Budd-Chiari Syndrome is a disease characterized by impaired blood flow and venous congestion in the liver, due to obstruction of the veins of the organ. The disease can be primary or secondary. It is quite rare, it can have a subacute, acute and chronic course. The leading symptoms of the pathology are intense pain in the right hypochondrium, enlargement of the liver in size, vomiting and yellowing of the skin. The acute form is extremely dangerous to human life, as it can lead to coma and death.
The syndrome develops due to occlusion of the veins adjacent to the liver and are responsible for its normal blood supply. The liver is an important organ for humans, which performs a huge number of functions, ensuring the normal functioning of the body as a whole. In this regard, any disruptions in its supply of blood instantly affect all life support systems, and cause general intoxication of the body.
The syndrome was named after the authors who gave the most complete description of this pathological condition. These are the doctor Budd (England) and the pathologist Chiari (Austria).
Gastroenterology considers Budd-Chiari syndrome to be a rare condition that affects one in 100,000 people. This syndrome is most often diagnosed in women aged 30 to 50 years. In pediatric practice, the disease is rare, the average age of patients is 35 years.
- Causes of Budd-Chiari Syndrome
- Symptoms of Budd-Chiari Syndrome
- Diagnosing Budd-Chiari Syndrome
- Treatment for Budd-Chiari Syndrome
Causes of Budd-Chiari Syndrome
The causes of Budd-Chiari syndrome are varied.
Statistics indicate the following numbers:
- Hematological disorders lead to the development of the disease in 18% of cases;
- Malignant neoplasms contribute to venous stasis in the liver in 9% of cases;
- The etiology of the development of the syndrome in 30% of cases (idiopathic disease) remains unclear.
The main cause of the pathology is considered to be congenital disorders of the development of blood vessels of the liver and its structural units, as well as occlusion and obliteration of the veins of the liver.
In other cases, the following factors are capable of provoking the development of pathology:
- Injuries to the peritoneum and abdomen, as well as surgical interventions leading to stenosis of the hepatic veins.
- Diseases of the liver, cirrhosis is especially dangerous in this regard.
- Malignant neoplasms.
- Hemodynamic disorders.
- Vein thrombosis.
- Taking certain medications.
- Infections, including: syphilis, amebiasis, tuberculosis, etc.
- Pregnancy and the childbirth process.
All these factors lead to the fact that the normal permeability of the veins is disrupted, congestion is formed, which over time destroy the structural components of the liver. In addition, tissue necrosis of the organ occurs against the background of increased intrahepatic pressure. The liver tries to receive blood from small arteries, but if large veins have suffered, then other venous branches are unable to cope with the load imposed on them. As a result, atrophy of the peripheral parts of the liver, an increase in its size. The vena cava is even more clamped by the hypertrophied tissues of the organ, which ultimately leads to its complete obstruction.
Symptoms of Budd-Chiari Syndrome
Vessels of different caliber are capable of suffering from obstruction in this syndrome. Symptoms of Budd-Chiari syndrome, or rather, the degree of their severity, directly depends on how many veins feeding the liver are damaged. The latent clinical picture is observed when one vein is dysfunctional. In this case, the patient does not feel any disturbances in well-being, does not notice the pathological manifestations of the disease. However, if two or more veins are involved in the process, then the body begins to react to the violation quite violently.
Symptoms of the acute stage of the disease:
- A sudden start.
The appearance of high-intensity pain in the right hypochondrium and in the abdomen.
- The appearance of nausea, accompanied by vomiting.
- The skin and sclera of the eyes are moderately yellow.
- The liver increases in size.
- The legs swell a lot, the veins all over the body swell, bulge out from under the skin. This indicates the involvement of the vena cava in the pathological process.
- If the work of the mesenteric vessels is disrupted, the patient develops diarrhea, the pain spreads to the entire abdominal cavity.
- Renal failure with ascites and hydrothorax develops several days after the onset of the disease. The patient's condition worsens every day, bloody vomiting develops, drug correction helps little. Vomiting of blood most often indicates that the veins in the lower third of the esophagus have ruptured.
The acute form of the disease most often ends with a coma and death of a person. But even if he survives, then in the vast majority of cases, cirrhosis or hepatocellular carcinoma develops.
The chronic form of the disease manifests itself in a slightly different way and is characterized by the following clinical picture:
- The patient's state of health is not greatly disturbed. Periodically, he experiences weakness and fatigue.
- The liver is enlarged.
- As the disease progresses, pains appear in the right hypochondrium, vomiting occurs.
- The peak of the disease is cirrhosis of the liver with the subsequent formation of liver failure.
According to statistics, the chronic form of the disease occurs in 80% of cases. In addition, in the medical literature, there are descriptions of the fulminant form of Budd-Chiari syndrome, when the symptoms increase rapidly and the disease progresses at a high rate. Jaundice and renal failure with ascites develop within a few days.
As for the subacute form of the disease, the patient develops ascites, increases blood clotting, and increases the size of the spleen.
Diagnosing Budd-Chiari Syndrome
Diagnosis of Budd-Chiari syndrome is carried out by a gastroenterologist. He can make an assumption based on the presence of ascites and hepatomegaly, while an increase in blood clotting is always observed.
In order to clarify his assumption, the doctor will send the patient for further examination:
- Complete blood count, which reveals an increase in ESR and a jump in leukocytes.
- A coagulogram will indicate an increase in prothrombosed time.
- An increase in the activity of liver enzymes is noticeable in blood biochemistry.
- Ultrasound of the liver allows you to visualize the expansion of the hepatic veins, an increase in the portal vein in size. It is also possible to detect a thrombus or venous stenosis, which interferes with the normal blood supply to the organ. Doppler ultrasound is not excluded.
- MRI, CT, X-ray of the abdominal organs are instrumental diagnostic methods that make it possible to assess the size of the liver, the severity of diffuse and vascular changes, and also to determine the cause of the development of a pathological condition.
- The data of angiography and hepato-biopsy are considered informative.
Treatment for Budd-Chiari Syndrome
Treatment of Budd-Chiari syndrome is possible only in a hospital setting. Therapeutic measures are primarily aimed at normalizing venous blood flow. At the same time, the symptoms of the disease are eliminated.
Medication for Budd-Chiari Syndrome
As for drug treatment, it can only bring temporary relief to the patient. Patients are prescribed diuretics to remove excess fluid from the body. For this, potassium-sparing drugs (Veroshpiron, Spironolactone) and diuretics (Furosemide, Lasix) are used.
The administration of drugs aimed at improving metabolic processes in the liver is shown. It can be Essentiale, Legalon, Lipoic acid, etc.
Glucocorticosteroids can relieve severe pain. Each patient is recommended to administer antiplatelet agents (Trombonil, Plidol, Zilt, etc.) and fibrinolytics (Fibrinolysin, Curantil, Parmidin, Heparin), which promote clot resorption, increase the rheological properties of blood. Nevertheless, if the patient does not receive surgical treatment, then only drug correction leads to the death of patients in 90% of cases.
Surgical treatment of Budd-Chiari syndrome
The operation is aimed at normalizing the blood supply to the liver. However, if the patient has already developed hepatic insufficiency, or if hepatic vein thrombosis is present, then the operation is contraindicated.
If no such complications are observed, then one of the following types of interventions is possible:
- Imposition of an anastomosis. In this case, part of the vessels that have undergone pathological changes are replaced with artificial ones, which ensures the restoration of blood flow.
- Bypass surgery. With this type of surgery, additional pathways for blood outflow are created. So, the inferior vena cava is connected to the right atrium.
- A liver transplant helps to improve the functioning of the body.
- Expansion of the vena cava or its replacement with a prosthesis is performed with stenosis of the superior vena cava.
- If the patient has resistant ascites and oliguria, then he is shown a lymphovenous anastomosis.
As for the forecast, it is almost always unfavorable. It is influenced by the patient's age, the presence of concomitant diseases, the presence of liver cirrhosis. If the patient does not receive the necessary treatment, then death occurs within 3 months to 3 years from the moment of manifestation of the disease. The syndrome is especially dangerous for its severe complications, including: severe hepatic failure, internal bleeding, hepatic encephalopathy. The fulminant form of the disease is very unfavorable for life. With a chronic form of pathology, the life of patients increases to 10 years, and with a successful liver transplant from 10 years or more.
The author of the article: Gorshenina Elena Ivanovna | Gastroenterologist
Education: Diploma in the specialty "General Medicine" received at the Russian State Medical University named after N. I. Pirogova (2005). Postgraduate studies in the specialty "Gastroenterology" - educational and scientific medical center.