From this article you will learn: what pathology is called the Budd Chiari syndrome, how dangerous it is, than the syndrome differs from the Badda-Chiari disease. Causes and treatment.
The causes of the disease
Badd-Chiari syndrome is a stasis of blood in the liver caused by a violation of outflow from an organ that develops against a background of various non-vascular pathologies and diseases( peritonitis, pericarditis).The result of blood flow disorders is liver damage and an increase in blood pressure in the portal vein( a large blood vessel that brings venous blood to the liver from unpaired organs).
Anatomical structure of the epigastric region of the abdominal cavity. Click on photo to enlarge Conditionally distinguish two forms of the disease with the same symptoms and manifestations:
- Disease, or primary pathology( cause - inflammation of the internal walls of blood vessels that supply blood to the liver).
- Badda-Chiari syndrome, or secondary pathology( occurs against a background of non-vascular diseases).
Syndrome is a fairly rare disease, according to statistics it is diagnosed in 1 out of 100 thousand people, in women from 40 to 50 years - several times more often than men.
In pathology for various reasons( peritonitis, vascular thrombosis), there is a narrowing of the adjacent and internal vessels of the liver. Slowing down the blood flow causes rebirth and necrosis of hepatocytes( organ cells) with the formation of scars and knots. The tissue thickens and increases in size, strongly squeezing the vessels.
As a result, blood flow disorders, ischemia( oxygen starvation) and atrophy( exhaustion) of the peripheral lobes of the liver, stagnation of blood in the central part, which quickly leads to an increase in pressure( portal hypertension) in the incoming vessels, develop.
The disease progresses rapidly to life-threatening complications. On a background of stagnant phenomena, renal and hepatic insufficiency( impaired function), cirrhosis( organ degeneration, replacement with a nonfunctional tissue) develops, a large amount of fluid accumulates in the chest cavity or abdomen( more than 500 ml, hydrothorax andascites).In 20% of the syndrome for a few days leads to a fatal outcome due to hepatic coma and internal bleeding.
Complete recovery of the Badd-Chiari syndrome is impossible. Drug therapy and surgical methods in the complex prolong the life of a patient with a chronic form( up to 10 years), but are ineffective in acute forms of the disease.
The medicamentous appointment at a pathology is made by the doctor-gastroenterologist, surgical correction is carried out by surgeons.
Causes of the disease
The immediate cause of the syndrome is the narrowing or complete invasion of the walls of the vessels, providing the outflow of venous blood from the organ. Provision of their appearance may:
- mechanical abdominal trauma( stroke, bruise);
- peritonitis( an acute inflammatory process in the abdominal cavity);
- pericarditis( inflammation of the outer connective tissue of the heart);
- liver disease( focal damage and cirrhosis);
- swelling of the abdominal cavity;
- congenital defects of the inferior vena cava( large vessel providing blood outflow from the liver);
- thrombosis( clotting of the blood vessel by a thrombus);
- polycythemia( a disease in which the amount of red blood cells - erythrocytes - increases in blood);
- paroxysmal nocturnal hemoglobinuria( a kind of anemia, nocturnal urine excretion with an admixture of blood);
- migrating visceral thrombophlebitis( simultaneous appearance of several large blood clots in different vessels);
- infection( streptococcal, tubercular bacillus);
- medicines( hormonal contraceptives).
In 25-30% of cases, the causes of the disease can not be established, such a pathology is called idiopathic.
Stages of liver damage. Click on the picture to enlarge Symptoms
The disease can develop in acute, subacute and chronic form:
- For the acute form is characterized by a rapid increase in the signs of the disease - acute pain, nausea, vomiting, the appearance of pronounced edema of the lower extremities, ascites and hydrothorax( fluid in the thoracic and abdominal cavity).The patient is difficult to move and perform any physical action. Usually the acute form of the Budda-Chiari syndrome and illness ends with coma and death in a few days.
- Subacute form proceeds with severe symptoms( enlargement of the liver and spleen, development of ascites), develops not so rapidly as acute, and is more often transformed into a chronic one. The patient's disability is limited.
- Chronic form in the early stages is asymptomatic( in 75-80%), it is possible the appearance of weakness and fatigue against the background of increasing insufficiency of blood supply to the body, not related to physical activity. Progressing, insufficiency provokes the appearance of severe symptoms, which are accompanied by pain, a feeling of raspiraniya in the left hypochondrium, vomiting, signs of cirrhosis of the liver. The work ability of the patient during this period is limited and may not recover( due to frequent complications of the process).
| Form of | Syndrome |
|---|---|
| Acute | Significant liver enlargement in size( hepatomegaly) Acute, unbearable pain in the epigastric region( under the lower edge of the sternum, under the spoon) or right upper quadrant Nausea, vomiting Weakness, fatigue Slight icterus of the eye scleraand skin Swelling of superficial veins on chest and abdomen Pronounced puffiness of legs Hydrotorax Hemorrhagic ascites( presence of blood in accumulated fluid caused bymorning bleeding) |
| Subacute | Significant increase in liver Increased spleen( splenomegaly) Discomfort and tenderness in the right upper quadrant( in the liver region) Weakness, increased fatigue Disturbance in physicochemical properties of blood( increase in viscosity, clotting) Ascite |
| Chronic inperiod of increase in insufficiency | Weakness, fatigue Strengthening pains on the right, in the hypochondrium Hepatomegaly, splenomegaly Vomiting Appearance clearlypronounced venous pattern on abdomen and chest Dyspepsia phenomena( indigestion) Internal bleeding Puffiness of the ankles Coagulation disorder Ascites |
Normal and enlarged spleen. Click on the image to enlarge A common occurrence for the Buddha-Chiari syndrome is the rapid development of serious complications:
- of renal and hepatic insufficiency;
- hydrothorax and hemorrhagic ascites;
- internal bleeding;
- cirrhosis;
- coma and death.
The average life span of a patient without treatment is from several months to 3 years.
Diagnosis
Budda-Chiari syndrome is pre-diagnosed with a significant increase in the liver and spleen, against a background of coagulation disorders and developing ascites.
Clinical laboratory diagnostics are assigned to confirm the diagnosis:
- an expanded blood test with counting of the number of platelets and leukocytes( in 70% increase in the number);
- definition of blood coagulation factors( change in coagulogram indices, increase in prothrombin time);
- biochemical blood tests( increase in alkaline phosphatase, activity of hepatic transaminases, a slight increase in bilirubin and a decrease in albumin).
Assay for hepatic assays. Click on the image to enlarge As instrumental methods use:
- Computer tomography and ultrasound( determine the degree and location of liver sites with impaired blood flow).
- Angiography of the blood vessels of the liver( an informative way to determine the localization of the causes of blood supply disorders( thrombosis, stenosis, occlusion) and the degree of vascular damage).
- Lower Cavalgia( examination of the inferior vena cava for obstruction of blood flow).
- Biopsy, or in the lifetime of taking biological material( allows to determine the degree of degeneration, necrosis or atrophy of cells).
Sometimes a hepatic vein can be catheterized( to determine the localization of thrombosis).
CT of abdominal cavity of patient with Badda-Chiari Syndrome Treatment methods
It is completely impossible to cure the disease, in most cases, drug therapy is ineffective, slightly and for a short time facilitates the patient's condition( prolongs life up to 2 years).
Conservative treatment
Treatment objectives:
- to eliminate the obstruction to blood flow and normalize blood supply to the organ;
- to reduce pressure in the portal vein;
- prevent the development of complications in the Buddha-Chiari syndrome.
| Drugs | Drugs |
|---|---|
| Anticoagulants( quarantil, kleksan) | Blood thinning, reducing the ability of platelets and erythrocytes to aggregate( clot), prevent the formation of thrombi |
| Thrombolytics( streptokinase, urokinase) | Solutions are used for the destruction( destruction, dissolution) of a thrombus |
| Preparations improving metabolism in liver cells( lipoic acid, Essential forte) | Improve metabolism in hepatocytes, increasing their resistance in the case of insufficient blood supply |
| Glucocorticoids( prednisolone) | Remove inflammation of liver tissue and vascular walls, improve metabolism, have anti-allergic and analgesic action |
| Diuretics( furosemide, spironolactone) | Assign to remove excess fluid in case of marked lower limb edema, |
Without surgical correction, conservativetreatment leads to the development of fatal complications and lethal outcome in 85-90%( for a period of 2 to 5 years).
Surgical treatment
Surgical methods allow to improve the prognosis and prolong the life of the patient( up to 10 years), contraindication to intervention - severe liver failure.
| Surgical methods | surgical methods | |
|---|---|---|
| anastomosing | anastomosis application Artificial hollow prostheses create a communication between damaged blood flow restoration vessels | |
| Shunting | Biological or artificial prostheses simulating vascular segments create a path for bypassing damaged veins for blood flow | |
| Prosthetic upper vena cava | Replace damaged areaupper vena cava by artificial or biological implant | |
| Balloon dilatation and stenting of the lowerhollow vein | Catheter with a balloon on the end widens the stenosis site, installs a metal structure( stent) into the vessel wall that prevents re-narrowing | |
| Membranotomy | Dissect septa or septa from connective tissue, enlarging the lumen of the | |
| vessel Liver transplant | Damaged organ is replaced by a biological transplanthealthy donor liver) |
Shunting in Badda-Chiari syndrome The most effective method is a liver transplant, a successfully performed surgeryevaet terms of the patient's life up to 10 years or more( a record life expectancy with Budd-Chiari syndrome - 22 years).
Forecast
Forecasts for the disease are unfavorable, a complete cure is impossible. In some cases( 15-20%), the pathology develops very quickly and in a few days leads to the appearance of fatal complications( acute hepatic insufficiency, internal bleeding, coma) and death.
In chronic course of the disease( 80-85%), manifestations of liver failure increase slowly( this takes from 1 to 6 months), eventually developing chronic impairments( insufficiency and cirrhosis), which lead to a lethal outcome of 85-90% infor 2-5 years.
Medication is ineffective, prolongs life expectancy by an average of 2 years. Surgical intervention( shunting, liver transplantation) can significantly improve the patient's condition. The maximum period of life after the operation is 10 years, the record period is 22 years.
