Budd-Chiari syndrome (BCS) is an obstruction of the draining major hepatic vein. If left untreated, BCS is extremely painful and results in liver failure. BCS is very rare; more commonly, there is occlusion of multiple small hepatic veins. However, BCS is strictly differentiated from this finding.
What is Budd-Chiari syndrome?
Budd-Chiari syndrome (BCS) refers to complete occlusion of the major hepatic vein. A BCS can be acute or chronic. In an acute BCS, the occlusion occurs suddenly and causes the patient’s condition to deteriorate rapidly. In a chronic course, blood outflow via the great hepatic vein is permanently impaired. The occlusion of the vein leads to blood stasis in the liver. This causes the liver to become abnormally “distended,” as a result of which the liver can no longer perform its functions. If left untreated, BCS leads to liver failure.
Causes
There are three main possible causes of BCS. The most common is thrombosis – a blood clot – in the large vein, which ultimately leads to occlusion. In addition, a liver tumor can be the cause, which – if it is located unfavorably and has reached a certain size – can occlude the vein. Sometimes it happens that a tumor surrounds the vein from the outside and thus compresses the vein. Another possible cause of BCS is inflammation of the liver, such as chronic or acute hepatitis.
Symptoms, complaints, and signs
In the worst case, Budd-Chiari syndrome can lead to the death of the affected person. Usually, this occurs when the syndrome is not treated. The affected person primarily suffers from very severe pain in the lower abdominal region. In the upper abdominal area, there is a strong feeling of pressure. This considerably reduces and restricts the quality of life of the affected person. Budd-Chiari syndrome also leads to the development of ascites. The spleen and liver also enlarge in the course of the disease, which can lead to severe pain. If treatment is not received, nausea, diarrhea and vomiting will occur. Water retention in the abdomen can also be associated with severe pain. Due to a subsequent failure of the liver, the affected person eventually dies of Budd-Chiari syndrome. The severe pain can also lead to loss of consciousness or even coma. It cannot be predicted whether the affected person will awaken from this coma. Often, Budd-Chiari syndrome also leads to severe psychological discomfort in the patients or in the relatives of the affected person, so that they are dependent on psychological treatment.
Diagnosis and course
Based on the typical course of BCS or impending liver failure, a physician can make an appropriate diagnosis quite quickly and accurately. He will ask the patient about possible causes (such as the presence of inflammation or similar) and palpate the abdomen. If the suspicion of BCS is confirmed, the physician will perform a sonography (ultrasound examination) and – if necessary – a liver venography to obtain a more precise picture of the occlusion. Acute BCS is very painful. Almost immediately after occlusion of the vein, severe pain occurs in the area of the right upper abdomen, often accompanied by a strong feeling of pressure in the entire abdominal cavity. Vomiting and violent nausea are also accompanying symptoms. In the later course, water retention in the abdominal cavity (ascites) may occur. The condition of a patient with acute BCS deteriorates dramatically within a short time. The condition can lead to coma and is not infrequently life threatening. The medical profession refers to chronic outflow obstruction when blood outflow via the hepatic vein is permanently impaired but not completely interrupted or constantly recurs. The consequence of chronic BCS is usually a pathologically enlarged liver, resulting in cirrhosis.
When should you see a doctor?
If there is severe abdominal pain and other signs that indicate serious disease of the internal organs, a doctor must be consulted immediately. In Budd-Chiari syndrome, the condition deteriorates rapidly, so immediate treatment is vital. At the latest when water retention in the abdomen is added to the typical symptoms, a visit to the doctor is necessary.If the affected person falls into a coma, the emergency physician must be alerted immediately. Severe vomiting and pain are also best treated by emergency medical services. Patients with chronic or acute hepatitis or liver inflammation are particularly at risk. People suffering from thrombosis or other diseases of the vessels and veins, or who have a liver tumor, should also go to their family doctor at the first symptoms. Other contacts are specialists in internal medicine or a specialist in venous diseases. It may be necessary to visit a specialist clinic after diagnosis, where a liver transplant is performed. Because of the risk of relapse, regular follow-up visits to the responsible physician are indicated after treatment.
Treatment and therapy
To restore optimal blood flow through the major hepatic vein, the physician will first attempt to dissolve the thrombosis with medication (thrombolysis) if BCS is present. If this does not succeed, the insertion of a shunt can be considered. In very simplified terms, a shunt serves to bypass the source of occlusion by means of a “detour”. It is also possible to remove the occlusion by means of a surgical technique tailored to the specific situation. If a BCS is chronic, i.e. if the occlusion of the large hepatic vein occurs frequently, the liver will be permanently damaged. To prevent this, the doctor will prescribe a drug to inhibit blood clotting (such as Marcumar). If this also fails, or if a patient suffers permanently from the side effects of the medication, a liver transplant may be indicated.
Outlook and prognosis
Budd-Chiari syndrome must be treated in all cases. There is no self-healing in this disease and death continues to occur in the affected individual if treatment is not initiated. Usually, the patient then dies due to liver failure. The syndrome is further also associated with very severe pain if no treatment is initiated. Treatment primarily involves the administration of medications to relieve the symptoms. However, if these are ineffective, patients rely on a shunt to relieve discomfort. If the disease becomes chronic, the liver is irreversibly damaged and the patient dies. In this case, a liver transplant is eventually necessary to keep the affected person alive. However, this can also lead to severe side effects and various complications, so that no general course of the disease can be given. In many cases, however, the prognosis for Budd-Chiari syndrome is relatively poor, resulting in a reduced life expectancy. An early diagnosis of Budd-Chiari syndrome always has a positive effect on the further course of the disease.
Prevention
BCS can only be prevented to a limited extent. Patients who have a tendency to develop BCS because of a previous illness – such as a tendency to thrombosis, the presence of tumor disease, or hepatitis – should have regular checkups. If there is a risk of developing chronic BCS, prophylactic use of an anticoagulant may be considered. It is also indicated not to put unnecessary stress on the liver, such as excessive consumption of alcohol or medications.
Follow-up
Follow-up is rarely possible in Budd-Chiari syndrome. The disease is primarily treated first with the help of medications, so these must be taken regularly. Likewise, possible interactions with other medications should be checked here and discussed with a physician. If drug treatment does not bring the desired success, Budd-Chiari syndrome must be treated by surgery. In some cases, however, the affected person’s liver is already so severely damaged that the patient will die if a transplant cannot be performed. After transplantation, the liver must be permanently monitored to avoid complications. The patient must be prepared for a longer stay in a hospital. Wound healing must also be encouraged. Unnecessary exertion or sports activities should be avoided. The patient must pay attention to a healthy lifestyle with a healthy diet. Alcohol and nicotine should be avoided completely.In most cases, despite treatment, the patient’s life expectancy is significantly reduced by Budd-Chiari syndrome. Even after successful treatment, the patient is dependent on taking medications and having regular checkups with a physician.
What you can do yourself
Individuals diagnosed with Budd-Chiari syndrome primarily require comprehensive medical treatment. Medical therapy can be supported by various self-help measures and the use of alternative remedies from natural medicine. First of all, the affected person should pay attention to strict personal hygiene. Since shunts are usually placed in Budd-Chiari syndrome, there is an increased risk of infection. This makes regular washing all the more important, especially in the affected area. Moderate outdoor exercise and a healthy diet can further promote recovery. If the affected area shows signs of inflammation, the doctor must be informed immediately. The physician will usually also recommend rest and bed rest for the patient. Sufficient rest is particularly important in the first weeks and months, as the disease can place a great strain on the body and mind. In order to avoid mental discomfort, a therapist should be consulted to accompany the physical treatment. Patients who feel depressed as a result of the disease or suffer from unusual mood swings are best advised to talk to their doctor. Often the symptoms can be alleviated by a change in medication, but in some cases further treatment by a therapist or psychotherapist is indicated.