Budd-Chiari syndrome


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Budd-Chiari Syndrome

 

Definition

Budd-Chiari syndrome is a rare problem that results from blood clotting in the veins flowing out of the liver (hepatic veins). The high pressure of blood in these veins leads to an enlarged liver, and to an accumulation of fluid in the abdomen, called ascites.

Description

The liver, the largest internal organ in the human body, is responsible for many vital physiologic processes. Blood flow through the liver nourishes the liver, carries in substances that the liver will process, and carries away substances that the liver has produced. When blood cannot flow out freely from the liver, blood pressure rises in the veins of the liver, leading to blood clots within the liver. Also, some of the blood plasma can leak through the walls of the veins and accumulate within the abdomen (ascites).

Causes and symptoms

The major symptoms include pain in the upper right-hand portion of the abdomen and a build-up of fluid in the abdomen. In the United States, blood disorders are the most common causes. Among these disorders are polycythemia vera (an increase in the number of red blood cells), and sickle cell anemia. In parts of the world where liver cancer is common, a form of liver cancer is the most frequent cause.
Other causes sometimes include:
  • certain infections
  • use of oral contraceptives
  • body changes in pregnancy and the postpartum period
  • phlebitis (inflammation of a vein)
  • injury to the abdomen
  • membranous webs (especially in Asia)

Key terms

Ascites — Accumulation of fluid in the abdomen.
Biopsy — Surgical removal of a tiny bit of tissue for examination under the microscope.
Catheter — A tubular surgical instrument.
Phlebitis — Inflammation of a vein.
Polycythemia vera — An excess number of red blood cells in the blood.
Sickle cell anemia — An inherited disease in which red blood cells take an unusual shape, leading to circulation problems.

Diagnosis

Diagnosis of Budd-Chiari syndrome can be made by an internist (a specialist in diseases of the internal organs), a gastroenterologist (a specialist in the diseases of the digestive system), or a general surgeon. On physical examination, the doctor will note that the liver is larger than normal. Often an ultrasound scan of the liver will show abnormalities in the size of the liver, an abnormal pattern of the veins in the liver, and other abnormalities. A CT scan will often show similar abnormalities.
Once these abnormalities are confirmed, the key test is called hepatic vein catheterization. In this test, a narrow tube is snaked through the body until it reaches the hepatic veins. An instrument at the tip of the catheter can measure the pressure within each segment of the hepatic vein.
In some cases, a tiny amount of radioactive material is injected into a patient, and then an abnormal pattern of radioactivity in the liver can be revealed. In other cases, a liver biopsy enables a physician to examine cells from the liver itself. Cells damaged by Budd-Chiari syndrome have a characteristic appearance easily identifiable to a physician.

Treatment

Surgery

Most patients with Budd-Chiari syndrome must have surgery. A surgeon will re-route blood flow around the clotted hepatic vein into a large vein called the vena cava. The exact technique will depend on the specific location of the clots and other factors. In certain patients, other surgical techniques may be used. For patients who otherwise would have less than six months to live, liver transplantation is sometimes performed.
In a few patients, a "balloon catheter" can open the blocked blood vessels, without the need for major surgery.

Drugs

Sometimes, anti-clotting drugs such as urokinase can be used for patients with a sudden onset of clotting in the veins of the liver. These drugs do not seem to work when the clots have become established.

Prognosis

If surgery is done before permanent liver damage sets in, long-term survival is possible. In these cases, damaged liver cells can actually recover. If patients are already very sick with liver disease, the surgery may not be as helpful.

Prevention

The best approach to prevention is to carefully control the blood disorders that can lead to Budd-Chiari syndrome.

Resources

Books

Gadacz, Thomas R., and John L. Cameron. "Budd-Chiari Syndrome and Surgery of the Hepatic Vasculature." In Shackelford's Surgery of the Alimentary Tract, edited by J.G. Turcotte, 3rd ed. Vol. 3. Philadelphia: W. B. Saunders Co., 1991.

Chi·a·ri syn·drome

(kē-ah'rē),
thrombosis of the hepatic vein with great enlargement of the liver and extensive development of collateral vessels, intractable ascites, and severe portal hypertension.

Budd-Chiari syndrome

(bŭd′-kē-ä′rē)
n.
Obstruction of the hepatic vein, often due to thrombosis, leading to enlargement of the liver, ascites, and often development of collateral veins.

Budd-Chiari syndrome

[bud′kē·är′ē]
Etymology: George Budd, English physician, 1808-1882; Hans Chiari, Czech-French pathologist, 1851-1916
a disorder of hepatic circulation, marked by occlusion of the hepatic veins, that leads to liver enlargement, ascites, extensive development of collateral vessels, and severe portal hypertension. It may be congenital. Also called Chiari's syndrome, Rokitansky's disease.
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Budd-Chiari syndrome

Budd-Chiari syndrome

Hepatic vein obstruction Hepatology A loosely defined term for hepatic venous outflow obstruction, which has been used by various authors for both thrombotic and nonthrombotic obstruction of the large hepatic veins and the inferior vena cava Etiology Intravascular lesions including malignancy, myeloproliferative disorders, PNH, infection–eg schistosomiasis, intravascular webs; BCS is characterized by clotting of blood in the hepatic vein due to coagulation 2º to polycythemia vera or linked to OC use Clinical Ascites Lab Abnormal LFTs DiffDx Hepatic failure, GI bleeding, starvation Management Unsatisfactory

Budd-Chi·a·ri syn·drome

(bŭd kē-ahr'ē sin'drōm)
Hepatic vein obstruction; usually caused by thrombosis in inferior vena cava secondary to hypercoagulable states, vena caval webs, tumors, trauma, and bone marrow transplantation procedures. Can be idiopathic; most often associated with hepatomegaly and ascites.
Synonym(s): Rokitansky syndrome.

Budd-Chiari syndrome

A rare condition of clotting of the blood (thrombosis) in the large veins of the liver from excessive numbers of blood red cells (POLYCYTHAEMIA); myeloproliferative disorders; SICKLE CELL ANAEMIA; PAROXYSMAL NOCTURNAL HAEMOGLOBINURIA; the antiphospholipid syndrome; deficiencies of protein C, protein S and antithrombin III; injury or other causes. There is pain, liver enlargement, yellowing of the skin (JAUNDICE), liver failure, a collection of fluid in the abdomen (ascites) and, unless a porto-systemic shunt or liver transplant is done, usually death within a year. (George Budd, 1808–1882, English physician, and Hans von Chiari, 1851–1916, Austrian pathologist).

Budd,

George, English physician, 1808-1882.
Budd cirrhosis - chronic enlargement of the liver without jaundice.
Budd disease
Budd syndrome - Synonym(s): Chiari syndrome
Budd-Chiari syndrome - Synonym(s): Chiari syndrome
Chiari-Budd syndrome - Synonym(s): Chiari syndrome

Chiari,

Hans, German pathologist, 1851-1916.
Arnold-Chiari deformity - Synonym(s): Arnold-Chiari malformation
Arnold-Chiari malformation - see under Arnold, Julius
Arnold-Chiari syndrome - Synonym(s): Arnold-Chiari malformation
Budd-Chiari syndrome - Synonym(s): Chiari syndrome
Chiari disease - Synonym(s): Chiari syndrome
Chiari net - abnormal fibrous or lacelike strands in the right atrium.
Chiari syndrome - thrombosis of the hepatic vein with great enlargement of the liver and extensive development of collateral vessels, intractable ascites, and severe portal hypertension. Synonym(s): Budd-Chiari syndrome; Budd syndrome; Chiari-Budd syndrome; Chiari disease; Rokitansky disease (2)
Chiari-Budd syndrome - Synonym(s): Chiari syndrome
Chiari II syndrome - elongation of medulla and cerebellar tonsils and vermis with displacement through the foramen magnum into the upper spinal canal.
References in periodicals archive ?
Budd-Chiari syndrome may be asymptomatic but commonly presents with abdominal pain, hepatomegaly, and ascites.
His post-mortem report said: "This man has died of an extremely rare and unusual complication of malignant disease, namely the acute Budd-Chiari syndrome.
This finding was more consistent with Budd-Chiari syndrome than malignancy.
1) Because chronic constrictive pericarditis can present with signs and symptoms similar to other disease processes such as chronic pulmonary emboli, Budd-Chiari syndrome, cirrhosis of the liver, or restrictive cardiomyopathy, the identification of diagnostic radiographic signs has become increasingly valuable.