Budd-Chiari Syndrome

　　The causes of the Budd-Chiari syndrome include thrombosis of the hepatic veins and inferior vena cava, compression by lesions of adjacent organs, and intrinsic lesions of the hepatic veins and inferior vena cava, such as congenital developmental abnormalities, occlusive phlebitis, and others.

　　1. The causes of this syndrome include thrombosis of the hepatic veins and inferior vena cava, compression by lesions of adjacent organs, and intrinsic lesions of the hepatic veins and inferior vena cava, such as congenital developmental abnormalities, occlusive phlebitis, and others.

　　1. Splenomegaly and hyperfunction of the spleen.

　　2. Ascites.

　　3. Treatment of other complications: hepatic encephalopathy, upper gastrointestinal bleeding, and hepatorenal syndrome. The virus can counteract the human immune system, causing immunosuppression, liver cell damage, and developing into liver cirrhosis. Liver cirrhosis is also a common cause of liver cancer.

　　This syndrome often has a chronic course, and its clinical manifestations mainly include two aspects:

　　1. Manifestations of hepatic vein reflux obstruction, often with progressive enlargement of the liver and spleen, varicosities of the esophagus and abdominal wall, ascites, pain in the liver area, and abdominal pain, etc. Jaundice is rare, and there may be varying degrees of fever and pain in the liver area during the acute phase of venous thrombosis, even shock and death. In the late stage, portal hypertension and signs of liver cirrhosis may appear.

　　2. Manifestations of inferior vena cava reflux obstruction, such as edema of the lower extremities, cyanosis, superficial venous varicosities, hyperpigmentation or ulcers, a feeling of沉重麻木 in the lower extremities, and the blood flow direction of varicose veins showing a consistent upward trend.

　　The manifestations vary greatly depending on the extent and degree of vascular involvement, the nature and state of the obstructive lesions, and other factors. It can be divided into acute, subacute, and chronic types.

　　1. Acute type:Mostly caused by complete obstruction of the hepatic veins, with the obstructive lesions mostly being thrombosis. It usually starts at the outlet of the hepatic veins, with the thrombus rapidly spreading to the inferior vena cava. The onset is acute, with sudden upper abdominal distension and pain, accompanied by nausea, vomiting, abdominal distension, diarrhea, resembling acute hepatitis. The liver gradually swells, becomes tender, and is often accompanied by jaundice, splenomegaly, and rapid increase in ascites. At the same time, there may be pleural effusion. In fulminant cases, hepatic encephalopathy can occur rapidly, with progressive jaundice, oliguria or anuria, and may be complicated by disseminated intravascular coagulation (DIC), multiple organ failure (MOSF), spontaneous bacterial peritonitis (SBF), and others. Most patients can die rapidly due to circulatory failure (shock), liver failure, or gastrointestinal bleeding within a few days or weeks. Ascites, liver enlargement, and the rapid onset of MOSF are the prominent manifestations of this disease.

　　2. Subacute type:It is usually involved simultaneously or sequentially by the hepatic veins and inferior vena cava, with refractory ascites, liver enlargement, and lower limb edema coexisting, followed by superficial varicose veins on the abdominal wall, lumbar back, and chest, with blood flow in an upward direction, which is an important feature that distinguishes Budd-Chiari syndrome from other diseases. Jaundice and splenomegaly are only seen in 1/3 of patients, and most are mild or moderate. Many cases show acute and persistent formation of ascites, increased abdominal pressure, elevation of the diaphragm, and in severe cases, abdominal compartment syndrome (abdominal compartmentsyndrome, ACS) may occur, causing systemic physiological disorders. When the abdominal pressure rises to 25cmH2O and 50cmH2O, oliguria and anuria appear, respectively. The chest volume and lung compliance decrease, cardiac output decreases, pulmonary vascular resistance increases, leading to hypoxemia and acidosis.

　   3. Chronic type:The course of the disease can last for several years, and it is more common in patients with diaphragmatic type obstruction, with mild disease, but with noticeable signs, such as large, tortuous, dilated veins on the chest and abdominal wall, hyperpigmentation in the area of the lower leg, and some chronic ulcers. Although there may be varying degrees of ascites, most tend to be relatively stable. There may also be jugular venous distension, varicocele, large inguinal hernia, umbilical hernia, hemorrhoids, etc. Esophageal varices often do not attract the patient's attention and are usually confirmed by endoscopy or X-ray angiography when the patient seeks medical attention due to sudden hematemesis, black stools, or the discovery of splenic enlargement. In this type of patient, liver enlargement is not as obvious as in the subacute type, and it is mostly half-liver size, but the degree of cirrhosis has increased, and the spleen is mostly of moderate size, rarely showing a large spleen as in the intrahepatic type of portal hypertension.

　　4. Late-stage patients:Due to malnutrition, protein loss, increased ascites, and weight loss, a typical 'spiderman' body shape may appear.

　　This disease is more common in young males, with a male-to-female ratio of about 1.2～2∶1, and age ranges from 2.5 to 75 years, with most cases occurring between 20 and 40 years old.

　　1. The degree of liver function injury is relatively mild, and there may be delayed excretion of indocyanine green, elevated serum alkaline phosphatase, transaminases, and decreased albumin.

　　2. B-ultrasound and CT examination can show the location, extent, degree of stenosis and occlusion of the hepatic veins and inferior vena cava, as well as changes in the shape of the liver.

　　3. Inferior vena cava angiography and selective hepatic venography can accurately determine the location, extent, nature, collateral circulation, and presence of external compression of the obstructive lesion.

　　4. Liver biopsy shows congestion and sinus dilation around the central vein of the lobule, and liver cirrhosis changes in the late stage.

　　1. Diet

　　(1) The diet for liver and spleen enlargement is not special, with sufficient calories, appropriate protein, rich and comprehensive vitamins, and appropriate fiber. It is advisable to cook with light flavors. Secondly, the dietary structure should be reasonable. More vegetables and fruits should be eaten to supplement sufficient vitamins and fiber, which also helps to promote digestive function. When liver function decreases, it often affects fat metabolism, so many patients with chronic hepatitis also have post-hepatitis fatty liver. Therefore, the diet should be low in fat and sugar (excessive sugar entering the human body is easily converted into fat), and high in protein. Protein diet should include both plant protein and animal protein, such as soy products, beef, chicken, fish, etc.

　　(2) In addition, the amount of food should be appropriate. During liver disease, digestive function is weakened, and overeating often leads to indigestion and also increases the burden on the liver. Therefore, it is proposed that eating to 80% of fullness is the best. Excessive eating and drinking is harmful to both the liver and gastrointestinal function.

　　2. Liver disease patients should avoid eating

　　(1) Avoid spicy food

　　(2) Avoid smoking

　　(3) Other

　　(4) Avoid emotional disorders

　　(5) Avoid fatigue

　　First, the Western medical treatment method of Banti syndrome

　　1. Medical Treatment

　　Medical treatment includes low-sodium diet, diuretics, nutritional support, and autologous ascites reinfusion, etc. For patients with acute onset within one week and simple thrombosis, anticoagulant therapy can be used, but most cases are diagnosed several weeks or months after thrombosis. For most cases, although conservative treatment can win time for the formation of collateral circulation, patients still need surgical treatment in the end. Patients with Budd-Chiari syndrome, especially advanced patients, often have refractory ascites and severe malnutrition. As a supportive therapy before surgery, medical treatment can improve the overall condition of the patient, reduce the mortality rate of surgery, and be conducive to the postoperative recovery of the patient.

　　2. Surgical Treatment

　　(1) Septal Rupture Technique:

　　①Balloon Catheter Dilation Technique: The method is to insert a balloon catheter through the femoral vein, position the balloon section of the catheter at the stenotic site under fluoroscopy, and inject an appropriate amount of contrast agent into the balloon to cause the balloon to expand and tear the septum. This method is suitable for septal obstruction with no thrombosis at the distal end. Possible complications of this surgery include cardiac tamponade, pulmonary embolism, and catheter breakage.

　　②Right Atrial Septal Rupture Technique: The method is to enter the thoracic cavity through a right anterior fourth intercostal extrathoracic incision or through a sternotomy, and make a longitudinal incision in the pericardium in front of the right phrenic nerve.

　　(2) Inferior Vena Cava to Right Atrium Shunt Operation:

　　①The Anterior Hepatic Approach: Through a median abdominal incision or a right anterior rectus muscle incision, the following methods can be used to expose the inferior vena cava: A. Perform a Kocher incision to free and flip the duodenum to the left to expose the inferior vena cava; B. Flip up the transverse colon and its mesentery, push the small intestine to the left, open the retroperitoneum below the horizontal part of the duodenum and on the right side of the superior mesenteric vein, and dissect towards the direction of the abdominal aorta. The inferior vena cava should be exposed for at least 4cm. Thoracic incisions can be made using a sternotomy or a right anterior extrathoracic incision, using a 14 or 16mm artificial vascular graft to perform an end-to-side anastomosis with the inferior vena cava and an end-to-side anastomosis with the right atrium. The artificial vascular graft usually enters the thoracic cavity from behind the transverse colon, in front of the stomach and liver.

　　② Post-hepatic approach: The patient is in a left lateral position, and the chest is entered from the right 7th rib. Open the pericardium to expose the thoracic segment of the inferior vena cava. Incise the diaphragm, dissect downward along the inferior vena cava until its dilated or relatively normal part, and anastomose one end of the artificial blood vessel with the dilated part of the inferior vena cava at the distal end of the stricture, and the other end with the inferior vena cava above and below the diaphragm or the right atrium. Compared with the anterior approach to the liver, the post-hepatic approach requires a shorter artificial blood vessel, has less chance of thrombosis, but is more difficult to operate, prone to bleeding, and has a relatively higher incidence of postoperative chyle pleural effusion. The inferior vena cava-right atrium shunt restores the blood return of the inferior vena cava and is suitable for cases with long inferior vena cava obstruction lesions and relatively patent hepatic veins.

　　(3) Superior mesenteric vein-right atrial shunt surgery: Enter the abdomen through an upper midabdominal incision, find the superior mesenteric vein at the root of the transverse mesocolon on the right side of the Treitz ligament. The thoracic incision can be a sternotomy or right anterior extrathoracic incision. Use a 14 or 16mm artificial blood vessel for side-to-side anastomosis with the superior mesenteric vein, and the other end for anastomosis with the right atrium.

　　(4) Radical surgery: For cases with high caval obstruction and septal type, chest can be entered from the right 7th rib, the thoracic and part of the abdominal segment of the inferior vena cava are dissected out, and the two ends of the lesion are controlled. The inferior vena cava is longitudinally incised, and the lesion is resected. If the obstruction lesion is extensive or there is a large amount of thrombus formation at the distal end, the hepatic segment of the inferior vena cava can be longitudinally incised under extracorporeal circulation, the septum, thrombus, and other lesions are resected, the hepatic vein is explored and its patency is restored, and the inferior vena cava is repaired with Gore-Tex or Dacron patch. Although radical surgery directly removes the primary lesion, there is still a possibility of recurrence in cases with concomitant inferior vena cava inflammation.

　　Secondly, traditional Chinese medicine treatment methods for Bei-Zha syndrome

　　1. Salvia miltiorrhiza injection, 4 milliliters each time, added to 40 milliliters of 50% glucose solution for intravenous injection, or use 10 to 20 milliliters of the injection, added to 500 milliliters of 5% glucose solution for intravenous drip, once a day, 15 times as one course of treatment. It can be used for 2 to 3 courses.

　　2. Ligustrazine injection, 80 to 160 milliliters each time, added to 500 milliliters of 5% glucose solution for intravenous drip, once a day, 10 times as one course of treatment, which can be used for 1 to 2 courses. Alternatively, use Thrombosis Relief 8 to 12 milliliters plus 250 milliliters of 0.9% normal saline for intravenous drip, once a day, 3 to 4 weeks as one course of treatment.

　　3. Puerarin injection, 200 to 400 mg each time, added to 250 to 500 milliliters of 10% glucose for intravenous drip. Once a day, 10 to 20 days as one course of treatment, which can be used for 2 to 3 courses.

　　4. Xueping tablets, taken orally, 3 tablets each time, three times a day.

　　5. Salvia miltiorrhiza tablets, taken orally, 3 to 4 tablets each time, three times a day. Alternatively, use compound moon root dropper pills, taken orally, 10 pills each time, three times a day.

　　6. Eucommia ulmoides injection, 2 milliliters each time, intramuscular injection, once or twice a day.

　　7. Single Formula and Prescriptions

　　(1) Yuangang Qianyang Tongmai Decoction

　　Prescription: Rehmannia glutinosa, Conus musculus, Os Draconis, Oyster shell, each 15 grams, Lycium barbarum, Paeonia lactiflora, Adenophora trachycarpa, Ophiopogon japonicus, Anemarrhena asphodeloides, Coptis chinensis, Achyranthes bidentata, Salvia miltiorrhiza, Paeonia lactiflora, Cicadae 10 grams, Equisetum hyemale 10 grams. Decoct and take the decoction again after discarding the dregs.

　　(2) Main Formula: Xuefu Zhuyu Decoction

　　Prescription: Angelica sinensis 10 grams, Rehmannia glutinosa 25 grams, Persica 10 grams, Carthamus tinctorius 6 grams, Paeonia lactiflora 15 grams, Atractylodes macrocephala 10 grams, Bupleurum chinense 6 grams, Achyranthes bidentata 12 grams, Ligusticum chuanxiong 6 grams, Platycodon grandiflorus 10 grams, Dendrotoa 10 grams, Scrophularia ningpoensis 15 grams. Decoct and take the decoction again after discarding the dregs.

　　If there is headache, irritability, bitter taste in the mouth, red tongue, add 30 grams of water buffalo horn, 10 grams of Scutellaria baicalensis, 10 grams of Gentiana scabra. For those with constipation, add 10 grams of Rhei and 10 grams of Xuanmingfen. For those with a large amount of mesenteric hemorrhage that is difficult to absorb, add scorpions, leeches, trigonella, and rhizoma et rhizoma Curcumae, etc., to enhance the power of removing blood stasis, but should not be taken for a long time. For those with headache and eye distension, add ophthalmolysis and Uncaria rhizoma. For young patients caused by inflammation, add Lonicera japonica, Viola phaeocalyx, and Forsythia. For those with dizziness and side pain, add Sparganium stoloniferum and Pelargonium.

　　3. Prognosis

　　The prognosis of this disease varies greatly due to the causes, locations, and degrees of obstruction. In terms of the cause of the disease, the obstruction caused by inflammation, due to the swelling of the vascular wall and endothelium, is reversible, unlike the obstruction caused by arteriosclerosis, which involves the thickening of the venous wall and the narrowing of the lumen due to subendothelial and endothelial cell proliferation, and is irreversible. Therefore, the prognosis of inflammatory obstruction is better than that of sclerotic obstruction. In terms of the location of the obstruction, branch obstruction is better than hemilateral obstruction, and hemilateral obstruction is better than total trunk obstruction. In terms of the degree of obstruction, incomplete obstruction (the non-ischemic one called by Hayreh) is better than complete obstruction (ischemic). Of course, the estimates of the above prognoses are not absolute. For example, whether an effective collateral circulation can be formed early, whether timely and reasonable treatment can be received, etc., all directly affect the prognosis. The presence of edema in the macula, which does not subside in the short term, will inevitably seriously damage the central vision. Especially in the case of complete occlusion of the trunk, where the fluorescence angiography shows a large area of non-perfused area, not only is the rate of blindness high, but also the incidence rate of neovascular glaucoma is high, and the prognosis is extremely poor.