shunt or IVC, making ongoing anticoagulation strategy and vascular follow-up central to management. Wolters KluwerWolters KluwerRecanalization and Reconstruction of a... : Vascular and Endovascular Surgery

In chronic Budd-Chiari syndrome, evaluate new liver lesions with multiphasic contrast imaging. Portal-vein tumor invasion is prognostically important in HCC; arterial-phase enhancement within thrombus, portal-vein expansion, and high diffusion-weighted MR signal favor tumor thrombus over bland thrombosis. BMJ+1BMJBritish Society of Gastroenterology guidelines for the ...Wolters KluwerBudd-Chiari Syndrome: Presentation, Management, and... : Official journal of the American College of Gastroenterology | ACG