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Budd-Chiari Syndrome

Updated : March 18, 2024





Background

In the absence of pericardial illness, heart conditions, or veno-occlusive disease (sinusoidal blockage syndrome), Budd-Chiari syndrome (BCS) is a rare ailment characterized by hepatic venous output tube obstruction, regardless of the degree and mechanism of blockage.

Obstruction brought on by a mostly venous condition is known as primary Budd-Chiari syndrome (thrombosis and phlebitis). The venae cavae and liver veins, on the other hand, may be compressed or invaded by a lesion that originates outside of the vein in secondary BCS (for example, a malignancy).

Epidemiology

Budd-Chiari syndrome is typically identified in the 3rd or 4th years of life in non-Asian nations, mostly in females. Hepatic vein obstruction is the most typical cause.

In Asian nations, males are more likely to experience it, and inferior vena cava occlusion or even a combination of inferior vena cava & liver vein obstruction is the most frequent cause.

Anatomy

Pathophysiology

A single liver vein obstruction typically goes unnoticed, but when two liver veins are blocked, the hepatic capsule is stretched due to venous compression. This may cause severe agony. The sinusoids start to widen, and interstitial fluid starts to filter out, causing hepatic congestion. Ascites develops when the amount of filtrated fluid starts to pass through the hepatic capsule beyond the lymph system’s ability to drain it.

There is portal vein hypertension and a reduction in blood circulation to the liver through the portal vein as a result of the thrombosis and blockage to venous drainage. As a result, the centrilobular hepatocellular suffers hypoxic damage. This can result in liver failure in severe acute instances, while ascites and hepatomegaly with maintained liver function can result in cases of severe.

Cirrhosis results from the development of fibrosis over time. Since its blood is directly directed into the inferior vena cava, the caudate lobe is the one that is most frequently afflicted by BCS. As a result, the caudate lobe enlarges when the liver veins are blocked.

Etiology

Budd-Chiari syndrome is the result of an underlying condition in 80% of instances, with a hypercoagulable condition being the most common culprit. When diagnosing and treating Budd-Chiari syndrome, this element must be considered.

The following are the primary contributing factors to BCS:

Budd-Chiari syndrome is the result of an underlying condition in 80% of instances, with a hypercoagulable condition being the most common culprit. When diagnosing and treating Budd-Chiari syndrome, this element must be considered.

The following are the primary contributing factors to BCS: 

Malignancy

Malignancy accounts for 10 percent of cases of BCS and can either directly compress arteries or invade them. All of these contribute to hypercoagulability and venous thrombosis, which can restrict blood flow.

Hepatocellular carcinoma, leiomyosarcoma, renal cell carcinoma, Wilms tumor, and right atrial myxoma are the most frequent cancers connected to the Budd-Chiari syndrome.

Myeloproliferative diseases 

Due to the nearly universal presence of hypercoagulability in myeloproliferative illnesses, including polycythemia vera & critical thrombocythemia, about half of all cases of Budd-Chiari syndrome are associated with these conditions.

Idiopathic

20 percent of instances are idiopathic.

The following hypercoagulable conditions also contribute to Budd-Chiari syndrome:

A mutation in factor V (Leiden) causes resistance to protein C antibodies to phospholipids syndrome, a lack of antithrombin protein C shortage of hemoglobinuria at night that is recurrent

During pregnancy, OCPs (oral contraceptives) 

A hypercoagulable state brought on by contraceptive pills & pregnancy accounts for roughly 20% of instances of Budd-Chiari syndrome.

Lesions of the liver 

The constraint of the vasculature can occasionally be caused by an infection or a lesion of the hepatic that takes up space.

Genetics

Prognostic Factors

A favorable prognosis for Budd-Chiari syndrome is influenced by:

  • Low serum creatinine level
  • Young age
  • Ascites is absent
  • Poor Child-Pugh rating

Consequences of a poor prognosis:

Involvement of the portal vein and/or the 3 hepatic capillaries

  • Age at the onset of the presentation was higher
  • Superior Child-Pugh rating
  • Chronic illness when first diagnosed

Clinical History

Age Group: 

  • While Budd-Chiari Syndrome can occur at any age, it is most diagnosed in adults, typically between the ages of 20 and 50. 

Physical Examination

Abdominal Examination: 

  • Hepatomegaly: The liver is often enlarged due to impaired venous outflow. A firm and tender liver may be palpable below the costal margin. 
  • Ascites: Distension can occur when fluid builds up in the abdominal cavity. Shifting dullness and a fluid wave may be elicited during examination. 
  • Splenomegaly: Enlargement of the spleen can occur as a consequence of portal hypertension. 

Jaundice: 

  • Scleral Icterus: Yellowing of the sclera (white part of the eyes) is a common sign of jaundice. 

Abdominal Pain: 

  • Right Upper Quadrant (RUQ) Pain: Individuals experiencing hepatomegaly and hepatic capsule stretching may report discomfort or pain in their right upper quadrant. 

Circulatory Examination: 

  • Peripheral Edema: Fluid retention and peripheral edema can occur in advanced cases due to decreased liver synthetic function and hypoalbuminemia. 

Skin Findings: 

  • Spider Angiomas: These are small, spider-like blood vessels that may be visible on the skin, often seen in liver disease. 
  • Palmar Erythema: Reddening of the palms can be present in liver disorders. 

Neurological Examination: 

  • Hepatic Encephalopathy Signs: In advanced cases, neurological symptoms such as confusion, asterixis (flapping tremor), and altered mental status may be present. 

Vascular Examination: 

  • Collateral Veins: Visible collateral veins on the abdominal wall, often referred to as “caput medusae,” may be a sign of portal hypertension. 

Age group

Associated comorbidity

  • Myeloproliferative Disorders: Budd-Chiari Syndrome is often associated with underlying conditions such as myeloproliferative disorders (e.g., polycythemia vera, essential thrombocythemia, and myelofibrosis). These conditions may lead to the development of blood clots within the hepatic veins. 
  • Hypercoagulable States: Conditions that increase the risk of blood clotting, such as inherited or acquired hypercoagulable states, may contribute to the development of Budd-Chiari Syndrome. 
  • Oral Contraceptive Use: Oral contraceptive usage has occasionally been linked, especially in women, to a higher incidence of Budd-Chiari syndrome. 

Associated activity

Acuity of presentation

  • Acute Onset: Budd-Chiari Syndrome can present acutely with sudden and severe symptoms. This may include abdominal pain, hepatomegaly (enlarged liver), ascites, and jaundice (yellowing of the skin and eyes). 
  • Chronic Presentation: In some cases, the onset of Budd-Chiari Syndrome may be more gradual, with individuals experiencing milder and nonspecific symptoms over a more extended period. This chronic form may lead to liver fibrosis and cirrhosis if left untreated. 

Differential Diagnoses

  • Hepatic Cirrhosis: Cirrhosis can lead to portal hypertension and liver dysfunction, which may cause symptoms such as ascites, hepatomegaly, and jaundice similar to BCS. 
  • Congestive Heart Failure (CHF): Right-sided heart failure can lead to congestion of the hepatic veins, resulting in symptoms like hepatomegaly and ascites. 
  • Acute Hepatitis: Acute inflammation of the liver can cause symptoms such as abdominal pain, hepatomegaly, and jaundice, mimicking acute BCS. 
  • Liver Abscess: An abscess in the liver can lead to symptoms such as fever,abdominal pain, and hepatomegaly, which may be mistaken for BCS. 
  • Portal Vein Thrombosis: Similar to BCS, portal vein thrombosis can cause portal hypertension, leading to symptoms like ascites and varices. 
  • Hepatic Vein Thrombosis (Non-Budd-Chiari): Thrombosis of the hepatic veins unrelated to the classical BCS presentation can occur, leading to similar symptoms. 
  • Malignant Hepatic Tumors: Primary or metastatic liver tumors can cause hepatomegaly, abdominal pain, and other symptoms that may overlap with BCS. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Medical Management: 

  • Anticoagulation: Anticoagulant medications, such as heparin and warfarin, are often a cornerstone of treatment. Anticoagulation helps prevent further blood clot formation and promotes the resolution of existing clots. 
  • Thrombolytic Therapy: In some cases, thrombolytic agents may be used to dissolve blood clots, especially in acute presentations with severe venous obstruction. 

Interventional Procedures: 

  • Angioplasty and Stenting: Percutaneous transluminal angioplasty (PTA) with or without stent placement is a common interventional approach. This helps to relieve venous obstruction and improve blood flow. 
  • Transjugular Intrahepatic Portosystemic Shunt (TIPS): TIPS is a procedure that involves creating a shunt between the hepatic vein and the portal vein to bypass the obstruction, reducing portal hypertension. 
  • Surgical Shunting: In some cases, surgical shunting procedures, such as portocaval or mesocaval shunts, may be considered. However, these are less commonly performed due to the availability of less invasive interventions. 

Liver Transplantation: 

  • For individuals with advanced or refractory Budd-Chiari Syndrome, particularly those with irreversible liver damage or complications such as cirrhosis, liver transplantation may be considered. 

Management of Underlying Conditions: 

  • If BCS is associated with an underlying disorder such as myeloproliferative disorders, the management of the primary condition is crucial. This may involve using medications to control blood cell counts or other disease-specific treatments. 

Symptomatic Treatment: 

  • Ascites, hepatic encephalopathy, and other complications may require specific symptomatic management, such as diuretics for fluid retention or medications to address encephalopathy. 

Follow-Up and Surveillance: 

  • Long-term follow-up is essential to monitor for symptom recurrence, assess interventions’ effectiveness, and manage potential complications. Regular imaging studies and laboratory tests are typically performed during follow-up. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Use of a non-pharmacological approach for treating Budd-Chiari Syndrome

Lifestyle Modifications: 

  • Dietary Changes: A low-sodium diet may be recommended to manage ascites and fluid retention associated with BCS. 
  • Alcohol Abstinence: If alcohol consumption is a contributing factor or exacerbating liver disease, abstinence is crucial to prevent further liver damage. 

Compression Stockings: 

  • Compression stockings may be recommended to manage peripheral edema and prevent complications related to venous insufficiency. 

Bed Rest: 

  • In acute presentations of BCS, bed rest may be advised to reduce the workload on the liver and decrease the risk of complications. 

Elevation of Legs: 

  • Lying or sitting with the legs elevated can assist enhance venous return and lessen edema. 

Nutritional Support: 

  • Nutritional support may be necessary, especially in cases where there is malnutrition or weight loss. Nutritional counseling and supplementation may be provided. 

Regular Exercise: 

  • While strenuous physical activity may be limited in some cases, regular, moderate exercise is generally encouraged to promote overall cardiovascular health and prevent deconditioning. 

Monitoring and Surveillance: 

  • Assessing the response to therapy and identifying any recurrence or problems requires routine monitoring using imaging examinations, tests for liver function, and other pertinent investigations. 

Psychosocial Support: 

  • Chronic illnesses such as BCS can have a significant impact on mental health. Psychosocial support, including counseling and support groups, may be beneficial for patients and their families. 

Weight Management: 

  • For general health, it’s critical to maintain a healthy weight. For certain people with obesity-related liver illness, losing weight may be advised. 

Avoidance of Contraceptives and Hormone Replacement Therapy: 

  • If BCS is associated with oral contraceptive use or hormone replacement therapy, discontinuation or alternative contraceptive methods may be advised. 

Role of Cardiovascular anticoagulants in the treatment of Budd-Chiari Syndrome

Anticoagulant therapy plays an important aspect in the management of Budd-Chiari Syndrome (BCS) by preventing further thrombus formation, promoting clot resolution, and reducing the risk of recurrence. Cardiovascular anticoagulants are often used in the treatment of BCS.  

  • Heparin: It is an injectable anticoagulant that is often used initially in the acute phase of BCS. It has a rapid onset of action and is typically administered intravenously. LMWH, such as enoxaparin or dalteparin, may be used as a bridge therapy or for long-term anticoagulation. LMWH has a more predictable anticoagulant effect, allowing for subcutaneous administration and less frequent dosing compared to UFH. 
  • Warfarin: It is an oral anticoagulant that is commonly used for long-term anticoagulation in BCS. It acts slowly and prevents the formation of clotting components that are dependent on vitamin K. Maintaining the anticoagulant effect within the therapeutic range requires routinely monitoring the international normalized ratio (INR). The target INR ranges between 2.0 and 3.0 for BCS. 

Role of Antifibrinolytic Agents in the treatment of Budd-Chiari Syndrome

If the body’s natural fibrinolytic system hasn’t been able to break down a pathological intraluminal thrombus or embolus, fibrinolytic medications can help. They are also employed in the quick correction of hemodynamic abnormalities and in the avoidance of recurrent thrombus development. 

  • Urokinase: Urokinase is a direct the plasminogen activator that activates the fibrinolytic system by converting the plasminogen into the enzyme plasmin, which then destroys fibrin blood clots fibrinogen, and other proteins in the plasma. The most common application is for superficial vessels and thrombosed catheters by local fibrinolysis. This chemical has the benefit of not being immunogenic. However, urokinase’s usage is restricted since it costs more than streptokinase. Urokinase is infused locally, straight into the thrombus site, without the need for a bolus, when used for local fibrinolysis. To accomplish clot lysis or maintain the patency of the afflicted conduit, the dosage needs to be changed.           
  • Alteplase: Tissue plasminogen activator alteplase is used to treat acute ischemic stroke, acute myocardial infarction, and pulmonary embolism. Its effectiveness and safety when used in conjunction with aspirin or heparin within the first twenty-four hours following the start of symptoms have not been studied. 

Role of Diuretic agents in the treatment of Budd-Chiari Syndrome

The primary goal in the treatment of Budd-Chiari Syndrome is to relieve the obstruction and restore normal blood flow to the liver. While diuretic agents are not the mainstay of treatment for BCS, they may be used as supportive therapy in certain cases. 

The role of diuretic agents in BCS is to manage complications related to fluid retention, which can occur as a consequence of impaired blood flow through the liver. They help to reduce excess fluid in the body, particularly in the abdominal cavity (ascites) and lower extremities (edema). By promoting diuresis, these agents alleviate symptoms associated with fluid overload and may improve the patient’s overall condition. 

  • Furosemide: This loop diuretic is often used to treat edema associated with liver disease, including Budd-Chiari Syndrome. It functions by preventing water and salt from being reabsorption in the kidneys’ loop of Henle. 
  • Spironolactone: This is a potassium-sparing diuretic that may be used in conjunction with furosemide. Spironolactone works by inhibiting the hormone aldosterone’s actions, which encourage water and salt retention. 

Use of Intervention with a procedure in treating Budd-Chiari Syndrome

  • Angioplasty: It involves the use of a balloon catheter to widen the narrowed or blocked blood vessels. In the context of BCS, angioplasty is often performed on the hepatic veins or the inferior vena cava (IVC) to dilate stenotic segments caused by blood clots or other obstructions. This procedure helps to restore venous patency and improve blood flow to the liver. 
  • Stent Placement: In cases where angioplasty alone is insufficient to maintain vessel patency, stent placement may be performed. A small mesh-like tube called a stent is put into a blocked or constricted vein to maintain its opening. Stents help to support the vessel walls and prevent re-narrowing (restenosis) after angioplasty. They are commonly used in the treatment of severe or recurrent Budd-Chiari Syndrome. 
  • Transjugular Intrahepatic Portosystemic Shunt (TIPS): TIPS is a specialized procedure that involves creating a shunt (artificial channel) within the liver to bypass the obstructed hepatic veins and improve blood flow. During TIPS placement, a catheter is inserted into the hepatic vein through the jugular vein under radiographic guidance. A stent is then deployed to create a connection between the hepatic vein and the hepatic vein or portal vein, bypassing the obstructed segment. TIPS is particularly useful in cases of refractory ascites or hepatic hydrothorax associated with BCS. 
  • Thrombectomy or Thrombolysis: In acute cases of BCS where there is a large thrombus obstructing blood flow, thrombectomy (surgical removal of the clot) or thrombolysis (administration of clot-dissolving medications) may be considered. These interventions aim to rapidly restore blood flow and prevent further liver damage. 
  • Liver Transplantation: When all previous treatments for Budd-Chiari Syndrome are ineffective and the patient has severe or refractory symptoms, a liver transplant may be the last resort. By surgically removing the damaged liver and replacing it with an appropriate donor’s liver, liver transplantation successfully restores regular functioning of the liver and blood flow. 

Use of phases in managing Budd-Chiari Syndrome

Acute Phase: 

  • Diagnosis: The initial phase involves prompt recognition and diagnosis of Budd-Chiari Syndrome based on clinical presentation, imaging studies (such as Doppler ultrasound, CT scan, or MRI), and laboratory tests. 
  • Anticoagulation: Immediate initiation of anticoagulant therapy is crucial to prevent further thrombosis and promote recanalization of the hepatic veins. Anticoagulation may include the use of heparin followed by oral anticoagulants like warfarin or direct oral anticoagulants (DOACs). 
  • Symptom Management: Treatment of acute symptoms such as abdominal pain, ascites, and hepatic dysfunction may involve supportive measures such as diuretics, analgesics, and dietary modifications. 
  • Thrombolysis or Thrombectomy: In cases of acute, extensive thrombosis causing severe liver ischemia, thrombolytic therapy or surgical thrombectomy may be considered to rapidly restore blood flow. 

Subacute Phase: 

  • Interventional Procedures: During this phase, interventional radiological procedures such as angioplasty, stent placement, or transjugular intrahepatic portosystemic shunt (TIPS) may be performed to relieve venous obstruction and improve hepatic blood flow. 
  • Monitoring: It is crucial to keep a close eye on imaging investigations, clinical symptoms, and liver function tests in order to monitor the disease’s development and determine how well the treatment is working.Adjustment of Anticoagulation: Anticoagulant therapy may be adjusted based on the patient’s response, coagulation profile, and risk of bleeding or thrombosis. 

Chronic Phase: 

  • Long-Term Anticoagulation: In patients with chronic or recurrent Budd-Chiari Syndrome, long-term anticoagulation therapy is often required to prevent thrombosis recurrence and maintain venous patency. 
  • Complication Management: Management of complications such as portal hypertension, ascites, hepatic encephalopathy, and hepatocellular carcinoma may be necessary in the chronic phase of BCS. This may involve pharmacological therapies, dietary modifications, and interventions such as TIPS or liver transplantation. 
  • Surveillance: Regular monitoring for complications and disease recurrence through imaging studies (e.g., Doppler ultrasound, CT/MRI scans) and clinical assessments is essential for early detection and intervention. 

End-Stage Phase: 

  • Liver Transplantation: In situations of resistant or severe Budd-Chiari Syndrome with permanent damage to the liver or failure, a liver transplant may be considered as a last resort therapy. 
  • Palliative Care: The interventions for patients with severe illness or who are not transplant candidates may include supportive treatment, symptom control, and quality of life enhancement. 

Medication

Media Gallary

Budd-Chiari Syndrome

Updated : March 18, 2024




In the absence of pericardial illness, heart conditions, or veno-occlusive disease (sinusoidal blockage syndrome), Budd-Chiari syndrome (BCS) is a rare ailment characterized by hepatic venous output tube obstruction, regardless of the degree and mechanism of blockage.

Obstruction brought on by a mostly venous condition is known as primary Budd-Chiari syndrome (thrombosis and phlebitis). The venae cavae and liver veins, on the other hand, may be compressed or invaded by a lesion that originates outside of the vein in secondary BCS (for example, a malignancy).

Budd-Chiari syndrome is typically identified in the 3rd or 4th years of life in non-Asian nations, mostly in females. Hepatic vein obstruction is the most typical cause.

In Asian nations, males are more likely to experience it, and inferior vena cava occlusion or even a combination of inferior vena cava & liver vein obstruction is the most frequent cause.

A single liver vein obstruction typically goes unnoticed, but when two liver veins are blocked, the hepatic capsule is stretched due to venous compression. This may cause severe agony. The sinusoids start to widen, and interstitial fluid starts to filter out, causing hepatic congestion. Ascites develops when the amount of filtrated fluid starts to pass through the hepatic capsule beyond the lymph system’s ability to drain it.

There is portal vein hypertension and a reduction in blood circulation to the liver through the portal vein as a result of the thrombosis and blockage to venous drainage. As a result, the centrilobular hepatocellular suffers hypoxic damage. This can result in liver failure in severe acute instances, while ascites and hepatomegaly with maintained liver function can result in cases of severe.

Cirrhosis results from the development of fibrosis over time. Since its blood is directly directed into the inferior vena cava, the caudate lobe is the one that is most frequently afflicted by BCS. As a result, the caudate lobe enlarges when the liver veins are blocked.

Budd-Chiari syndrome is the result of an underlying condition in 80% of instances, with a hypercoagulable condition being the most common culprit. When diagnosing and treating Budd-Chiari syndrome, this element must be considered.

The following are the primary contributing factors to BCS:

Budd-Chiari syndrome is the result of an underlying condition in 80% of instances, with a hypercoagulable condition being the most common culprit. When diagnosing and treating Budd-Chiari syndrome, this element must be considered.

The following are the primary contributing factors to BCS: 

Malignancy

Malignancy accounts for 10 percent of cases of BCS and can either directly compress arteries or invade them. All of these contribute to hypercoagulability and venous thrombosis, which can restrict blood flow.

Hepatocellular carcinoma, leiomyosarcoma, renal cell carcinoma, Wilms tumor, and right atrial myxoma are the most frequent cancers connected to the Budd-Chiari syndrome.

Myeloproliferative diseases 

Due to the nearly universal presence of hypercoagulability in myeloproliferative illnesses, including polycythemia vera & critical thrombocythemia, about half of all cases of Budd-Chiari syndrome are associated with these conditions.

Idiopathic

20 percent of instances are idiopathic.

The following hypercoagulable conditions also contribute to Budd-Chiari syndrome:

A mutation in factor V (Leiden) causes resistance to protein C antibodies to phospholipids syndrome, a lack of antithrombin protein C shortage of hemoglobinuria at night that is recurrent

During pregnancy, OCPs (oral contraceptives) 

A hypercoagulable state brought on by contraceptive pills & pregnancy accounts for roughly 20% of instances of Budd-Chiari syndrome.

Lesions of the liver 

The constraint of the vasculature can occasionally be caused by an infection or a lesion of the hepatic that takes up space.

A favorable prognosis for Budd-Chiari syndrome is influenced by:

  • Low serum creatinine level
  • Young age
  • Ascites is absent
  • Poor Child-Pugh rating

Consequences of a poor prognosis:

Involvement of the portal vein and/or the 3 hepatic capillaries

  • Age at the onset of the presentation was higher
  • Superior Child-Pugh rating
  • Chronic illness when first diagnosed

Age Group: