Bulga syndrome (BSC) (Budd-Chiari Syndrome BSC) is a posthepatic type of portal hypertension caused by partial or complete obstruction of the hepatic vein and/or the inferior vena cava segment above its opening, with impaired hepatic excretion as the main manifestation of the syndrome, also known as hepatic vein obstruction syndrome. This syndrome was reported by Budd and Chiari in 1846 and 1889, respectively. The main clinical features are abdominal pain, hepatosplenomegaly, ascites and lower extremity edema. The syndrome is relatively rare and can be seen at any age, with a prevalence between 20 and 40 years of age. [Etiology] There are a wide variety of causes of BCS, which can be categorized into two main groups, primary and secondary, depending on the etiology. In some cases, it is difficult to find the cause of the disease, and some people call it idiopathic. The causes of this syndrome include thrombosis of the hepatic vein and inferior vena cava, compression by lesions in neighboring organs, and lesions of the hepatic vein and inferior vena cava themselves, such as congenital developmental anomalies and occlusive phlebitis. Common etiology:1 congenital factors, mainly refers to the inferior vena cava septum.2. Hypercoagulable and hyperviscous state.3. Toxins, including endogenous and exogenous have a certain relationship.4 non-thrombotic obstruction in the vena cava.5 exogenous compression.6. vascular wall lesions, 7, transverse septal factors.8. abdominal trauma.9 Others. (I) Hepatic segmental occlusive lesions of the inferior vena cava may have narrowing, near complete occlusion, or a membranous central hole, with either a thin or thick membrane, or a long segmental occlusion, and thus also named membranous obstruction of the inferior vena cava (MOVC), narrowing, but from the histopathology of the membrane it is thought to be due to thrombus mechanization. Thrombosis along the superior mesenteric vein has been demonstrated, and membranes can also form in the portal vein as a sequela of thrombosis. It is also found in children, but the age of coincidence is 30 years, and the doctrine of congenital vascular malformation has been proposed. Inferior vena cava occlusive disease is located above or below the opening of the hepatic vein, and the opening may or may not be occluded or only one opening is occluded, the membrane also has a thickness of varying thickness, and the attached wall thrombus mechanization makes the inferior vena cava constricted and narrowed. The local anatomy of congenital vascular malformations should be uniform, but in fact it is not. Histologically, it is difficult to distinguish between normal fibrosis and thrombus mechanization, but in the latter case endothelial and smooth muscle fibers are lined, and the mechanized thrombus often shows capillary hyperplasia and ferrous hemosiderin deposits. It has been reported that the thrombus at the opening of the hepatic vein may form a membrane, and the size of the thrombus mechanization determines the thickness of the membrane. Symptoms of hepatic segmental obstruction of the inferior vena cava are due to incomplete obstruction at the onset of the disease, in the process of mechanization due to fibrinolysis and fibrosis can make the thrombus shrink, if the thrombus is small, the membrane formed by the thin membrane which has a hole. Clinical studies using ultrasound or inferior vena cava angiography can see the transition from thrombosis to complete occlusion or significant narrowing of the membrane. Diseases that predispose to thrombosis include systemic lupus erythematosus, oral contraceptives (foreign), and primary myelodysplasia, which can have inferior vena cava obstruction and hepatic vein thrombosis. Yet why do thromboses tend to occur in the hepatic segment of the inferior vena cava? Thrombosis of the inferior vena cava can occur in leukemia vasculitis, as well as in other diseases. It has been suggested that the following factors make the hepatic segment of the inferior vena cava particularly susceptible to thrombosis: (1) respiratory movement of the diaphragm can damage the lining of the inferior vena cava; (2) coughing can exacerbate mechanical damage; (3) vortexing of blood flow at a right angle between the hepatic vein and the inferior vena cava occurs at this segment; (4) post-traumatic hypercoagulable state in two cases, which contributed to the membranous obstruction in the inferior vena cava that had not been involved. (ii) The etiology of hepatic venous outflow tract obstruction has two types: secondary and primary. Secondary obstruction is mostly due to tumor, liver cyst, trauma, large nodule, and amebic liver abscess compression, and its treatment is different from that of idiopathic obstruction. Hepatic venous thrombosis, which used to be considered the classic Buga syndrome, has an occult myeloproliferative disorder, which can be detected and diagnosed by bone marrow culture and erythrocyte lineage colony formation. Defects in hematopoietic lineage stem cells can also produce a prothrombotic state. Infected patients with positive blood cultures, cannulation for inferior vena cava angiography sometimes provokes the occurrence of chills, fever, cellular thrombophlebitis, and most of these patients have a low economic status and poor nutrition. Pathological changes] The hepatic vein and inferior vena cava can be seen to have thrombosis, and there is often occlusion in the acute stage, and the hepatic vein is inflammatory in the chronic stage, with thickening of the vein wall, thrombus mechanization and re-communication of the lumen. The liver and spleen are enlarged, and microscopy shows dilatation of the central veins of the hepatic lobules, congestion, hemorrhage and dilatation of the hepatic sinusoids, atrophy and necrosis of the central hepatocytes, and fibrosis of the central area of the lobules in the late stage, with regeneration of liver tissue and cirrhosis. Pathophysiology] Obstruction of hepatic venous return and increased pressure lead to dilatation and stasis of the central hepatic vein and hepatic venous sinusoids. This leads to portal hypertension manifestations such as intractable ascites, hepatosplenomegaly esophagogastric fundus varices. As the gastrointestinal stasis and swelling lead to abdominal distension, maldigestion and malabsorption, anemia, and hypoproteinemia. If early restoration of hepatic venous return can be reversed, if long-term unresolved eventually lead to hepatic cirrhosis, a small number of can form hepatocellular carcinoma. Meanwhile, due to the obstruction of inferior vena cava, it can cause swelling of perineum of both lower limbs and varicose veins of chest, waist and back, and the manifestation of varicose veins is obvious and extensive. The obstruction of renal venous return can lead to renal insufficiency. Due to the stagnation of blood in the lower half of the body, the volume of return blood is reduced, the heart shrinks, and the patient often has symptoms such as heart traces and shortness of breath after minor activities. [Clinical manifestations] The syndrome is mostly chronic, and its clinical manifestations mainly include two aspects: 1. The manifestation of hepatic venous return obstruction, often with progressive hepatosplenomegaly, esophageal and abdominal wall varices, ascites and hepatic pain, abdominal pain, etc. Jaundice is rare. In the acute stage of venous thrombosis, there may be different degrees of fever and pain in the liver area, and even death by shock. In the late stage, portal hypertension and cirrhosis may appear. 2.Disorders of inferior vena cava reflux, such as lower limb edema, cyanosis, superficial varicose veins, hyperpigmentation or ulceration, and heavy numbness in lower limbs. The direction of blood flow in the varicose veins is consistent and upward. Auxiliary examination] 1, the degree of liver function damage is mild, there may be delayed excretion of indocyanine green, serum alkaline phosphatase, transaminase elevation, albumin decrease. 2.B-type ultrasound and CT examination can show the site, scope, degree of stenosis and occlusion of the hepatic vein and inferior vena cava, as well as the morphological changes of the liver. 3.Inferior vena cava venography and selective hepatic venography can accurately determine the location, scope, nature, collateral circulation and the presence or absence of external compression of the obstructing lesion. 4. Liver biopsy shows congestion and sinusoidal dilatation around the central vein of the lobule, and cirrhosis in the advanced stage. 【Diagnosis】 The main diagnosis is based on hepatosplenomegaly, ascites and the characteristic manifestation of extensive abdominal wall varices. There are three important features of this syndrome: 1. The age is relatively young. 2. The varicose veins are located in the lateral chest, abdominal wall, and below the umbilicus, and the direction of blood flow is also from bottom to top. 3. Although it looks like cirrhosis, there is no obvious damage to liver function. Hepatic and inferior vena cava angiography is the main method to confirm the diagnosis. Differential diagnosis] It should be differentiated from cryptogenic cirrhosis, post hepatitis cirrhosis, portal vein thrombosis and constrictive pericarditis. Therapeutic measures] Treatment should be based on etiologic and symptomatic treatment. For thrombosis, anticoagulant treatment can be tried. For simple venous stenosis and incomplete occlusion, metal elastic stents can be placed. For those with complete occlusion of veins, complicated etiology or those who are not suitable for intravenous catheterization, surgical treatment can be adopted, which is aimed at relieving the lower limbs and abdominal cavity from blood stasis and portal hypertension, with surgical methods such as portal inferior vena cava shunt, fragmentation of membranes, septal resection, splenopulmonary fixation, and occluded vein incision and repair surgery. With the development of interventional radiology technology, interventional therapy has become the preferred method of BCS treatment. Many scholars have carried out a lot of research in this area, and have put forward their own views on BCS in different situations, and have not yet formed a unified standard of operation for treatment, but the specific treatment methods are more or less the same, which are summarized as follows. (I) Simple hepatic vein obstruction type BCS: This type of BCS includes two kinds of cases: (1) limited hepatic vein obstruction, hepatic veins, including the paracolic vein orifice of the membranous or short-segmental obstruction, the distal hepatic vein is obviously dilated, and the hepatic collateral circulation is increased; therefore, hepatic vein obstruction can be solved by connecting the dilated distal hepatic vein and inferior vena cava through the perforation, and the specific method of operation is transjugular or percutaneous transhepatic hepatic vein opening. The specific operation method is transjugular vein or percutaneous transhepatic hepatic vein opening. (ii) Widespread obstruction of hepatic veins, ultrasound and angiography show that the hepatic veins are generally thin or basically no display, such BCS can only use transjugular intrahepatic portacaval shunt (TIPSS). Hepatic vein opening via the transjugular route: ① Firstly, the septal puncture needle or Rups2100 hepatic puncture device was delivered to the level of the hepatic vein orifice via the jugular vein. Then adjust the needle tip direction according to the anatomical position and use a soft-tipped straight guidewire to explore the obstructed hepatic vein orifice. If the guidewire is able to pass through a highly narrowed or occluded hepatic vein, the puncture system can be delivered directly into the hepatic vein along the guidewire. If the guidewire cannot enter the hepatic vein, hepatic vein puncture can be performed directly on the basis of accurate localization. When the puncture needle enters the hepatic vein, the metal needle is withdrawn first, and the outer casing is retained for contrast observation. (iii) If the occluded hepatic vein cannot be penetrated, direct intrahepatic puncture can be performed from the posterior segment of the IVC to a depth of 3-5 cm, and hepatic venography can be performed after the branches of the hepatic vein have been detected, and then occlusion puncture can be performed after the location and morphology of the major occluded veins in the liver have been shown. 2.Hepatic vein opening by combined percutaneous transhepatic and transjugular venous route: this method is suitable for those who fail to pass through the transjugular venous route, i.e., the percutaneous transhepatic operation is carried out on the basis of established right jugular venous puncture channel. Under fluoroscopic or ultrasound guidance, a percutaneous hepatic needle is used to puncture the right or left middle hepatic vein from the 8th or 9th intercostal space in the right mid-axillary line or under the xiphoid process. After successful hepatic venography, a 5F dilator or straight catheter was inserted along the guidewire, and the hepatic vein was passed through the guidewire to perform collateral hepatic vein perforation. After successful puncture, the guidewire was first sent into the superior vena cava via the IVC and right atrium, and then removed via the internal jugular vein. Thus, a percutaneous channel is established through the liver, hepatic vein, IVC, right atrium, superior vena cava to the internal jugular vein, and then hepatic venoplasty is performed via the jugular vein route. ② If the collateral hepatic vein access is unsuccessful, the guidewire in the hepatic vein can also be used as a marker, and then hepatic vein access can be performed via the jugular vein under the guidance of bidirectional fluoroscopy. 3.Percutaneous transluminal balloon dilatation angioplasty (PTA): after successful opening of membranous and segmental stenosis or occlusive lesions of the hepatic vein, balloon catheters of different diameters are used according to the diameter of the local vein, and treatment is carried out to dilate the channel of the perforated channel with PTA. When the effect of channel expansion is not obvious, an endovascular stent is built into the channel, which solves the obstruction and also prevents the occurrence of restenosis. 4, TIPSS: specific operation method ……… (ii) IVC lesions: in domestic BCS, such lesions are more common. This type of BCS includes the following cases: posthepatic segmental IVC membranous stenosis or occlusion, which may be accompanied by intracavitary thrombosis in the distal lumen of the lesion; this type of PTA treatment should be preferred. Posthepatic IVC segmental stenosis or occlusion, lesion length greater than 1cm; PTA and endovascular stenting are preferred. 1, posthepatic IVC segmental stenosis or occlusion of interventional therapy: PTA is the first choice of posthepatic IVC membranous stenosis or occlusion of the treatment method, most cases can obtain satisfactory medium and long term effects by using this technology only. ① first in the occlusion of the opening operation based on the delivery of ultra-stiff guidewire, and over the lesion blood vessels; ② on the stenosis of IVC stenosis lesions in the internal diameter of 5mm or less, the first choice of 8 to 12mm diameter balloon for initial expansion, and then 18 to 24mm balloon expansion; on the internal passage of IVC stenosis lesions greater than 5mm, can be directly with a large balloon expansion; ③ on the lesions of the old and stiff, a single large balloon catheter can not make its If the lesion is old and stiff, and a single large balloon catheter cannot dilate it adequately, double balloon method or multiple balloon method can be used for simultaneous dilation; ④ After the balloon catheter is dilated satisfactorily, then venography and venous pressure measurement will be carried out, and the decision of whether or not to use ES treatment will be made according to the situation. ⑤ For IVC membranous lesion with intraluminal thrombosis, local thrombolysis should be performed by injecting urokinase through catheter first, and then PTA and stenting should be performed after the thrombus or fresh thrombus on the surface is dissolved, so as to make use of the tension of the vascular stent to press the chunky thrombus against the vascular wall, so as to avoid dislodging of chunky embolus after lumen is completely opened. 2.Post hepatic IVC segmental stenosis or occlusion: PTA and endovascular stenting are preferred, the opening of the occluded segment of the vessel is the key to the success of interventional therapy, and endovascular stenting is the main method of treating such lesions. (1) Firstly, the pair-end marking, bi-directional localization, and contrast tracking method of IVC opening. Operation method: ① Occluded segment end-to-end angiography: firstly, a pigtail catheter is fed from the femoral vein and jugular vein to the proximal and distal ends of the occluded segment of the IVC respectively. Then a Y-shaped connecting tube is used to perform bidirectional or unidirectional contralateral angiography of the occluded segment in order to clearly show the location, scope and morphology of the occluded segment of the IVC. ② Replace the 10-12F femoral vein catheter sheath, and through this sheath, send the outer casing of the J-cannula needle along the guidewire to the distal end of the IVC occluded segment, withdraw the guidewire, insert the metal needle into the outer casing and fix it. (iii) The catheter at the proximal end of the IVC occluded segment was retained as a localization marker for the penetration procedure. ④Under positive and lateral two-way fluoroscopic monitoring, adjust the position and angle of the tip of the trocar needle with reference to the contrast image, and after confirming that there is no error, slowly push the trocar needle into the occluded lesion and inject a little contrast agent into every 0.5-1.0 cm of the needle, observe the position of the tip of the needle and pay attention to whether there are any signs of extravascular puncture. ⑤ When the tip of the trocar reaches the curved part, the direction and angle of the tip are adjusted again to keep it on the same axis with the proximal end of the proximal positioning mark in both the positive and lateral positions. Then continue to push the puncture needle in the direction of the right atrium until the occluded segment is penetrated, and imaging confirms that the outer casing has entered the right atrium, and then pull out the metal needle and replace the ultrarigid guidewire. (6) After delivering the ultrarigid guidewire into the superior vena cava, a 10 to 12F dilator was replaced to pre-dilate the occluded segment. so that an 8 to 10 mm balloon catheter can be delivered for initial opening. (2) After IVC opening, PTA of the occluded segment is performed to prepare for endovascular stent placement. (3) Endovascular stent placement: On the basis of PTA, the stent delivery device is first delivered into the IVC along the guidewire and over the lesion site. Then the inner core is pulled out to retain the guidewire. The stent is then compressed along the guidewire and sent into the delivery device through the introduction tube, and then pushed into the lesion lumen under fluoroscopic guidance. ② After accurate positioning, slowly withdraw the stent delivery device while fixing the pusher. (iii) If the stent unfolds poorly after placement, balloon dilatation can be performed again to obtain satisfactory efficacy.