Buga syndrome (BLldd a Chiari syndroYne) is a post-hepatic portal hypertension characterized by obstructive lesions of the hepatic veins and the inferior vena cava above its opening, often accompanied by inferior vena cava hypertension, due to various causes. The main factors include: (1) congenital macrovascular malformation; (2) hypercoagulable and hyperviscous state; (3) toxins; (4) intraluminal non-thrombotic obstruction; (5) exogenous compression; (6) vascular wall lesions; (7) diaphragmatic factors; (8) abdominal trauma, etc. Etiology of Hou Summit, Department of Gastroesophageal Reflux Disease, Aviation General Hospital of China Medical University: George Budd (1845) and Hans Chiari (1899) reported the clinical and pathological features of cases of hepatic vein thrombosis due to hepatic phlebitis, respectively. The cluster of symptoms caused by hepatic venous obstruction was later referred to as Budd-Chiari syndrome.
Hepatic vein obstruction or inferior vena cava obstruction is mostly due to (i) hepatic vein thrombosis due to hypercoagulable state of blood (caused by oral contraceptives, erythrocytosis); (ii) foreign compression of veins by tumors; (iii) invasion of hepatic veins by cancer (e.g., hepatocellular carcinoma) or inferior vena cava (e.g., renal carcinoma, adrenal carcinoma); and (iv) congenital abnormal development of inferior vena cava (septum formation, stenosis, atresia). In western countries such as Britain and the United States, cases of hepatic vein thrombosis are predominant and often do not involve the inferior vena cava. In contrast, in Eastern countries, such as China, India, and Japan, 1/3 of cases are due to malformation of the septum of the hepatic segment of the inferior vena cava.
The septum of the hepatic segment of the inferior vena cava is usually very thin, 1 to 2 mm thick, located 3 to 4 cm from the opening of the inferior vena cava in the right atrium. 41% of the septum is above the opening of the hepatic vein, 40% of the septum runs obliquely from the lower left to the upper right, between the left and middle hepatic veins and the opening of the right hepatic vein, separating the veins, and 19% is below the opening of the hepatic vein. In the case of inferior vena cava septum, stenosis or atretic malformation, the hepatic vein may have no opening, the opening may be blocked by a thrombus, or the opening may be patent. Even if the hepatic vein opening is patent, hepatic venous blood return may be impeded by obstruction of the proximal inferior vena cava.
Clinical manifestations.
In the acute phase of simple hepatic vein thrombosis, patients have fever, right upper abdominal pain, rapid onset of massive ascites, jaundice, hepatomegaly, tenderness in the liver area, and oliguria. Death can occur within days or weeks due to circulatory collapse (shock), liver failure, or gastrointestinal bleeding. The non-acute phase of simple hepatic vein thrombosis is characterized by portal hypertension, hepatosplenomegaly, intractable ascites, and bleeding from ruptured esophageal varices.
In simple inferior vena cava obstruction, there are superficial varices of the thoracic and abdominal walls and back (venous blood flow from bottom to top) and varicose veins, swelling, hyperpigmentation and ulcers of the lower extremities.
Patients may have shortness of breath due to reduced blood return to the heart due to obstruction of the hepatic and inferior vena cava.
It varies according to the number of vessels involved, the degree of involvement and the nature and state of the obstructing lesion. It can be divided into acute, subacute and chronic types.
Acute type: Mostly caused by complete obstruction of hepatic veins, the obstructive lesions are mostly thrombosis. Most of them start at the exit of the hepatic vein, and the thrombus can rapidly multiply to the inferior vena cava. The onset of the disease is rapid, with sudden onset of epigastric distention and pain, accompanied by nausea, vomiting, abdominal distention, diarrhea, resembling fulminant hepatitis, progressive enlargement of the liver, pressure pain, mostly accompanied by jaundice, splenomegaly, rapid growth of ascites, and at the same time, pleural effusion. In fulminant cases, hepatic encephalopathy may rapidly develop, with progressive worsening of jaundice, oliguria or anuria, and may be complicated by diffuse intravascular coagulation (DIC), multiorgan failure (MOSF), spontaneous bacterial peritonitis, (SBF), etc. Most can die rapidly within days or weeks due to circulatory collapse (shock), hepatic failure, or gastrointestinal bleeding. Ascites, hepatomegaly and the rapid appearance of MOSF are the prominent manifestations of the disease.
Subacute type: Mostly, the hepatic veins and inferior vena cava are involved simultaneously or successively. Intractable ascites, hepatomegaly and lower limb edema are often present simultaneously, followed by superficial varices of the abdominal wall, low back and chest, whose blood flow is directed upward, an important feature that distinguishes Budd-Chiari syndrome from other diseases. Jaundice and hepatosplenomegaly are seen in only 1/3 of patients and are mostly mild or moderate. In many cases, ascites formation is rapid and persistent, with elevated abdominal pressure and diaphragm elevation, and in severe cases, abdominal compartment syndrome (ACS) may develop, causing systemic physiological disturbances. If the abdominal pressure rises to 25 cmH2O and 50 cmH2O, oliguria and anuria will occur, respectively. Thoracic volume and pulmonary compliance decrease, cardiac output decreases, pulmonary vascular resistance increases, and hypoxemia and acidosis occur.
Chronic type: The disease can last for more than several years, mostly in patients with septal obstruction, and is mostly mild, but there are mostly striking signs, such as thick, sinuous, angry veins in the chest and abdominal wall, pigmentation seen in the foot and shoe area, and in some cases, chronic ulcers. Although ascites may be present to varying degrees, most tend to be relatively stable. There may also be angry jugular veins, varicose veins of the spermatic cord, giant inguinal hernias, umbilical hernias, and hemorrhoids. Esophageal varices are often unnoticed by the patient and are only confirmed by endoscopy or radiography when sudden vomiting of blood, black stool or enlarged spleen is detected. Hepatomegaly in this type of patient is less pronounced than in subacute cases and is mostly hemihepatomegaly, but the degree of sclerosis increases, and the spleen is mostly moderate, rarely the giant spleen seen in intrahepatic portal hypertension.
In advanced patients, the typical “spider” physique may appear due to malnutrition, protein loss, increased ascites, and wasting.
The disease is more common in young men, with a male to female ratio of about (1.2 to 2):1, aged 2.5 to 75 years, with 20 to 40 years being the most common.
Treatment.
1.Interventional surgery treatment: interventional surgery is preferred for Buga syndrome, which is less traumatic and has good effect. If the inferior vena cava or hepatic vein is combined with thrombosis, it can be treated with intubation thrombolysis first, and balloon dilation is feasible after complete dissolution of the thrombus to widen the stenotic section. If the effect of balloon dilation is poor, the hepatic vein and or inferior vena cava stenting treatment is feasible.
2.Internal medicine treatment.
Internal treatment includes low-salt diet, diuretic, nutritional support, autologous ascites transfusion and so on. Patients in the acute phase with simple thrombosis within 1 week of onset can be treated with anticoagulants, but most cases are not diagnosed until weeks or months after thrombosis. In most cases, conservative treatment may buy time for collateral circulation to develop, but the patient will eventually require surgery. Patients with Bard-Gialli syndrome, especially in advanced stages, often have intractable ascites and severe malnutrition. As a supportive therapy before surgery, medical treatment can improve the patient’s general condition, reduce surgical mortality, and facilitate the patient’s postoperative recovery.
3.Surgical treatment.
(1) septal laceration: Trans-right atrial septal laceration: the method is to enter the thoracic cavity through the right anterior 4th rib external thoracotomy or through the sternotomy, and cut the pericardium longitudinally in front of the right phrenic nerve.
(2) Inferior vena cava-right atrial shunt.
(1) Anterior hepatic approach: enter the abdomen through a median abdominal incision or a right rectus abdominis incision, and the inferior vena cava can be exposed by the following methods: A. make a Kocher incision to free and turn up the duodenum to the left to reveal the inferior vena cava; B. turn up the transverse colon and its mesentery, push the small intestine to the left, open the retroperitoneum below the level of the duodenum and to the right of the superior mesenteric vein, and dissect in the direction of the abdominal aorta. The inferior vena cava should be exposed for at least 4 cm. a transthoracic incision can be made through a sternotomy or a right anterior external thoracic incision, and a 14- or 16-mm diameter artificial vessel is used to perform a terminal anastomosis to the inferior vena cava at one end and a right auricular anastomosis at the other end. The artificial vessel usually passes behind the transverse colon, in front of the stomach and liver, and then into the thoracic cavity.
The posterior hepatic route: The patient is placed in the left lateral position and the chest is entered from the right 7th rib. The pericardium is opened to reveal the thoracic segment of the inferior vena cava. The diaphragm is incised and the inferior vena cava is dissected down to its dilated or relatively normal portion. The artificial vessel is anastomosed to the distal dilated portion of the narrowed inferior vena cava at one end and to the inferior vena cava or right auricle of the diaphragm at the other end. Compared with the anterior hepatic route, the posterior hepatic route requires shorter artificial vessels and less chance of thrombosis, but the procedure is more difficult, prone to bleeding, and has a relatively higher incidence of postoperative celiac pleural effusion. Inferior vena cava-right auricular shunt restores blood return to the inferior vena cava and is suitable for cases where the inferior vena cava has a long obstructive lesion and the hepatic veins are relatively unobstructed.
(3) Superior mesenteric vein-right atrium shunt: The abdomen is entered through a median incision in the upper abdomen, and the superior mesenteric vein is sought at the root of the transverse colic mesentery, to the right of the flexor ligament. The thoracic incision can be made through a sternotomy or a right anterior external thoracotomy. A 14- or 16-mm-diameter artificial vessel is used to perform a terminal anastomosis to the superior mesenteric vein at one end and a right auricular anastomosis at the other end.
(4) Radical surgery: For septal type cases with high vena cava obstruction, the lesion can be removed by dissecting out the thoracic segment and part of the ventral segment of the inferior vena cava and controlling the two ends of the lesion. If the obstructive lesion is extensive or there is a large amount of thrombosis distally, the inferior vena cava of the hepatic segment can be dissected longitudinally under extracorporeal circulation to remove the septum and thrombus and other lesions, explore the hepatic vein and restore its patency, and repair the inferior vena cava with Gore-Tex or Dacron patches. Although radical surgery directly removes the primary lesion, there is still a possibility of recurrence in cases with concomitant inferior vena cava inflammation.