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Budd-Chiari Syndrome (BCS)

Overview

Budd-Chiari syndrome (BCS) is a disorder in which hepatic veins (veins in the liver) are obstructed or constricted. This obstruction results in blood backing up further into the liver, which causes the liver to enlarge. Additionally, the spleen could enlarge as well.

BCS is a clinical disorder with a wide range of prognoses; it may be treatable or fatal. Unlike with other long-term liver illnesses, patients' prognoses are generally tolerable with the right treatment. Because many conditions, including blood or malignant diseases, may be exacerbated by BCS, it is an uncommon but relevant condition.

Additional disorders that Budd-Chiari Syndrome might bring on include:

·         Portal hypertension: A condition where the portal vein, which delivers blood out of the intestines to the liver, is under greater pressure.

·         Esophageal varices: These are twisted veins in the feeding tube.

·         Ascites: An accumulation of liquid in the abdomen.

·         Cirrhosis: The liver becomes scarred in cirrhosis.

 

·         Varicose veins: These are abnormal, bulging blood vessels in the tummy.

Causes

In 75% of the patients, a hypercoagulable condition could be established, while in the other 25%, more than one causative factor may be involved. The following are some factors that make your blood clot and may lead to Budd-Chiari syndrome.

·         Myeloproliferative disorders, which involve the blood and bone marrow, cause thrombocytopenia and polycythemia.

·         Sickle cell disease: It is a blood disorder in which red cells become sickle-shaped instead of round-shaped.

·         Inflammatory bowel disease (IBD) causes swelling of the digestive system.

 

·         Pregnancy can also be a risk factor for Budd-Chiari syndrome.

Types

Primary Budd-Chiari syndrome

The primary cause of Budd-Chiari syndrome is hepatic vein thrombosis. In decreasing order of incidence, the following are linked to hepatic vein thrombosis:

·         Vera polycythemia

·         Paraoximal nocturnal hemoglobinuria

·         Liver cancer

·         Pregnancy

·         Postpartum period

·         Lupus anticoagulants 

Secondary Budd-Chiari syndrome

Hepatic vein compression due to pressure from outside can cause secondary Budd-Chiari syndrome. Additionally, Budd-Chiari syndrome is occasionally observed with inferior vena caval stenosis, congenital venous webs, and TB. Protein C and protein S deficiencies, the Factor V Leiden mutation, and hereditary anti-thrombin deficiency are a few examples of genetic inclinations. Any use of birth control pills is a significant non-hereditary risk factor. Antiphospholipid syndrome, aspergillosis, Behçet's disease, dacarbazine, pregnancy, and trauma are additional risk elements.

Epidemiology

According to meta-analyses, the combined yearly incidence and prevalence of BCS were 1 per million and 11 per million, respectively. In the general community, this condition affects 1 in 100,000 people.

Signs And Symptoms

Budd-Chiari syndrome signs and symptoms include:

·         Upper abdominal discomfort

·         Upper abdominal swelling 

·         Yellow color to one's skin 

·         A large liver

·         Bleeding from the food pipe

·         Vomiting

·         Expanded spleen

·         Leg’s swelling

·         Liver dysfunction

·         Liver encephalopathy, in which the patient is confused or unconscious.

 

·         Lethargy

Diagnosis

A proper history, detailed examination, and certain tests are used to identify Budd-Chiari syndrome. Your doctor will inquire about your symptoms and check for Budd-Chiari symptoms such as abdominal swelling or pain. Your doctor will also order blood tests to assess your liver's health and determine whether you have a higher risk of blood clots.

Liver function tests

The upper limit of the normal range for serum transferase levels might well be exceeded by more than five times. The levels of bilirubin and serum alkaline phosphatase also rise. However, the level of serum albumin decreases.

Doppler ultrasonography

The first diagnostic test should be hepatic Doppler ultrasonography. Sensitive ultrasound findings of the condition include hepatic vein collaterals.

Angiography

Using a contrast dye injected into the body during an angiography, a specialized X-ray, physicians may inspect blood channels.

MRI Scan

MRIs are imaging tests that allow doctors to see the body's soft tissues and organs. Although MRI is more costly than CT scanning, it can assess the inferior vena cava and detect hepatic vein occlusion.

Liver biopsy

During a biopsy, a tiny piece of hepatic tissue is taken and seen under a microscope. A liver biopsy is crucial in distinguishing BCS from veno-occlusive disorder, characterized by non-thrombotic blockage of hepatic venules.

Hepatic venography

 

Hepatic venography is a method used to assess the pressure in the inferior vena cava, thrombosis severity, and liver veins. A spider-web pattern supports the diagnosis of BCS.

Differential Diagnosis

The following conditions can have effects similar to Budd-Chiari syndrome: A differential diagnosis may benefit from comparisons:

·         Hepatic Arterial Lesions

 

·         Polycythemia vera

Treatment

Blood-thinning medications

Early diagnosis of Budd-Chiari syndrome may allow for the use of anticoagulants or other clot-dissolving medicines as a form of therapy. These medications aid in preventing blood clot formation. Examples are warfarin and heparin.

Procedure

Through a technique known as angioplasty, doctors can occasionally cure individuals by expanding the troubled veins. By doing so, you may be able to avoid blood clot-related blood vessel pressure buildup and prevent blood from backing up. Unclogging a vein and inserting a stent into it might be the third therapy. Stents are tiny tubes that are inserted into the vein to allow blood to flow freely and unhindered.

Transjugular Intrahepatic Portosystemic Shunt

TIPS  is a non-invasive technique involving a stent placement in the liver. The doctor will connect the portal vein with any hepatic vein and then keep a stent in it to keep it intact.

 Percutaneous Transluminal Angioplasty

In this technique, the doctor puts a catheter via the skin into blood channels, and balloon inflation is done where there is a clot in the blood channel.

Transplantation of the liver

 

Finally, liver transplantation may well be considered as a therapy option if a person has had extensive liver impairment.

Prognosis

Patients who do not receive treatment have a dismal prognosis and will likely pass away within three months to three years of their diagnosis due to growing liver failure. However, after portosystemic shunting, individuals with the condition had a 38–87% median survival.

Lifestyle Modifications

The following lifestyle changes can help you live with this disease:

·         For the management of ascites, a low-sodium diet is advised.

·         Eating healthy foods like fruits, veggies, lean meat, and whole grains should be a top priority.

·         Even if you're consuming less than before, consuming too much fatty food might still harm your liver.

·         To keep your body hydrated, you should also consume sufficient water daily.

·         Eat foods that can lower amounts of harmful cholesterol.

·         It's important to refrain from drinking since alcohol can harm your liver, particularly if it's already damaged, even if your cirrhosis is unrelated to alcohol consumption.

 

·         Apples, grapes, and citrus-like lemons should all be included in your diet as they are good for the liver. To raise antioxidant levels in the body and shield your liver from toxins, consume grapes in their natural form, in the form of grape juice, or add grape seed preparations to your diet.