What is Budd-Chiari syndrome? The disease was first named and defined by two foreigners, Budd and Chiari, so people call this type of disease Budd-Chiari syndrome, which translates to Buga syndrome. How does Budd-Chiari syndrome develop? Budd-Chiari syndrome is a form of post-hepatic portal hypertension caused by obstructive lesions of the hepatic veins and/or the inferior vena cava above its opening, often accompanied by a syndrome of inferior vena cava obstruction. The obstruction can occur anywhere from the output veins of the hepatic lobules to the inferior vena cava of the hepatic segment, hence the name hepatic venous obstruction syndrome. What is portal hypertension? The term portal vein refers to the portal venous system, which receives reflux venous blood from internal organs (liver, spleen, gastrointestinal, etc.), which is then metabolized by the liver and returned to the heart. Portal hypertension refers to an increase in pressure within the portal venous system, which can lead to difficulty in the return of blood to its corresponding internal organs and consequent blood stasis. What are the manifestations of Buga syndrome? There are three types of Buga syndrome: hepatic venous obstruction, inferior vena cava obstruction, and a combination of both, and thus the manifestations are different. In pure hepatic vein obstruction, symptoms of portal hypertension are predominant, manifested by enlargement of the liver and spleen, bruising and edema in the gastrointestinal tract, followed by loss of appetite, dyspepsia, even blood in the stool and vomiting, etc., with ascites, cirrhosis and jaundice at the end stage; in inferior vena cava obstruction, clinical manifestations of both portal hypertension and inferior vena cava obstruction syndrome are present, with edema in both lower limbs, varicose veins and persistent ulcers in the lower limbs. Who is at risk of developing Buga syndrome? In Western countries, Buga syndrome is mostly caused by a hypercoagulable state of blood flow leading to hepatic vein thrombosis without involving the inferior vena cava, or by external pressure on the inferior vena cava from a markedly enlarged liver (especially the caudate lobe) secondary to inferior vena cava hypertension. In eastern countries, such as China, India, Japan and Korea, developmental anomalies are more common. Most often there is a septum, either webbed or sieve-shaped, between the inferior vena cava and the superior vena cava. There are also high-risk groups, such as frequent oral contraceptive use, abdominal tumors or pregnancy that can lead to the development of the disease. Can I be treated with medication for Buga syndrome? The outcome and treatment options for Buga syndrome vary from cause to cause. Inferior vena cava septal type and abnormal development of hepatic veins are not treatable by medication. In the case of inferior vena cava thrombosis caused by a hypercoagulable state of the blood due to oral contraceptives, etc., it is necessary to stop the use of pro-hypercoagulant drugs after successful treatment. Does Buga syndrome always require surgery? There are very good communications (veins) between the portal system and the vena cava. Under normal circumstances, these communicating veins are slender and in a hidden state, but when the pressure in the portal system is elevated, the blood backs up, thus also initiating these hidden vessels, which thicken and enlarge to assist in the return of blood to the internal organs. In some patients the onset is slow, compensated vessels gradually form without significant complications and do not affect the quality of life, and can be considered for observation and follow-up without surgical treatment, but this is still a rare occurrence, and the vast majority of patients still require surgical treatment. What are the surgical treatment options for Buga syndrome? Surgical treatment of Buga syndrome includes both symptom relief and resolution of the lesion. For symptom relief, there is sclerotherapy of the varicose veins in the fundus of the stomach, etc. In contrast, surgery is required to address the root cause of all symptoms of the disease. There are traditional open surgical procedures and emerging interventional treatments. Open surgery is very invasive and even requires extracorporeal circulation. Interventional treatment, on the other hand, is less invasive and more effective, and is a new treatment method that has emerged rapidly in recent years.