Budd chiari syndrome, the occlusion of hepatic veins (Obstruction of back flow of blood from liver to heart due to blockage) is an unusual condition which may be manifested by pain in the abdomen, ascites (build-up of fluid in the abdomen), hepatomegaly (liver enlargement) and a poor prognosis.
Anywhere throughout the vein's path, from the hepatic venules to the inferior vena cava (large vein that carry deoxygenated blood) junction and the right atrium (heart chamber), the obstruction could be thrombotic (related to blood clot) or non-thrombotic.
This disorder is an uncommon ailment considering its occurrence in 1 of 100000 individuals. In medical language Budd chiari syndrome is also known as hepatic venous outflow obstruction (HVOO).
Budd chiari syndrome is a diverse clinical disorder that can either be fatal if left untreated or may be managed with efficient treatment. When managed appropriately, the patients' prognosis is acceptable when compared to other chronic liver illnesses. Budd chiari syndrome can aggravate several conditions, including hematologic or malignant diseases, making it a rare significant syndrome.
Budd Chiari syndrome definition
Budd-Chiari syndrome is a rare liver condition that is defined as, obstruction to the outflow of blood in hepatic veins at any point, ranging from the small hepatic vein to the junction of the right atrium (heart chamber) and inferior vena cava (largest vein in the body carrying deoxygenated blood).
Budd chiari syndrome meaning
The phrase "Budd-Chiari" was first used in the late 1800s in reference to the eminent work of internist George Budd a British physician and Austrian pathologist Hans Chiari.
A brief report on three patients who experienced hepatic vein blockage was published by George Budd in 1845. The illness, which has never been documented before, was linked to alcoholism and sepsis. 53 years later, Hans Chiari proposed the theory that syphilis was the reason for the hepatic vein blockage, adding pathological and clinical connections to the discussion postulated by George Budd.
Hepatic venous outflow obstruction is a union of 4 words in which.
With a low incidence and prevalence in the general population, budd-chiari syndrome is an atypical serious liver condition. The incidence recorded in the literature varies from 0.2 to 4.1 cases per 10 lakh people annually, with an estimated prevalence of 1.4–4.0 cases per 10 lakh people in Western countries and 2.4–7.7 cases per million people in Asian countries. In Western studies, the prevalence was higher in women (52–69%), whereas in Asian studies, men were more often affected (48–70%).
Budd chiari syndrome has higher prevalence in developing countries like India.
Based on the location where the obstructive lesion is originated, Budd Chiari syndrome can be categorized into two types they are:
Depending on whether the obstruction happens suddenly or gradually, various signs and symptoms may be experienced which may include:
Budd-Chiari syndrome develops because of an underlying etiology in 80% of instances. These instances are primarily associated with a hypercoagulable state (a condition that increases the risk of blood clotting). The predominant causes of budd-chiari syndrome include:
Thrombosis (blood clot with complete or partial blockage)
Hepatic vein blockage is primarily caused by thrombosis. Venous thrombosis, especially hepatic vein thrombosis, requires the coexistence of one or more thrombogenic (prone to blood clot formation) diseases and a triggering stimulus. The majority of budd chiari syndrome patients have a medical condition that makes them more susceptible to blood clotting.
Myeloproliferative disorders (blood cancers that result in an excessive production of blood cells in the body)
The primary cause of hepatic vein thrombosis is myeloproliferative disorders. Since some myeloproliferative illnesses, such as polycythemia vera (blood cancer) and essential thrombocythemia (blood cancer that increase platelet count), are invariably associated with hypercoagulability (condition that increases the risk of blood clotting), these conditions are linked to about half of the cases of Budd-Chiari syndrome.
Malignancy (the existence of cancerous cells with the potential to spread to different parts of the body)
Malignancy is linked to 10% of cases of Budd-Chiari syndrome, and it either directly compresses or invades blood vessels. Malignancy accompanied by hypercoagulability cause venous thrombosis and blockage. Other types of cancers that are more predominant to cause Budd Chiari syndrome include:
Liver lesions (abnormal cell growth in the liver that may be cancerous or non-cancerous)
Compression of the vasculature can occasionally result from an infection or a lesion that takes up space in the liver. liver lesions can give rise to this disorder due to:
Pregnancy and use of oral contraceptive pills
An increased risk of budd chiari syndrome is associated with the use of oral contraceptives, especially those with a high estrogen level. Several reports states that patients in whom budd chiari syndrome was occurred due to oral contraceptives or pregnancy, may also have an underlying thrombophilia, either acquired or inherited.
Idiopathic (disease with no identifiable cause)
It is demonstrated that 20% cases of budd chiari syndrome occur due to unknown cause.
Gastric diseases
Studies have demonstrated that when underlying thrombophilias (condition that cause blood clots formation too easily) are present, budd chiari syndrome may be caused by ulcerative colitis, celiac disease, or abdominal trauma.
Budd-Chiari syndrome, may be caused by additional hypercoagulable conditions, such as:
Several risk factors have been linked to Budd-Chiari Syndrome, which may raise the chances of developing the disorder. Some of them include:
Budd-Chiari syndrome complications are primarily associated with the severity of liver failure and underlying medical problems. Untreated Budd-Chiari syndrome generally may have complications such as:
Managing the risk factors that give rise to Budd-Chiari Syndrome is the main strategy for preventing this disorder, along with general tactics that could aid in the prevention, such as:
The diagnosis of Budd-Chiari syndrome cannot be made with a single test. Based on traditional clinical signs and factors that increase the risk of thrombosis, such as the existence of cancer, the hepatologist / cardiologist may diagnose budd chiari syndrome by using below mentioned diagnostic procedures:
Budd chiari syndrome radiology
In order to reduce blockage, stop clot progression, minimize progressive liver injury and avoid or control consequences, this condition can be treated by below-mentioned treatment options:
Medical management (treatment using medicine)
Interventional therapy (treatment involving minimally invasive techniques)
The reason, how the symptoms manifest, and the extent of the sickness all influence the course of treatment for budd chiari syndrome. A multidisciplinary approach using a combination of medical and interventional therapy is necessary for the management, which is frequently challenging.
Yes. Appropriate treatment may reverse this condition. According to a 2011–2016 study, the technical and clinical success rates of endovascular therapy for budd chiari syndrome of the hepatic vein type were 100% and 95.6%, respectively. After a year, the primary and secondary patency rates were 80.0% and 93.8%, 72.8% and 90.3%, and 67.9% and 91.2%, respectively. After one year, survival was 96.9%, followed by two years at 93.4% and five years at 91.2%.
Eighty percent of cases have an underlying reason, most of which have to do with a hypercoagulable state, contributing to the development of budd-chiari syndrome. How ever conditions like liver lesions, cancers may have the potency to cause budd chiari syndrome.
If budd chiari syndrome left untreated, the prognosis of the disease shall be poor. Death may occur in patients between 3months to 3 years of the diagnosis due to progressive degeneration of liver. Nonetheless, the 5-year survival rate for individuals with the condition after portosystemic shunting is 38–87 percent. After liver transplantation, the actuarial 5-year survival rate is 70%.
No. Budd chiari syndrome is not an autoimmune disease. However autoimmune disorders such as systemic lupus erythematosus and Sjögren syndrome, (an autoimmune condition characterized by the patient's white blood cells attacking the tear and salivary glands, resulting in persistently dry mouth and eyes) etc may increase the risk of patient to develop budd chiari syndrome.
Abdominal pain, ascites (buildup of fluid in the abdomen) and hepatomegaly (liver enlargement) are the three main classical traids that most individuals with budd-chiari syndrome express. It takes a high level of suspicion to identify this ailment because the signs are non-specific and present in a variety of ways.
The clinical manifestations of liver failure can differ; they might be acute or fulminant (severe and sudden in onset) subacute (signs or symptoms for less than six months without validation of liver cirrhosis), or chronic (signs or symptoms for more than six months with evidence of portal hypertension (increased blood pressure in the vein that carry blood to liver from intestines) and cirrhosis).
It is essential to incorporate items such as fruits, whole grains, lean proteins, healthy fats etc that are beneficial to the liver into diet when managing budd-chiari syndrome. Two important aspects of treating the illness are lowering inflammation and promoting liver function, which are essential for controlling the condition.
Both the males and females are equally affected by budd chiari syndrome. The majority of cases typically impact people in the twenty- to forty-year-old age range.
Venous outflow obstruction at the hepatic veins or inferior vena cava characterizes budd chiari syndrome. Whereas portal vein thrombosis results from occurs from occlusion in the extra hepatic venous system (veins outside liver)
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