[Etiology and pathophysiology].
The disease is characterized by post-hepatic portal hypertension with or without inferior vena cava hypertension caused by obstructive lesions of the hepatic veins and/or the inferior vena cava of its opening. it was described by Budd and Chiari in 1845 and 1899 respectively, hence the name
Budd-Gahr’s syndrome. In Western countries, it is most often caused by a hypercoagulable state of blood flow leading to hepatic vein thrombosis, often without involvement of the inferior vena cava, or secondary to inferior vena cava hypertension due to significant extrahepatic compression of the inferior vena cava. In contrast, in Eastern countries, inferior vena cava lesions or developmental abnormalities are more common. There are three types of the disease, namely, limited stenosis or obstruction of the inferior vena cava septum (type I), diffuse stenosis or obstruction (type II), and obstruction of the hepatic veins (type III). Type I accounts for about 57%; type II accounts for about 38%; and about 5%. The age of onset of the disease varies depending on the cause of onset, but the onset of the disease is early due to congenital developmental abnormalities, the earliest being 2.5 years old. The earliest onset is 2.5 years old, but it occurs mostly between the ages of 20 and 40. The age of onset is later in cases of acquired causes. In cases of hepatic vein obstruction alone, portal hypertension is the main symptom, while in cases of combined inferior vena cava obstruction, both portal hypertension and inferior vena cava obstruction are present.
The symptoms of both portal hypertension and inferior vena cava obstruction syndrome are present. In late stage patients, due to severe ascites, repeated laparotomy for abdominal decompression to relieve symptoms, protein loss continuously, and finally death from severe malnutrition, gastrointestinal hemorrhage by rupture of esophageal varices, or liver and kidney failure.
【Clinical manifestations
(A) manifestation of portal hypertension
Patients mainly have indigestion, intractable ascites, hypersplenism and other manifestations. Vomiting blood and black stool may appear in the late stage.
(B) Inferior vena cava obstruction manifestation
The obstruction of inferior vena cava return may lead to swelling of both lower limbs, perineum and bottom-up superficial varicose veins of chest and abdominal wall and low back, which may cause palpitations, and even slight activity may cause symptoms of cardiac insufficiency such as panic and shortness of breath. Due to the obstruction of renal venous return, it can cause the manifestation of renal insufficiency, including reduced urine volume and generalized edema.
Diagnosis
(I) Symptoms and signs: Those who have manifestation of portal hypertension with thoracic and abdominal wall, especially back and lumbar and bilateral varicose veins of lower limbs should be highly suspected of Buga’s syndrome.
(II) Auxiliary examinations.
1. Liver function: early liver function is not obviously abnormal, with the development of the lesion, plasma albumin decreases, globulin increases, the ratio of white and ball is inverted, transaminases and bilirubin increase, prolonged prothrombin time.
2. Barium swallow x-ray of esophagus: advanced esophagus may have worm-like changes.
3. B-mode ultrasound: It is a simple, reliable and convenient non-invasive screening tool. The diagnostic accuracy rate is over 90%. Stenosis and occlusion of the inferior vena cava proximal to the hepatic vein can be seen. Slowed hepatic venous blood flow. B-mode ultrasound can also detect early Buga’s syndrome during health screening.
4. Inferior vena cava angiography: It is the best method to diagnose this disease. It can clearly show the site of the lesion, the degree, type and extent of obstruction. It has guiding significance for treatment.
5. Percutaneous hepatic venography by hepatic puncture: it can show whether there is obstruction of hepatic vein or not. In addition to the same significance of the above methods, in appropriate cases, it can be done as dilation and stenting treatment, and it can also help to predict the outcome of surgery and prognosis.
6. CT and MRI: It is also helpful to clarify the lesion, but not as accurate as the above methods.
【Treatment】.
Surgical treatment is the only effective way to treat Bu-ga syndrome. Different treatment plans should be selected according to different pathological types and the function of various organs of the body.
I. Treatment of limited inferior vena cava obstruction or stenosis
(A) Restricted inferior vena cava obstruction with patent hepatic vein
1. Firstly, balloon catheter dilation and necessary internal support frame placement are used
2. Finger dissection via the right atrium
3. Trans-right atrial dissection with transfemoral vein meeting dissection, dilation and endoprosthesis
4. Inferior vena cava-right atrial artificial vessel diversion
5. Radical correction
(B) Treatment of limited inferior vena cava obstruction with hepatic vein obstruction
Radical correction
Treatment of long obstruction or stenosis of inferior vena cava
1.Superior mesenteric vein-right atrium artificial vessel diversion
2.Splenic vein-right atrium artificial vessel diversion
3.portal vein-right atrium artificial vessel diversion
4.Superior mesenteric vein-internal jugular vein via retrosternal artificial vessel diversion
C. Treatment of early inferior vena cava passage and hepatic vein obstruction (type C)
1.Hepatic vein outflow tract angioplasty
2.Superior mesenteric vein – vena cava diversion
3.Spleen vein-renal vein diversion
4.Portal vein – vena cava diversion
IV. Other
For patients with liver failure, hepatic coma or secondary severe hepatic steatosis, liver transplantation is the only effective treatment route.
For advanced patients with abnormally debilitating systemic conditions who cannot tolerate surgery, non-surgical treatment is adopted. It includes symptomatic treatment, thrombolytic treatment with urokinase in the acute phase and herbal treatment.
[Postoperative complications
1. Cardiac insufficiency
It is a common postoperative complication of this disease. It is mainly due to the preoperative blood stasis in the lower part of the body, which significantly reduces the amount of return blood, shrinks the heart, reduces cardiac output, and can cause poor cardiac function symptoms such as panic and shortness of breath even with slight activity. After the hepatic vein and/or inferior vena cava obstruction is lifted, the amount of blood returned to the heart suddenly increases, adding to the burden of the already malfunctioning heart, and heart failure occurs. In order to prevent heart failure, after the inferior vena cava obstruction is lifted, cardiac stimulation and diuretic treatment should be given immediately, including Cetiran 0.4mg,
Tachyphylaxis 40~100mg intravenously will help to reduce the occurrence of heart failure.
2. Ascites or celiac disease
Before surgery, due to obstruction of inferior vena cava reflux, there is no outlet for hepatic venous blood, while plasma flows into the hepatic lymphatic gap, resulting in overloaded hepatic lymphatic fluid leaking out through the hepatic fibrous capsule into the abdominal cavity, which becomes stubborn and difficult to subside ascites, and in a few patients, celiac disease is formed due to rupture of high-pressure dilated lymphatic vessels. Intraoperative damage to the dilated lymphatic vessels is more likely to result in celiac abdomen. If there is no injury to the celiac pond, the existing ascites or celiac disease may gradually subside on its own after surgery. If there is celiac pond injury, it can be closed gradually after non-surgical treatment through intravenous nutrition.
3. Hemothorax
It is directly related to open-heart surgery, mostly due to incomplete intraoperative hemostasis, anastomotic fistula, improper placement of closed chest drainage or postoperative anticoagulation therapy. A small amount of hemothorax can be closely observed, if the bleeding is large, the chest should be opened in time to stop the bleeding and closed drainage of the chest cavity. If it is caused by anticoagulation therapy, attention should be paid to the relevant monitoring indicators and timely adjustment of anticoagulant drugs and doses.
4. Hepatic encephalopathy
The incidence of this complication is directly related to the size of the anastomosis, as described in section 7 of this chapter. The rate of hepatic encephalopathy after intestinal-atrial diversion is not high in cases of Baubgar’s syndrome, where liver function is often better than in cases of hepatic sclerosis.