Budd-Chiari syndrome is a type of posterior portal hypertension characterized by inferior vena cava hypertension caused by obstructive lesions of the hepatic vein and the inferior vena cava above its opening. In the acute phase, the patient has fever, pain in the upper right abdomen, a large amount of abdominal effusion, jaundice, hepatomegaly, tenderness in the liver area, and oliguria. The disease is more common in young males, the ratio of male to female is about (1.2-2):1, and the age is 2.5-75 years old, and it is more common in 20-40 years old.
Mainly include: ①natural macrovascular malformation; ②high coagulation and high viscosity state; ③toxin; ④nonthrombotic obstruction in the cavity; ⑤exogenous compression; ⑥vascular wall disease; ⑦diaphragmatic factor; ⑧abdominal trauma, etc.
2 clinical manifestations
In the acute stage of simple hepatic vein thrombosis, patients have fever, right upper abdominal pain, a large amount of abdominal effusion, jaundice, hepatomegaly, tenderness in the liver area, and oliguria. Death can occur from circulatory failure, liver failure, or gastrointestinal bleeding within a few days or weeks. The manifestations of simple hepatic vein thrombosis in the non-acute phase are portal hypertension, hepatosplenomegaly, refractory ascites, and bleeding from esophageal varices. Simple inferior vena cava obstruction, there are superficial varicose veins on the chest and abdomen wall and back (venous blood flow from bottom to top) and varicose veins, edema, pigmentation and ulcers of the lower limbs. Due to obstruction of the hepatic vein and inferior vena cava, the blood return to the heart is reduced, and the patient may have shortness of breath. It depends on the number of blood vessels involved, the degree of involvement, and the nature and state of the obstructive lesion. Can be divided into acute type, subacute type and chronic type.
1. Acute type
Most of the hepatic veins are completely obstructed, and the obstructive lesions are mostly thrombosis. It usually starts at the outlet of the hepatic vein, and thrombus can rapidly multiply to the inferior vena cava. Sudden onset, sudden upper abdominal pain, nausea, vomiting, abdominal distension, diarrhea, similar to fulminant hepatitis, liver progressive enlargement, tenderness, often accompanied by jaundice, splenomegaly, rapid growth of abdominal effusion, and may also Pleural effusion. Fulminant patients may quickly develop hepatic encephalopathy, progressively worsen jaundice, oliguria or anuria, and may be complicated by diffuse intravascular coagulation (DIC), multiple organ failure (MOSF), spontaneous bacterial peritonitis, (SBF) Most of them can die quickly due to circulatory failure (shock), liver failure or gastrointestinal bleeding within a few days or weeks.
Most of the hepatic veins and inferior vena cava are involved simultaneously or successively. Intractable peritoneal effusion, hepatomegaly and lower extremity edema are mostly present at the same time, and then there are superficial varicose veins on the abdominal wall, lower back and chest. The blood flow direction is upward, which is Buja Syndrome distinguishes important features from other diseases. Jaundice and hepatosplenomegaly are only seen in one-third of patients, and most of them are mild or moderate. In many cases, the formation of abdominal effusion is rapid and long-lasting, the abdominal pressure rises, and the diaphragm rises. In severe cases, abdominal compartment syndrome (ACS) may occur, causing systemic physiological disorders. Oliguria and anuria appear. Chest volume and lung compliance decrease, cardiac output decreases, pulmonary vascular resistance increases, and hypoxemia and acidosis occur.
The course of the disease can be as long as several years. It is more common in patients with diaphragm obstruction. The condition is milder, but there are more eye-catching signs, such as thick and winding swollen veins on the chest and abdomen walls. Pigmentation is seen in the boot area. The appearance of chronic ulcers. Although there may be varying degrees of abdominal effusion, most tend to be relatively stable. There may still be distended jugular vein, varicocele, huge inguinal hernia, umbilical hernia, hemorrhoids, etc.
Advanced patients may have a typical "Spider-Man" posture due to malnutrition, protein loss, increased abdominal effusion, and weight loss.
1. Laboratory examination
Hematology examination, acute phase cases may have multiple blood signs such as increased hematocrit and hemoglobin, routine blood examination may have increased white blood cell count, but it is not characteristic. In advanced chronic cases, if there is upper gastrointestinal bleeding, splenomegaly, or hypersplenism, there may be anemia or decreased platelet and white blood cell counts. Liver function tests, acute type patients may have increased serum bilirubin, ALT, AST, ALP increase, prothrombin time prolonged and serum albumin decreased, chronic type cases, liver function tests mostly have no significant changes. Examination of ascites, if it is not accompanied by spontaneous bacterial peritonitis, the protein concentration is often less than 30g/L, and the number of cells does not show an increase. Immunological examination showed no obvious characteristic changes in serum IgA, lgM, IgG, IgE and C3.
2. B ultrasound examination
Abdominal ultrasound can make a preliminary correct diagnosis for most cases, and its coincidence rate can reach more than 95%. The location and length of hepatic vein and inferior vena cava obstruction can be detected at the top of the diaphragm and at the second hepatic portal to determine whether it is a diaphragm type. In acute Budd-Chiari syndrome, liver enlargement and ascites are mostly prominent manifestations. Doppler ultrasound has high diagnostic value. Therefore, abdominal ultrasound exploration is the preferred, valuable, non-invasive examination for Budd-Chiari syndrome.
3. Hepatic vein, inferior vena cava, portal vein and arteriography
Angiography is the most valuable method to establish the diagnosis of B-CS. Commonly used angiography include the following: ①Inferior vena cava and pressure measurement; ②Percutaneous hepatic venography (PTHV); ③Percutaneous splenic puncture of the portal vein Angiography (PTSP); ④Arteriography.
4. CT scan
In the acute phase of Budd-Chiari syndrome, CT plain scan showed diffuse low-density swelling of the liver accompanied by large amounts of ascites. The specific manifestation of CT scan is a highly degraded filling defect (60～70Hu) in the posterior segment of the inferior vena cava and the main hepatic vein. Enhanced scanning is of great significance to the diagnosis of Budd-Chiari syndrome.
5. Magnetic resonance (MRI)
In Budd-Chiari syndrome, MRI can show low-intensity signals of the liver parenchyma, suggesting liver congestion and increased free water in the tissue. MRI can clearly show the open state of the hepatic vein and inferior vena cava, and even the fresh thrombus in the blood vessel can be combined with the mechanism Differentiation of thrombus or tumor thrombus; MRI can also show the spider web-like changes in the medial hepatic branch circulation, as well as the lateral hepatic branch circulation.
6. Liver nuclide scan
The venous blood in the tail lobe of the liver flows back directly from the short hepatic vein to the inferior vena cava. When the simple hepatic vein is blocked, the short hepatic vein is unobstructed, and the radioactivity in the liver area is sparse and the caudal lobe is dense in the isotope scan. Radionuclide scanning is not specific for the diagnosis of Budd-Chiari syndrome. Only some cases have relatively increased radioactive absorption in the caudate lobe, which has important reference value in the identification of cavernous hepatic hemangioma.
Gastroscopy is not very helpful in the diagnosis of Budd-Chiari syndrome. However, in chronic cases, especially those who have had gastrointestinal bleeding, the cause and location of the bleeding can be further understood; for suspicious or difficult to identify cases, a biopsy can be taken directly to make the diagnosis more clear. Laparoscopic biopsy has the advantages of being safer and more reliable.
8. Liver biopsy
In the acute stage of simple hepatic vein thrombosis, hepatic central veins, sinusoids and lymphatic vessels are dilated, sinusoidal blood stagnation, and diffuse hepatic hemorrhage. Blood cells leak from the sinusoids into the perisinusal space and mix with the cells of the liver plate. There is necrosis of liver cells around the central vein. After a while, the liver plate cells are replaced by red blood cells. In the late stage, the necrotic liver cells in the central area of the liver lobules are replaced by fibrous tissues, resulting in liver cirrhosis, the rest of the liver cells are regenerated, and the liver veins and sinusoids are expanded.
Acute Budd-Chiari syndrome is mostly characterized by right upper abdominal pain, a large amount of ascites, and liver enlargement; chronic cases are mostly characterized by liver enlargement, formation of portal-body collateral circulation and persistent ascites. Non-invasive real-time ultrasound, Doppler ultrasound and CT scan can prompt the clinical diagnosis of Budd-Chiari syndrome in more than 95% of cases.
1. Interventional surgical treatment
Interventional surgery is the first choice for Budd-Chiari syndrome, with less trauma and good results. Inferior vena cava or hepatic vein with thrombosis, thrombolysis can be treated by intubation first. After the thrombus is completely dissolved, balloon dilatation can be used to expand the narrowed vessel. Patients with poor balloon expansion can be treated with hepatic vein and or inferior vena cava stents.
2. Medical treatment
Medical treatment includes low-salt diet, diuresis, nutritional support, and reinfusion of autologous ascites. For patients in the acute phase with simple thrombosis within 1 week of onset, anticoagulants can be used, but most cases are not diagnosed until a few weeks or months after thrombosis. For most cases, conservative treatment can win the time for the formation of collateral circulation, but the patient still needs surgery in the end. Patients with Budd-Chiari syndrome, especially those in advanced stages, often have refractory ascites and severe malnutrition. As a supportive treatment before surgery, medical treatment can improve the patient's overall condition, reduce surgical mortality, and facilitate postoperative recovery.
3. Surgical treatment
(1) Diaphragm tear operation: The right atrial diaphragm tear operation: the method is to enter the thoracic cavity through the right anterior fourth cost extrathoracic incision or through the sternum incision to enter the thoracic cavity.
(2) Inferior vena cava-right atrial shunt.
(3) Superior mesenteric vein-right atrial shunt.
(4) Radical surgery Although radical surgery directly removes the primary lesion, there is still the possibility of recurrence in cases accompanied by inflammation of the inferior vena cava