布加氏综合征（Budd-chiari syndrome）

布加氏综合征（Budd-chiari syndrome） 布加氏综合征(Budd-chiari syndrome) 【布加综合征】 (blldd一chiari syndroyne) 布加综合征是由各种原因所致肝静脉和其开口以上段下腔静脉阻塞性病变、 引起的常伴有下腔静脉高压为特点的一种肝后门脉高压征. 其发病因素主要包括: ?先天性大血管畸形; ?高凝和高粘状态; ?毒素; ?腔内非血栓性阻塞; ?外源性压迫; ?血管壁病变; ?横膈因素; ?腹部创伤等. 布加氏综合征又称巴德 - 吉亚里综合症. 是由于肝静脉或其开口以上的下腔静脉受邻近病变侵犯、压迫或腔内血栓形成等原因引起的 部分或完全性阻塞, 下腔静脉血液回流因之障碍而出现 以门静脉高压或门静脉和下腔静脉高压微主要特征的一系列临床征候群.这是一种血管源性疾病. 布加氏征是一种罕见疑难病, 因其无明显特异性症状, 常易被误诊误治. 因其临床症状及其转归酷似肝炎后肝硬化, 故有人称该病是肝炎的 "姐妹" 病. 肝炎是由病毒感染侵及肝脏, 造成肝细胞损害, 需要依靠药物治疗; 布加氏综合征则是肝脏和下腔静脉回流受阻、肝内瘀血肿胀, 造成肝细胞损害. 需要依靠介入或手术解除静脉血回流受阻才能得到有效的治疗. 布加氏综合征具有中青年发病多, 男性发病多, 肝脏和下腔静脉同时阻塞的特点. 患病后, 由于肝脏和下腔静脉压力均升高, 可比正常高出2倍. 故诊断要点为: "一黑" - 下肢皮肤色素沉着. "二大" - 肝、脾瘀血性肿大. "三曲张" - 胸腹壁静脉、精索静脉、大隐静脉曲张. "二多" - 中青年发病多、男性发病多. 肝脏超声检查是无创伤且能最早、最快发现本病的方法, 故称为 "前哨检查". 下腔静脉造影既能明确诊断, 又能分清类型, 且能为治疗提供良好依据, 故称其为 "黄金检查标准". 【症状】 临床表现取决于阻塞的部位、程度以及侧支循环的状况. 轻度阻塞可无明确的症状或为原发病变的症状所掩盖; 一旦完全阻塞, 症状和体征可很典型.下腔静脉下段的阻塞所引起的征状. 主要是下腔静脉高压状态: ?下肢静脉郁滞; 两下肢以至阴囊明显肿胀, 每于行走、运动后加剧, 平卧休息后减轻. 下肢浅静脉曲张, 皮肤出现营养性改变, 如皮肤光薄、脱毛、搔痒、湿疹、色素沉着, 甚至形成经久不愈的溃疡. 尤以两下肢足靴区最为明显. ?胸腹壁静脉曲张, 大多是竖直长链状, 直径可达10m m以上, 有时也可盘曲成团, 似静脉瘤样改变. Varicose veins are usually located in the anterior wall of the chest and abdomen, and also in the chest, abdomen, side wall and back. The direction of blood flow is upward. If the lesion involves renal vein or above plane, it leads to renal vein hypertension, renal blood flow decrease and renal dysfunction. It is characterized by low back pain, swelling of the kidneys, and proteinuria and hematuria. If you enter the chronic phase, it is due to long-term proteinuria, swelling of the body, increased blood cholesterol and so on, The so-called renal degeneration syndrome can be formed. The lesion involved hepatic veins or above planes, You can have the inferior vena cava pressure, portal hypertension (including hepatosplenomegaly, ascites, esophageal varices and upper gastrointestinal bleeding) and Cardiac reserve function insufficiency (including dynamic, palpitations, shortness of breath) the clinical manifestations of the three groups. Acute hepatic venous obstruction can result from sudden ascites and hepatic coma. Most of the patients with inferior vena cava obstruction syndrome had better liver function, and those with white globulin inversion or abnormal liver function accounted for about 1/3, Because of this disease, the pathological changes of liver cells are secondary and mild. If the inferior vena cava obstruction is caused by the tumor, there is a tumor and pain in the tumor itself, Invasion or metastasis of hepatomegaly, jaundice, gastrointestinal dysfunction and hemoptysis, chest pain, etc.. [type] Budd Chiari syndrome in clinic can be divided into the following types: Asymptomatic: Although there were hepatic vein thrombosis, there was no obvious circulatory disturbance. It was found incidentally only in hepatic venography and B ultrasound examination. Acute type: acute onset, epigastric pain, nausea, vomiting, jaundice, ascites and hepatomegaly, death can be in the short term. Type: chronic onset and slow development, the gradual emergence of abdominal distention, liver discomfort, pain and hepatomegaly, After many years lead to cirrhosis, splenomegaly, ascites, occurrence of gastrointestinal hemorrhage. [pathogeny] Hepatic vein obstruction or inferior vena cava obstruction is more due to (1) the formation of hepatic vein thrombosis caused by high blood coagulation (oral contraceptives and polycythemia); External compression of vein by tumor; Cancer invasion of hepatic veins (such as liver cancer) or inferior vena cava (such as renal carcinoma and adrenal cancer); Congenital dysplasia of the inferior vena cava (septum formation, stenosis, atresia). Depending on how many blood vessels are involved, the extent of involvement, and the nature and condition of obstruction. They can be divided into acute type, subacute type and chronic type. Acute type: mostly caused by complete occlusion of hepatic veins, and the obstruction is mostly thrombosis. Most of them begin at the outlet of the hepatic vein, and the thrombus can rapidly propagate into the inferior vena cava. Abrupt onset, sudden abdominal pain, nausea, vomiting, abdominal distension, diarrhea, mimicking fulminant hepatitis, The liver was enlarged, tenderness, accompanied by jaundice, splenomegaly, ascites, rapid growth, and pleural effusion. Fulminant hepatic encephalopathy can occur rapidly, progressive jaundice, oliguria or oliguria, complicated by disseminated intravascular coagulation (DIC), Multiple organ failure (MOSF), spontaneous bacterial peritonitis, and (SBF), Most can die rapidly within days or weeks due to circulatory failure (shock), liver failure, or gastrointestinal bleeding. Ascites, hepatomegaly and rapid emergence of MOSF, is a prominent manifestation of this disease. Subacute type: mostly hepatic veins and inferior vena cava are affected simultaneously or successively. Intractable ascites, hepatomegaly and edema of the lower extremities are common at the same time, The abdominal wall, the back of the waist and the superficial varicose veins of the chest, with the direction of blood flow upward, are the important features of Budd-Chiari syndrome which are different from other diseases. Jaundice and hepatosplenomegaly were only found in 1/3 patients, and mostly mild or moderate. In many cases, ascites develops rapidly and steadily, abdominal pressure rises, and the diaphragm is raised. Severe abdominal compartment syndrome may cause systemic physical disturbances. If the abdominal pressure rises to 25cmH2O and 50cmH2O, oliguria and oliguria occur respectively. Chest volume and lung compliance decreased, cardiac output decreased, pulmonary vascular resistance increased, hypoxemia and acidosis occurred. Chronic type: the course of disease can be more than a few years, mostly in patients with membranous obstruction, the condition is more mild, but there are many signs of attention, For example, a large, meandering, angry vein of the chest wall, pigmentation in the foot boot area, and some chronic ulcers. Although ascites can be found in different degrees, most of them tend to be relatively stable. The fair has a distended neck veins, varicocele, giant inguinal hernia, umbilical hernia, hemorrhoids etc.. Esophageal varices often can cause patients to pay attention, in the sudden hematemesis and melena or splenomegaly and medical treatment, endoscopic or X-ray angiography was confirmed. Most of the patients with liver swelling are less obvious than those with subacute, and most of them are semi large liver, but the degree of sclerosis has increased, It is moderate, rarely appears as intrahepatic portal hypertension of megalosplenia. Advanced patients, due to malnutrition, protein loss, ascites increase, weight loss, can appear typical "Spider Man" posture. The disease is more common in young men, the ratio of men and women is about (1.2 ~ 2): 1, the age of 2.5 to 75 years old, 20~40 years old is more common. [inspection] (I) laboratory examination Hematology examination, the acute phase of the case may have hematocrit and increased hemoglobin and other signs of blood manifestations, blood routine examination may be increased in white blood cells, but not characteristic. (two) B ultrasound and abdominal B ultrasound can make the most accurate diagnosis for most cases, and the coincidence rate is over 95%. (three) hepatic vein, inferior vena cava, portal vein and arteriography Angiography is the most valuable method of establishing B-CS diagnosis, Commonly used radiography has following kinds: (1) contrast and manometry of inferior vena cava; (2) percutaneous transhepatic venography (PTHV); (3) percutaneous splenic puncture and portal venography (PTSP); (4) arteriography. (four) CT scan, in the acute phase of Budd-Chiari syndrome, the CT scan showed diffuse, low density and accompanied by massive ascites. The specificity of CT scan is that there is a high filling defect (60 ~ 70Hu) in the inferior vena cava, the posterior hepatic vein and the main hepatic vein. Enhanced scan is important for the diagnosis of Budd-Chiari syndrome. After injection of contrast agents, 30s showed a patchy enhancement (central speckled area) near the hepatic hilum, and the area around the liver was not markedly enhanced, And extensive portal vein development, suggesting portal vein blood leaving the liver. After injection of contrast agents, 60s showed a low density band shadow with an enhancement of margin, or hepatic vein and inferior vena cava filling defect This phenomenon highly suggests intraluminal thrombus formation, and the marginal enhancement is due to the development of the vessel wall nutrient vessels. (five) MRI can display the low intensity signal of liver parenchyma during Budd-Chiari (MRI) imaging, suggesting that the liver congestion and the free water in the tissue increase, MRI can clearly show the open condition of the hepatic vein and inferior vena cava, and even differentiate the fresh thrombus from the blood vessel with the mechanical thrombus or tumor thrombus; MRI can also show the changes in the circulation of the medial branches of the liver and show the extrahepatic collateral circulation as well, Therefore, MRI may be considered as one of the non-invasive methods for the examination of Budd-Chiari syndrome. (six) the venous blood of liver tail lobe was scanned directly by hepatic radionuclide, and it was directly returned to inferior vena cava by short hepatic vein. The hepatic short vein is unobstructed when simple hepatic vein obstruction is found. The radioisotope scanning shows that the liver area is sparsely radioactive, while the caudate lobe is densely radioactive. Radionuclide scanning is not specific for the diagnosis of Budd-Chiari's syndrome, but only in some cases is the relative increase in radioactive absorption in the caudate lobe, It is of important reference value in the differential diagnosis of cavernous hemangioma of the liver. (seven) endoscopic gastroscopy is of little help in the diagnosis of Budd-Chiari syndrome. But in chronic cases, especially those with gastrointestinal bleeding, further understanding of the cause and location of bleeding is possible; In cases of questionable or difficult identification, biopsies taken under direct vision are more definitive. Laparoscopic biopsy has a safer and more reliable advantage. (eight) liver biopsy, simple hepatic vein thrombosis, acute stage, hepatic lobule, central vein, hepatic sinusoid and lymphatic Guan Kuozhang, Hepatic sinusoid, liver diffuse hemorrhage. The blood cells leak from the hepatic sinus into the sinus space and are mixed with the cells of the hepatic plate. Necrosis of hepatocytes around the central vein. Liver cells were replaced by red blood cells for some time. Advanced hepatic centrilobular necrosis of the liver cells were replaced by fibrous tissue, cirrhosis, The liver cells were regenerated and the hepatic veins and hepatic sinusoids dilated. [diagnosis] Acute Budd-Chiari syndrome is characterized by right upper abdominal pain, massive ascites and hepatomegaly; Chronic cases are characterized by enlargement of the liver, collateral circulation of the portal body and persistent ascites. Noninvasive real-time ultrasound and Doppler ultrasonography and CT scan could indicate the clinical diagnosis of Budd-Chiari syndrome in more than 95% of the cases, Careful analysis of medical history and systematic physical examination should not be neglected, However, the diagnosis of Budd-Chiari syndrome depends on the inferior vena cava, hepatic venography and liver biopsy. [treatment] 1. interventional surgery: Choice of interventional therapy of Budd Chiari syndrome, small trauma, good effect. The inferior vena cava or the hepatic vein combined with thrombus can be treated with intubation and thrombolytic therapy. When the thrombus is completely dissolved, balloon dilation can be used to expand the stenosis. If the effect of balloon dilatation is poor, it is feasible to treat hepatic vein or inferior vena cava stent implantation. 2. internal medicine treatment Medical treatment includes low salt diet, diuresis, nutritional support and reinfusion of autologous ascites. For patients with acute thrombosis within 1 weeks of the onset of thrombosis, anticoagulant therapy is available, but most cases are diagnosed only weeks or months after thrombosis. In most cases, conservative treatment may allow for the formation of collateral circulation, but the patient still needs surgery at the end. Budd Chiari syndrome patients, especially in patients with advanced, often intractable ascites, severe malnutrition. As a supportive treatment before surgery, medical treatment can improve the general condition of patients, reduce operative mortality, and facilitate postoperative rehabilitation. 3. surgical treatment (1) septal tear: right atrial septal tear: the approach is through the right fourth rib thoracotomy incision or through the sternum incision into the chest, In the right phrenic nerve anterior longitudinal incision of the pericardium. (2) right inferior vena cava shunt: (3) right shunt of superior mesenteric vein to right atrium: The above abdominal median incision into the abdomen, looking for the superior mesenteric vein in the transverse mesocolon, Qu ligament on the right side of the root. Sternal incision or right anterolateral thoracotomy incision can be used for breast incision. One end of the artificial vessel with a diameter of 14 or 16mm was anastomosed to the superior mesenteric vein at one end, The other end coincides with the right atrial appendage. (4) radical surgery: For the diaphragm type with higher vena cava obstruction, the chest can be inserted into the chest from the right seventh rib, Both ends of control of the disease dissection of thoracic and abdominal part of inferior vena cava after longitudinal incision, excision of inferior vena cava. If the obstruction is extensive or the distal part has massive thrombus formation, may under the extracorporeal circulation, the longitudinal cutting liver section inferior vena, will remove the diaphragm, the thrombus and so on the pathological changes, The hepatic vein was explored and its patency was restored. The inferior vena cava was repaired with Gore-Tex or Dacron patches. Although radical surgery removes the primary lesion directly, there is still a possibility of recurrence in cases with inflammation of the inferior vena cava. Acute hepatic veno occlusive diseases, such as those caused by thrombosis, should be treated with anticoagulation as soon as possible. Membranous obstruction of the inferior vena cava in the lower segment of the liver can be considered as a bypass of the inferior right atrium, and the patient with incomplete obstruction may be operated or intervened, As with balloon angioplasty, balloon angioplasty and stenting. Surgical treatment, according to the condition for devascularization and splenectomy, splenorenal shunt, to reduce portal vein pressure, cure hypersplenism. To sum up, traditional surgery requires surgery on the large vessels, not only trauma, slow recovery, but also complex operation, great risk So in recent years, interventional therapy has become the preferred treatment of Budd Chiari syndrome. Inferior vena cava obstruction caused by thrombosis of the lower limb or deep pelvic vein, In acute phase, the affected limb can be raised, and the thrombolytic, anticoagulant and anti coagulation drugs are used, such as urokinase, streptokinase, heparin, and coumarin derivatives, Low molecular dextran, pan health etc.. In order to remove ascites, low salt diet can be added and diuretic drugs are used. If pulmonary embolism symptoms occur, the inferior vena cava ligation or inferior vena cava filter can be taken into consideration to prevent re embolization. For patients with chronic stage, there is no obvious improvement in the condition of active medical treatment. Surgical treatment can be considered to restore the inferior vena cava blood flow. (I) indication of operation Surgical indications for inferior vena cava obstruction should be strictly controlled. 1. patients with chronic thrombosis of inferior vena cava are treated with active medical treatment without obvious improvement. 2. obstruction of inferior vena cava septum. 3. malignant tumors cause, and may remove primary lesions, preservation or reconstruction of the inferior vena cava. (two) operation contraindication. 1. hepatic failure patients. 2. malignant tumors cannot be removed or removed. 3., the body condition is poor, can not tolerate surgery. For minor symptoms and longer course of disease, the body condition is better, the operation should be carefully considered. Especially, the inferior vena cava right atrial appendage bypass grafting is very traumatic and can cause hepatic and renal failure; Part of the chest wall by measuring vein damage; coagulation mechanism difference can cause mediastinal hematoma; Postoperative blood pressure surge can also lead to acute heart failure and so on. Therefore need to carefully weigh the pros and cons, and then decides the operation problems.