Budd-Chiari syndrome

　　The condition is caused by obstruction of the inferior vena cava above the hepatic veins and (or) the outlet of the hepatic veins. In Western countries, Budd-Chiari syndrome is often due to hypercoagulable states leading to hepatic vein thrombosis. It usually does not involve the inferior vena cava, or it may secondary hypertension of the inferior vena cava due to the obvious external pressure on the inferior vena cava by an enlarged liver.

　　而在东方国家，则以发育异常为多见。在胚胎发育过程中，心下静脉向下、肝总静脉向上生长，而后二者连接后必须沟通。若二者发育到一定阶段而停止，即可导致下腔静脉不连或连接不全。发育异常多为隔膜型、或蹼状、或筛状、或膜状。部分病人为肝静脉内血栓形成，血栓也可繁衍至肝后下腔静脉，形成肝静脉-下腔静脉阻塞。

　　In Eastern countries, developmental abnormalities are more common. During the process of embryonic development, the inferior vena cava grows downward under the heart, and the hepatic veins grow upward. After the latter two connect, they must communicate. If they stop growing at a certain stage, it can lead to incomplete or incomplete connection of the inferior vena cava. Developmental abnormalities are mostly septal type, or web-like, or sieve-like, or membranous. Some patients have intravascular thrombosis in the hepatic veins, and the thrombus can also propagate to the inferior vena cava behind the liver, forming a hepatic vein-inferior vena cava obstruction.

　　Clinical manifestations include liver and spleen enlargement and tenderness, progressive massive ascites, mild jaundice, etc. If there is inferior vena cava obstruction, there will be obvious lower limb edema, varicose veins of the chest and abdominal wall. A few patients have acute onset, often suffer from other diseases such as renal cancer, primary liver cancer, thrombophlebitis, etc., presenting with abdominal pain, vomiting, liver enlargement, ascites, and jaundice, which can quickly lead to delirium, coma, liver function failure, and death.

　　Banti's syndrome is caused by obstruction of the inferior vena cava above the hepatic veins and (or) the outlet of the hepatic veins, leading to liver congestion, liver enlargement, liver cirrhosis in the late stage, splenomegaly, ascites, and portal hypertension, etc.

　　1. Diet should provide sufficient nutrition, food should be diverse, provide high-priced proteins containing amino acids, a variety of vitamins, low-fat, and low-residue diet, to prevent rough and fibrous foods from damaging the esophageal veins, causing massive bleeding.

　　2. Patients with high blood ammonia or poor liver function should limit protein intake to prevent liver coma. Patients with ascites should consume low-salt or salt-free diet.

　　3. Measure the abdominal circumference and measure urine output daily, abdominal obesity can be a major method to self-distinguish fatty liver.

　　4. Pay attention to changes in bleeding, purpura, fever, and changes in mental and neurological symptoms, and contact the doctor in a timely manner.

　　5. Selenium supplementation for liver protection, selenium can help the activity of glutathione peroxidase in the liver reach normal levels, which has a good effect on liver protection. Selenium yeast powder, Schisandra chinensis component of the liver protection tablet, has a good effect on liver protection, immune regulation, and has a good effect on liver and liver protection.

　　In chronic patients, serum transaminase is normal or slightly elevated, prothrombin activity is not low, half of the patients have functional renal failure, and acute patients have significantly elevated serum transaminase levels and decreased thrombin activity during the onset period.

　　Under imaging examination, inferior vena cava angiography can obtain a confirmed diagnosis, and Doppler examination can detect venous dilation above the obstruction.

　　1. Dietary requirements:

　　1. Consume low-salt soft food or semi-liquid food to ensure sufficient calories and protein.

　　2. Do not eat the following acidic foods: oil-refined, pickled, cola, hamburger, milk, sugar, nuts, beans, meat dishes.

　　3. Avoid spicy foods.

　　4. Avoid high copper diet.

　　2. Pay attention to daily life:

　　1. Avoid smoking.

　　2. Avoid irregular lifestyle.

　　1. Interventional treatment

　　Percutaneous transluminal angioplasty (PTA) or intravascular balloon dilatation, a relatively safe, simple, minimally invasive, almost zero mortality rate, and no obvious contraindications treatment method established in recent years. Eguchi first used this method to successfully treat inferior vena cava membranous obstruction. Due to its above advantages and high efficacy, it has become the first choice for treating membranous obstructions and can also be used for the treatment of阶段性 obstruction. The surgical method of PTA is not complex and can be performed after the stenosis or occlusion of the inferior vena cava is confirmed by venography. If it is completely occluded, a rigid catheter or heart valve knife is first used to open the septum, and then balloons of different diameters are used to dilate the stenotic area. A 30% iohexol is used as the inflation agent and pressure transmission agent for pressure injection, and the situation of stenosis dilation is observed simultaneously. To understand the treatment effect, venography and pressure measurement can be performed again after dilation. In recent years, some scholars have also used laser to打通 the stenotic ring and then perform balloon dilation. To prevent recurrence, a new treatment method called percutaneous transluminal stent placement (PTS) has been developed on the basis of PTA in recent years. Due to its high efficacy and low recurrence rate, it is more welcomed by doctors and patients. The method is similar to PTA, and a 2cm expandable metallic stent is introduced after balloon dilation to open the stenotic area, thus establishing a venous flow channel. In the past, there were fewer reports of PTS in China, but in recent years, the number of reports has gradually increased, showing a trend of gradually replacing PTA.

　　2. Surgical treatment

　　Surgical treatment has various methods, and the number of cases reported for various surgical methods is not many. Due to the lack of long-term efficacy observation, it is difficult to compare various surgical methods. The choice of surgical method should be based on the patient's condition, such as whether they have bleeding, ascites, and their tolerance to surgery.

　　1. Membrane fragmentation: The operator uses the index finger to enter the inferior vena cava through the right atrial opening, using the finger to break through the septum. A vascular dilator can be used if necessary. This method is simple, quick, and effective, hence its adoption. Membrane fragmentation is applicable to inferior vena cava obstructions above the diaphragm that can be reached by the index finger.

　　2. Shunt surgery:

　　(1) Shunt surgery to reduce portal pressure: It is suitable for patients with obstructed hepatic veins and patent inferior vena cava. The surgical methods include portal-caval shunt and superior mesenteric vein-inferior vena cava shunt, etc.

　　(2) Shunt surgery to reduce the pressure of the inferior vena cava: The main methods include inferior vena cava-right atrium bridging surgery.

　　(3) Shunt surgery to reduce the pressure of the hepatic veins and inferior vena cava: The main surgical methods include mesenteric vein-right atrium bridging surgery.

　　Shunt surgery can quickly relieve the blood stasis at the distal end of the blocked vein, and after surgery, patients can be seen with reduced liver and spleen size, regression of ascites, and reduced superficial varicose veins, making it an effective treatment method. However, shunt surgery has a large trauma, the surgery is also difficult, and serious complications such as compression of implanted vessels, new thrombosis formation, heart failure, hepatic encephalopathy, and pulmonary embolism may occur, which are difficult for some patients to tolerate, and should be paid more attention to.

　　3. Splenopulmonary fixation: Splenopulmonary fixation is suitable for patients with high portal pressure, ascites, and esophageal varices. It was first used in 1962 by Akita in Japan to treat portal hypertension and in 1964 for BCS. Akita summarized that the operation is effective, can reduce portal pressure, measured to divert 20% of the total liver blood flow, simple and safe; it can maintain effective liver blood flow; there are no embolic complications; it has no effect on lung function. The spleen itself can play a regulatory role in blood flow distribution. However, the short-term effect of this operation is not significant, and it is not suitable for emergency patients with severe gastrointestinal bleeding or those with intractable ascites before surgery.

　　4. Liver transplantation: If the condition cannot be well controlled, it develops into severe liver damage, and liver failure occurs, liver transplantation surgery can be considered. Liver transplantation is an effective method to save the patient's life, but it requires a high level of technical expertise, is expensive, and also requires timely provision of liver donors. The patient will still need comprehensive treatment in the long term after surgery, including anti-rejection therapy, and there are still certain difficulties in promotion.

　　5. Other surgical methods: There are also methods of resection of the affected tissue, which may be a more superior treatment method.

　　3. Prognosis

　　Ultimately, death can occur due to hepatic failure and gastrointestinal bleeding caused by congestive liver cirrhosis, with poor prognosis.