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
Physical Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
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Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
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by Stage
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References
https://www.ncbi.nlm.nih.gov/books/NBK558941/
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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
https://www.ncbi.nlm.nih.gov/books/NBK558941/
medtigo
Budd-Chiari Syndrome
Updated :
November 23, 2022
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
https://www.ncbi.nlm.nih.gov/books/NBK558941/
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