History

Fact Explanation
Abdominal pain Budd Chiari Syndrome is caused by the occlusion of the hepatic vein. Right hypochondrial abdominal pain, is a typical symptom in patients with Budd-Chiari syndrome. This is mainly due to enlargement of liver causing stretching of the liver capsule.[1,2]
Abdominal mass Due to hepatomegaly and splenomegaly. [1,2]
Abdominal distention Is due to associated ascites. The chronic form of the disease manifests similar to complications of cirrhosis.[1,2]
Nausea and vomiting Frequently found in the fulminant and acute forms of Budd-Chiari syndrome.[1]
Yellowish discoloration of sclera and mucous membranes Less commonly found symptom in the fulminant and acute forms of Budd-Chiari syndrome.[1]
Ankle swelling Due to obstruction of the venous out flow with occlusion of the inferior venecava and also due to reduced albumin levels in chronic stages of the disease, patients will develop ankle oedema.[1]
Hemetamesis, melaena The chronic form of Budd-Chiari syndrome is manifested with complications of cirrhosis such as formation of esophageal varices leading to hemetamesis and melena.[1]
Symptoms suggestive of hepatic encephalopathy Patients with the fulminant and chronic form of the syndrome with decompensated cirrhosis may present with symptoms of hepatic encephalopathy.[1]
History of thrombophilic condition Thrombophilic conditions with hypercoagulable states, facilitate venous thrombosis and patients with these conditions both hereditary and acquired have a increased risk of developing Budd-Chiari syndrome.[1,2]
History of predisposing other hematological disorders Myeloproliferative disorders are the most common causes of the Budd–Chiari syndrome. Polycythemia vera is the cause in some patients with Budd-Chiari syndrome. Essential thrombocythemia and myelofibrosis are less commonly found causes.[1,2]
History of other causative disorders Paroxysmal nocturnal hemoglobinuria and Anti Phospholipid Syndrome are well known causes of Budd-Chiari syndrome.[1,2]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282
  2. HOEKSTRA J, JANSSEN HL. Vascular liver disorders (I): diagnosis, treatment and prognosis of Budd-Chiari syndrome. Neth J Med [online] 2008 Sep, 66(8):334-9 [viewed 30 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/18809980

Examination

Fact Explanation
Mild jaundice Due to deposition of bilirubin in sclera and mucous membranes.[1]
Hepatomegaly Obstruction of hepatic venous outflow will cause hepatic venous congestion and cause enlargement of the liver.[1]
Ascites Obstruction of the hepatic venous outflow tract results in increased hepatic sinusoidal pressure and portal hypertension which in turn produces accumulation of fluid in the peritoneal cavity, which is called ascites. [1]
Splenomegaly Portal hypertension will give rise to enlargement of the spleen. [1]
Pallor Pallor can be detected because of anemia in these patients. Anemia can be due to several causes such as anemia of chronic disease, iron deficiency anemia due to bleeding from varices, due underlying myeloproliferative disorders etc.[1]
Peripheral stigmata of chronic liver disease - spider nevi, hair loss, gnaecomastia, leukonychia etc. Patients with chronic stage of Budd-Chiari syndrome will manifest signs of chronic liver disease.[1]
Abdominal tenderness Due to hepatomegaly, ascites. Commonly found in acute and fulminant types of disease.[1]
Ankle edema Due to venous out flow obstruction. In later stages reduced albumin level in serum also aggravate ankle edema.[1]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282

Differential Diagnoses

Fact Explanation
Tricuspid regurgitation The presence of hepatojugular reflux favors cardiac cause of ascites such as tricuspid regurgitation. Need echocardiogram for the diagnosis.[1]
Constrictive pericarditis Chronic inflammation of the pericardium will result in impaired filling of the ventricles and reduced ventricular function. Echocardiogram will show increased pericardial thickness which aid the diagnosis. Suspect in patients with predisposing factors such as history of tuberculosis, mediastinal irradiation, and previous cardiac surgical procedures.[1,5]
Right atrial myxoma Patients complain fatigue, ankle oedema, abdominal distention due to ascites when the atrial myxoma causing right sided heart failure. Finger clubbing, elevated jugular venous pressure may be found and echocardiogram will need for the diagnosis.[1,3]
Right-sided heart failure/Congestive heart failure Consider if the patient is complaining exertional dyspnea, orthopnea, paroxysmal dyspnea, ankle swelling due to edema or if patient is having risk factors for the development of heart failure, such ischemic heart disease, hypertension, valvular heart disease. Echocardiogram will aid in diagnosis. [2]
Alcoholic liver disease Consider in patients having history of heavy alcohol consumption. Ascitic fluid analysis with determination of serum:ascitic fluid albumin gradient will help differentiation from Budd-Chiari syndrome.[4]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282
  2. KING M, KINGERY J, CASEY B. Diagnosis and evaluation of heart failure. Am Fam Physician [online] 2012 Jun 15, 85(12):1161-8 [viewed 02 June 2014] Available from: http://www.aafp.org/afp/2012/0615/p1161.html
  3. LONE RA, AHANGER AG, SINGH S, MEHMOOD W, SHAH S, LONE G, DAR A, BHAT M, SHARMA M, LATEEF W. Atrial Myxoma: Trends in Management Int J Health Sci (Qassim) [online] 2008 Jul, 2(2):141-151 [viewed 02 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3068734
  4. O'SHEA RS, DASARATHY S, MCCULLOUGH AJ, PRACTICE GUIDELINE COMMITTEE OF THE AMERICAN ASSOCIATION FOR THE STUDY OF LIVER DISEASES, PRACTICE PARAMETERS COMMITTEE OF THE AMERICAN COLLEGE OF GASTROENTEROLOGY. Alcoholic liver disease. Hepatology [online] 2010 Jan, 51(1):307-28 [viewed 02 June 2014] Available from: doi:10.1002/hep.23258
  5. Guidelines on the Diagnosis and Management of Pericardial Diseases Executive SummaryThe Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. European Heart Journal [online] 2004 April, 25(7):587-610 [viewed 02 June 2014] Available from: doi:10.1016/j.ehj.2004.02.002

Investigations - for Diagnosis

Fact Explanation
Liver enzymes - Serum aspartate and alanine aminotransferase (AST and ALT) Enzyme levels may be more than five times the upper limit of the normal range in the fulminant and acute forms of the Budd–Chiari syndrome, whereas increases are smaller in the subacute form.[1]
Serum alkaline phosphatase Serum alkaline phosphatase level also increases to a varying extent.[1]
Serum bilirubin Increased serum total bilirubin level can be found.[1]
Serum albumin Serum albumin level will be decreased with the progression of the cirrhosis.[1]
Ascitic fluid analysis The serum–ascitic fluid albumin gradient(SAAG) is high, with the total protein level in the ascitic fluid usually more than 2.5 g/dL.[1]
Doppler ultrasonography of the liver Investigation of choice for the initial assessment of patients suspecting Budd-Chiari syndrome. Necrotic areas of the liver can be seen.[1]
Contrast-enhanced computed tomographic (CT) scan CT scanning, is recommended to demonstrate the venous anatomy and the architecture of the liver when a transjugular intrahepatic portosystemic shunt is being considered.[1]
Magnetic resonance imaging (MRI) Though it is more expensive than CT scanning and less readily available, MRI will visualize hepatic-vein thrombosis, whole length of the inferior vena cava and may help differentiation of the acute form of the Budd–Chiari syndrome from the subacute and chronic forms.[1]
Echocardiography Needed only in some patients to exclude tricuspid regurgitation, constrictive pericarditis, or a right atrial myxoma.[1]
Hepatic venography A “spiderweb” pattern on hepatic venography will confirm the diagnosis of Budd-Chiari syndrome.[1]
Liver biopsy A transjugular liver biopsy of both the right and left lobes should be carried out at the time of the angiographic investigation to confirm the diagnosis and to help guide therapy, though the appropriateness of biopsy has not yet been established.[1]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282

Investigations - Fitness for Management

Fact Explanation
Full blood count To check hemoglobin level and white cell count before liver transplantation. [1]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282

Investigations - Followup

Fact Explanation
Doppler ultrasonography of liver The risk of re-stenosis with angioplasty procedures is high and need regular follow-up with the use of Doppler ultrasonography to determine venous patency. [1]
Na and creatinine Should be measured to assess the response to treatment. It is defined as ongoing response when ascites is detectable but responsive to low-dose diuretics with normal Na and creatinine values.[2]
Factor V and bilirubin level When there is an ongoing response with treatment to ascites, Factor V is increasing (if initially low) and bilirubin decreasing (if initially high).[2]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282
  2. MANCUSO A. Budd-Chiari syndrome management: Lights and shadows World J Hepatol [online] 2011 Oct 27, 3(10):262-264 [viewed 30 May 2014] Available from: doi:10.4254/wjh.v3.i10.262

Management - Specific Treatments

Fact Explanation
Multidisciplinary approach Management of the patient will need experts from several disciplines of medicine such as participation of a hepatologist, a hematologist, an interventional radiologist and a surgeon to provide best care to the patient.[1]
Management of ascites 1) Dietary measures: Restrict sodium intake to 90 mmol/day. 2) Drug therapy: Spironolactone and furosemide to achieve a negative sodium balance. 3) Large-volume paracentesis and intravenous infusions of albumin are necessary when ascites is tense or refractory to diuretic therapy.[1]
Anticoagulation therapy To prevent further extension of the venous thrombosis. In the initial stages of the disease heparin is the recommended drug and warfarin is indicated for long term use. When giving warfarin, target international normalized ratio for prothrombin time is 2.0 - 2.5.[1,2]
Management of the underlying causes Detectable underlying causes should be treated.[1]
Thrombolytic Therapy Considered in patients with the acute form of the Budd–Chiari syndrome. Urokinase (240,000 U/hour for 2 hours, followed by 60,000 U/hour) or tissue plasminogen activator (0.5 to 1.0 mg/hour) is infused directly into the thrombosed hepatic vein for about 24 hours by a transfemoral or transjugular route. [1,2,3]
Angioplasty with or without stenting Percutaneous or transhepatic angioplasty of localized segments of the narrowed hepatic vein or inferior vena caval webs can be used to relieve symptoms.[1,2,3,4]
Transjugular Intrahepatic Portosystemic Shunts (TIPS) Considered when thrombolytic therapy and angioplasty are failed. Useful in patients with an occluded inferior vena cava, those in whom the portal vein–infrahepatic vena caval pressure gradient is <10 mm Hg, and those with poor hepatic reserve.[1,2,3,4]
Surgical shunts Portosystemic surgical shunts, may reverse hepatic necrosis and prevent cirrhosis by relieving sinusoidal hypertension. A surgical portosystemic shunt is considered in patients with the subacute form of Budd-Chiari syndrome when the underlying cause is associated with a favorable long-term outcome.[1,4]
Liver Transplantation The indications for transplantation include fulminant hepatic failure, cirrhosis, and the failure of a portosystemic shunt, provided that the underlying disease is associated with a favorable long-term prognosis.[1,2,4]
Symptomatic therapy for complications due to portal hypertension Patients will develop complications with disease progression and these have to be managed accordingly.[2]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 30 May 2014] Available from: doi:10.1056/NEJMra020282
  2. MANCUSO A. Budd-Chiari syndrome management: Lights and shadows World J Hepatol [online] 2011 Oct 27, 3(10):262-264 [viewed 30 May 2014] Available from: doi:10.4254/wjh.v3.i10.262
  3. HOEKSTRA J, JANSSEN HL. Vascular liver disorders (I): diagnosis, treatment and prognosis of Budd-Chiari syndrome. Neth J Med [online] 2008 Sep, 66(8):334-9 [viewed 30 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/18809980
  4. HEFAIEDH R, CHEIKH M, MARSAOUI L, ENNAIFER R, ROMDHANE H, BEN NEJMA H, BEL HADJ N, ARFA N, KHALFALLAH MT. The Budd-Chiari syndrome. Tunis Med [online] 2013 Jun, 91(6):376-81 [viewed 30 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23868034