Budd-Chiari syndrome - Clinicals, Diagnosis, and Management

Gastroenterology

Clinicals - 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. 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.
Abdominal mass Due to hepatomegaly and splenomegaly. Abdominal mass
Due to hepatomegaly and splenomegaly.
Abdominal distention Is due to associated ascites. The chronic form of the disease manifests similar to complications of cirrhosis. Abdominal distention
Is due to associated ascites. The chronic form of the disease manifests similar to complications of cirrhosis.
Nausea and vomiting Frequently found in the fulminant and acute forms of Budd-Chiari syndrome. Nausea and vomiting
Frequently found in the fulminant and acute forms of Budd-Chiari syndrome.
Yellowish discoloration of sclera and mucous membranes Less commonly found symptom in the fulminant and acute forms of Budd-Chiari syndrome. Yellowish discoloration of sclera and mucous membranes
Less commonly found symptom in the fulminant and acute forms of Budd-Chiari syndrome.
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. 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.
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. 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.
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. 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.
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. 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.
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. 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.
History of other causative disorders Paroxysmal nocturnal hemoglobinuria and Anti Phospholipid Syndrome are well known causes of Budd-Chiari syndrome. History of other causative disorders
Paroxysmal nocturnal hemoglobinuria and Anti Phospholipid Syndrome are well known causes of Budd-Chiari syndrome.

Clinicals - Examination

Fact Explanation
Mild jaundice Due to deposition of bilirubin in sclera and mucous membranes. Mild jaundice
Due to deposition of bilirubin in sclera and mucous membranes.
Hepatomegaly Obstruction of hepatic venous outflow will cause hepatic venous congestion and cause enlargement of the liver. Hepatomegaly
Obstruction of hepatic venous outflow will cause hepatic venous congestion and cause enlargement of the liver.
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. 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.
Splenomegaly Portal hypertension will give rise to enlargement of the spleen. Splenomegaly
Portal hypertension will give rise to enlargement of the spleen.
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. 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.
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. 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.
Abdominal tenderness Due to hepatomegaly, ascites. Commonly found in acute and fulminant types of disease. Abdominal tenderness
Due to hepatomegaly, ascites. Commonly found in acute and fulminant types of disease.
Ankle edema Due to venous out flow obstruction. In later stages reduced albumin level in serum also aggravate ankle edema. Ankle edema
Due to venous out flow obstruction. In later stages reduced albumin level in serum also aggravate ankle edema.

Investigations - 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. 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.
Serum alkaline phosphatase Serum alkaline phosphatase level also increases to a varying extent. Serum alkaline phosphatase
Serum alkaline phosphatase level also increases to a varying extent.
Serum bilirubin Increased serum total bilirubin level can be found. Serum bilirubin
Increased serum total bilirubin level can be found.
Serum albumin Serum albumin level will be decreased with the progression of the cirrhosis. Serum albumin
Serum albumin level will be decreased with the progression of the cirrhosis.
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. 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.
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. 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.
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. 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.
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. 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.
Echocardiography Needed only in some patients to exclude tricuspid regurgitation, constrictive pericarditis, or a right atrial myxoma. Echocardiography
Needed only in some patients to exclude tricuspid regurgitation, constrictive pericarditis, or a right atrial myxoma.
Hepatic venography A “spiderweb” pattern on hepatic venography will confirm the diagnosis of Budd-Chiari syndrome. Hepatic venography
A “spiderweb” pattern on hepatic venography will confirm the diagnosis of Budd-Chiari syndrome.
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. 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.

Investigations - Management

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. 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.
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. 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.
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). 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).
Full blood count To check hemoglobin level and white cell count before liver transplantation. Full blood count
To check hemoglobin level and white cell count before liver transplantation.

Management - Specific

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. 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.
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.
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.
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. 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.
Management of the underlying causes Detectable underlying causes should be treated. Management of the underlying causes
Detectable underlying causes should be treated.
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.
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.
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. 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.
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. 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.
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. 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.
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. 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.
Symptomatic therapy for complications due to portal hypertension Patients will develop complications with disease progression and these have to be managed accordingly. Symptomatic therapy for complications due to portal hypertension
Patients will develop complications with disease progression and these have to be managed accordingly.

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