Budd-Chiari syndrome is due to the blockage of the hepatic venous outflow. For more details, read the article below.
Budd-Chiari syndrome is a rare complication named after British physician George Budd and Austrian pathologist Hans Chiari. It is characterized by obstruction of the outflow in the hepatic veins (veins that carry deoxygenated blood from the liver to the heart). It occurs in one in a million of the general population. The clinical manifestations are nonspecific, and imaging may be difficult in the early stages. Generally, obstruction of a single hepatic vein is not evident clinically, and the clinical manifestations are seen when two veins are blocked. However, if left untreated, the condition may become fatal.
The liver has many functions that include filtering all the blood in the body. When the outflow of blood is blocked fully or partially, it results in Budd-Chiari syndrome. The obstruction could be thrombotic (blood clots inside the blood vessel) or non-thrombotic, occurring anywhere between the hepatic vein to the inferior vena cava or the right atrium. This can result in high blood pressure of the portal vein, known as portal hypertension. This causes reduced hepatic perfusion, which eventually results in liver congestion. Then the blood flow to the liver cells is reduced, leading to an injury of the liver cells followed by the death of the liver cells. It is followed by progressive fibrosis or scarring and ultimately liver cirrhosis or liver failure.
BCS occurs commonly in women and young adults. It could be primary or secondary, depending on the origin of the obstructive lesion. If the obstruction is due to the hepatic venous lesion like a thrombosis, it is considered a primary BCS. If the block is due to tumor invasion or extrinsic causes, then it is a secondary BCS. Thrombosis is the primary cause of hepatic vein obstruction. Other causes include:
Hematologic diseases, especially myeloproliferative disorders.
Thrombotic diseases include paroxysmal nocturnal hemoglobinuria (characterized by destruction of red blood cells), antiphospholipid syndrome (disorder in which the immune system mistakenly affects the normal proteins), etc.
Membranous web-like obstruction of the hepatic vein.
Interruption of the diaphragm, which is congenital.
The use of oral contraceptives or pregnancy.
Infections such as tuberculosis, syphilis, aspergillosis.
Plant or herbal sources of pyrrolizidine alkaloids are toxic.
Protein S deficiency.
Leiomyosarcoma of inferior vena cava.
Polycythemia vera (type of blood cancer where there are excess red blood cells).
The clinical signs and symptoms in the patient depend upon the extent and acuteness of the hepatic venous obstruction. BCS includes the following signs and symptoms:
Severe upper abdominal pain.
Fluid accumulation within the peritoneal cavity (ascites).
Elevated liver enzymes.
Encephalopathy (damage to the brain).
Severe lactic acidosis.
Doppler ultrasonography of the liver, computed tomography(CT), magnetic resonance imaging (MRI), hepatic venography, metabolic function tests, cavograms, and liver biopsy are available diagnostic methods. The liver biopsy shows high-grade venous congestion, centrilobular liver cell atrophy, and thrombi within the terminal hepatic venules. Diagnosis can be based on the following:
Abrupt onset of ascites (fluid in the abdomen) and painful hepatomegaly (enlarged liver).
Massive ascites with preserved liver functions. On examination of the ascitic fluid in chronic patients, they usually have a high protein concentration (greater than two g/dL). The serum ascites-albumin gradient is generally less than 1.1.
Sinusoidal dilation in liver biopsy without heart disease.
Fulminant hepatic failure is associated with hepatomegaly and ascites.
Unexplained chronic liver disease.
Liver disease with a blood clotting disorder.
WBC (white blood cell) count is usually less than 500 per microlitre.
In acute cases, hepatomegaly, splenomegaly, and heterogeneous echotexture are present. There will be peripheral atrophy of the affected regions, regenerative nodules, gallbladder wall thickening, and ascites in chronic cases.
Color Doppler -
The following findings are seen in the color Doppler test:
No flow or inappropriately directed flow in the right hepatic vein.
The discontinuity between the central hepatic vein and the inferior vena cava. Reversed flow in hepatic veins.
Intra and extrahepatic collaterals, low or absent flow in the inferior vena cava. The high resistive index within the hepatic artery, tumor thrombus within the inferior vena cava.
Computed Tomography(CT) Findings -
The early enhancement of the caudate lobe (only part of the liver that is in contact with the inferior vena cava) and central liver,
Delayed enhancement of the peripheral liver with accompanying low central density (flip-flop appearance),
Nutmeg liver (inhomogeneous mottled liver),
Inability to identify hepatic veins.
Angiography Findings -
On hepatic venography, complete occlusion of hepatic vein(s) may be noted, which may or may not be associated with narrowing of the intrahepatic inferior vena cava.
The primary goal of the treatment should be to improve hepatic perfusion and preserve the functioning of the hepatic cells.
Medical Treatment -
It includes anticoagulant therapy- intravenous Heparin initially and then by Warfarin orally. Other measures to control ascites and gastrointestinal bleeding are taken. This includes reducing the salt intake in the diet, using diuretics such as Spironolactone and Furosemide. In the case of refractory ascites, large-volume paracentesis with albumin infusion is done. Normal serum albumin and bilirubin level indicate an improvement in liver functions. Thus liver function tests help evaluate the effectiveness of therapy. The medical treatment may alleviate the patient’s symptoms temporarily, but the disease progresses.
Surgical Treatment -
Percutaneous transhepatic balloon angioplasty (PTBA).
Surgical shunts may be placed to divert the blood flow around the obstruction of the liver. The TIPS (transjugular intrahepatic portosystemic shunts) can be employed. If all the hepatic veins are blocked, the portal veins can be approached by the intrahepatic part of the inferior vena cava. This procedure is called DIPS (direct intrahepatic portocaval shunt).
Liver transplantation is generally performed in the case of fulminant liver failure when the shunts fail.
Cirrhosis of the liver.
Ascites (fluid build-up in the abdomen).
Varicose veins in the esophagus.
Graft infection, rejection, arterial or venous thrombosis.
Gallbladder, kidney problems.
Low liver function can affect digestion.
Patients with chronic Budd-Chiari syndrome are at risk for hepatocellular carcinoma and should be monitored with serum alpha-fetoprotein.
The survival of the patient with Budd-Chiari syndrome is mainly dependent on the underlying cause. In general, nearly two-thirds of the patients survive for ten years. Some negative prognostic indicators like ascites, encephalopathy, elevated Child-Pugh scores, elevated prothrombin time, etc., indicate the patient's prognosis.
Yes, Budd-Chiari syndrome is curable. The disease, if diagnosed at the earliest, can be cured efficiently. The treatment includes administering drugs like Heparin, surgical treatment, and liver transplants in severe cases.
Budd-Chiari syndrome is a rare condition. It is mainly characterized by the blockage of hepatic veins to the liver. It affects one in a million of the adult population.
Yes, Budd-Chiari syndrome can be painful. It can cause severe abdominal pain in the upper right abdomen. The severity of pain will depend on the extent and acuteness of hepatic venous obstruction.
Budd-Chiari syndrome can equally affect both males and females. The presence of blood disorders, metastatic invasions, abdominal trauma, sickle-cell disease, use of oral contraceptives, Behcet’s disease, and immunosuppressant drugs can increase the chances of the disease.
Budd-Chiari syndrome can be a serious condition if left untreated. If there is no treatment, there can be complete blockage of the hepatic vein, and the patient can die within three years. Patients with partially blocked veins may live longer. Life expectancy can vary in individuals.
Budd-Chiari syndrome can be both acute and chronic. In acute cases, there can be severe blockage of the vein and is accompanied by severe pain. In chronic conditions, the onset is gradual.
Yes, Budd-Chiari syndrome may cause liver cirrhosis. But it is a rare cause that contributes to liver cirrhosis. Chronic Budd-Chiari syndrome is responsible for liver cirrhosis.
Budd-Chiari syndrome is characterized by the blockage of the hepatic vein. Thrombosis is the main cause of blockage of the hepatic vein. Primary myeloproliferative diseases are responsible for thrombosis and the main causative factor for Budd-Chiari syndrome.
In acute cases of Budd-Chiari syndrome, it is often characterized by hepatomegaly, splenomegaly, and heterogeneous echotexture. In chronic cases, gallbladder wall thickening, ascites, regenerative nodules, peripheral atrophy of affected regions, and hypertrophied caudate lobe can be seen in the ultrasound.
The Budd-Chiari syndrome could be primary or secondary depending upon the pathophysiology of the disease. The primary type is mainly caused by the congenital blockage of hepatic veins, whereas the secondary can be due to tumor invasion or any other extrinsic causes.
Last reviewed at:
28 Jan 2022 - 5 min read
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