Budd Chiari Syndrome life expectancy, Treatment, Symptoms & Causes: Badd-Chiari Syndrome disease is called an obstruction (obstruction, thrombosis) of the veins supplying the liver in the area of their fusion with the inferior vena cava. Such vascular occlusion is caused by primary or secondary causes: congenital anomalies, acquired thrombosis and vascular inflammation (endophlebitis) and leads to disruption of the normal outflow of blood from the liver. Such a blockage of the hepatic veins can be acute, subacute or chronic and amounts to 13-61% of all cases of endophlebitis with thrombosis. It leads to disruption of the functioning of the liver and damage to its cells (hepatocytes). Further, the disease causes a malfunction of other systems and leads to disability or death of the patient.
Budd-Chiari syndrome is more common in women with hematological diseases, after 40-50 years, and the frequency of its development is 1: 1000 thousand people. Within 10 years, about 55% of patients with this pathology survive, and death occurs from severe renal failure.
Content in This Article
- budd chiari syndrome causes
- budd chiari syndrome Symptoms
- Emergency conditions for Budd-Chiari syndrome
- budd chiari syndrome diagnosis
- Budd Chiari Syndrome Treatment
budd chiari syndrome causes
The most common Badd-Chiari syndrome is the result of congenital abnormalities of the development of liver vessels or hereditary blood pathologies. The development of this disease contributes to:
- congenital or acquired hematological disorders: coagulopathy (deficiency of protein S, C or prothrombin II, paroxysmal hemoglobinuria, polycythemia, antiphospholipid syndrome, etc.), myeloproliferative diseases, sickle cell anemia, long-term use of hormonal contraceptive anemia, long-term use of hormonal contraceptive systems, households, for example, for households, people, for people, for example, for cases of hormonal contraceptives, etc. systemic vasculitis;
- neoplasms: liver carcinomas, inferior vena cava leiomyoma, myxoma of the heart, adrenal tumors, nephroblastoma;
- mechanical causes: narrowing of the hepatic vein, membranous occlusion of the inferior vena cava, hypoplasia of the suprahepatic veins, obstruction of the inferior vena cava after injury or surgery;
- migrating thrombophlebitis ;
- chronic infections: syphilis, amebiasis, aspergillosis, filariasis, tuberculosis, echinococcosis, peritonitis, pericarditis or abscesses;
- cirrhosis of the liver of various etiologies.
According to statistics, in 20-30% of patients, Budd-Chiari syndrome is idiopathic, in 18% of cases it is provoked by blood coagulation abnormalities, and in 9% – by malignant neoplasms.
For etiological reasons for the development of Budd-Chiari syndrome is classified as follows:
- Idiopathic: unexplained etiology.
- Congenital: develops as a result of fusion or stenosis of the inferior vena cava.
- Post-traumatic: caused by injuries, exposure to ionizing radiation, phlebitis, taking immunostimulants.
- Thrombotic: caused by hematological disorders.
- Caused by oncological diseases: triggered by hepatocellular carcinomas, adrenal neoplasms, leiomyosarcomas, etc.
- Developing against the background of various types of cirrhosis.
All of the above diseases and conditions can lead to the manifestation of Budd-Chiari syndrome, since they are the etiological causes of thrombosis, stenosis or obliteration of blood vessels. Asymptomatically, only obstruction of one of the hepatic veins can occur, but with the development of blockage in the lumens of two venous vessels, there is a violation of venous circulation and an increase in intravenous pressure. This leads to the development of hepatomegaly, which, in turn, provokes overdistension of the hepatic capsule and causes painful sensations in the right hypochondrium.
Subsequently, in the majority of patients, venous blood outflow begins to occur through the paravertebral veins, unpaired vein and intercostal vessels, but such drainage is only partially effective, and venous congestion leads to central hypertrophy and peripheral atrophy of the liver. Also, almost 50% of patients with this diagnosis may develop a pathological increase in the size of the caudate hepatic lobe, which causes obstruction of the lumen of the inferior vena cava. Over time, such changes lead to the formation of fulminant liver failure, occurring against the background of hepatocyte necrosis, vascular paresis, encephalopathy, impaired coagulation or cirrhosis of the liver.
According to the localization of the site of thrombosis and endophlebitis, Budd-Chiari syndrome can be of three types:
- I – inferior vena cava with secondary obliteration of the hepatic vein;
- II – obliteration of large venous vessels of the liver;
- III – obliteration of small venous vessels of the liver.
The nature of the clinical manifestation of Budd-Chiari syndrome depends on the location of the blockage of the hepatic vessels, the nature of the course of the disease (acute, fulminant, subacute or chronic) and the presence of certain accompanying pathologies in the patient.
The most common chronic development of Budd-Chiari disease is, when the syndrome is not accompanied by any visible signs for a long time and can only be detected by palpation or instrumental examination (ultrasound, radiography, CT, etc.) of the liver. Over time, the patient appear:
- pain in the liver;
- severe hepatomegaly;
- compaction of the liver tissue.
In some cases, the patient has an expansion of the superficial venous vessels along the anterior wall of the abdomen and chest. In the later stages thrombosis of the splash vessels, pronounced portal hypertension and liver failure develops.
In acute or subacute development of the syndrome, which is observed in 10-15% of cases, the patient is diagnosed:
- the appearance of acute and rapidly increasing pain in the right hypochondrium;
- moderate jaundice (some patients may be absent);
- nausea or vomiting;
- pathological enlargement of the liver;
- varicose expansion and swelling of the superficial venous vessels in the areas of the anterior abdominal and chest wall;
- swelling of the legs .
In case of acute course, the Budd-Chiari disease progresses rapidly and after a few days, 90% of patients develop abdominal protrusion and fluid accumulation in the abdominal cavity (ascites), which is sometimes combined with hydrothorax and is not corrected by diuretic therapy . At the latest stages, 10–20% of patients show signs of hepatic encephalopathy and episodes of venous bleeding from the dilated vessels of the stomach or esophagus.
The fulminant form of Budd-Chiari syndrome is quite rare. It is characterized by the rapid development of ascites, a rapid increase in the size of the liver, severe jaundice and rapidly progressive liver failure.
- In the absence of adequate and timely surgical and therapeutic treatment, patients with Budd-Chiari syndrome die from such complications caused by portal hypertension:
- bleeding varicose veins of the esophagus or stomach;
- hypersplenism (a significant increase in the spleen in size and the destruction of its red blood cells, platelets, leukocytes and other blood cells).
A larger percentage of patients with Budd-Chiari’s disease, accompanied by a complete blockage of the veins, die within 3 years of renal failure.
Emergency conditions for Budd-Chiari syndrome
In some cases, this syndrome is complicated by internal bleeding and urinary disorders. In order to provide timely assistance, patients with this pathology should remember that, when certain conditions appear, they need to urgently call the ambulance team. These signs may be:
- rapid progression of symptoms;
- vomit color of coffee grounds;
- tarry stools;
- a sharp decrease in the amount of urine.
budd chiari syndrome diagnosis
A doctor may suspect the development of Budd-Chiari syndrome when a patient has liver enlargement, signs of ascites, cirrhosis of the liver, liver failure in combination with abnormalities detected in laboratory biochemical blood tests. For this purpose, functional liver tests are performed with risk factors for thrombosis: deficiency of antithrombin-III, protein C, S, resistance to activated protein C.
To clarify the diagnosis, the patient is recommended to undergo a series of instrumental studies:
- Doppler ultrasound of abdominal vessels and liver;
- CT scan;
If the results of all studies are questionable, the patient is prescribed a liver biopsy, which allows to detect signs of hepatocyte atrophy, thrombosis of the terminal venules of the liver and venous congestion.
Budd Chiari Syndrome Treatment
Treatment of Budd-Chiari syndrome can be performed by a surgeon or a gastroenterologist in a hospital setting. In the absence of signs of liver failure, surgical treatment is indicated for patients whose purpose is to impose anastomoses (joints) between the hepatic blood vessels. For this, the following types of operations can be carried out:
- balloon dilatation.
In case of membranous fusion or stenosis of the inferior vena cava, recovery of venous blood flow is performed in the case of trans-atrial membranotomy, dilation, bypass vein shunting with the atrium or replacement of the stenosis sites with prostheses. In the most severe cases, with total liver tissue damage by cirrhosis and other irreversible functional disorders in its work, a liver transplant may be recommended to the patient.
To prepare the patient for surgery, to restore blood flow in the liver in the acute period and during rehabilitation after surgical treatment, patients are prescribed symptomatic drug therapy. Against the background of its use, the doctor must prescribe the patient control tests for the level of electrolytes in the blood, prothrombin time and makes adjustments in the scheme of therapy and dosing of certain drugs. The complex of drug therapy may include drugs such pharmacological groups:
- drugs that contribute to the normalization of metabolism in hepatocytes: Essentiale forte, Phosphogliv, Heptral, Hafitol, etc
- diuretics: Lasix, Furosemide, Veroshtilakton, Veroshpiron;
- anticoagulants and thrombolytics: Fragmin, Clexane, Streptokinase, Urokinase, Aktilize;
Drug therapy cannot completely replace surgical treatment, since it provides only a short-lasting effect, and the two-year survival of non-operated patients with Budd-Chiari syndrome is no more than 80-85%.