Budd-Chiari syndrome: Etiology, diagnosis and management

M. C. Mitchell, J. K. Boitnott, S. Kaufman, J. L. Cameron, W. C. Maddrey

Research output: Contribution to journalArticle

290 Citations (Scopus)

Abstract

Hepatic venous thrombosis (the Budd-Chiari syndrome) is a rare disorder characterized by right upper quadrant abdominal pain, hepatomegaly and ascites. In the past 10 years we have evaluated 12 patients with this syndrome. Most patients have an acute onset of symptoms but occasionally the disorder appears insidiously over a long period of time. The most frequent underlying conditions associated with the development of hepatic vein thrombosis are hematologic disorders including polycythemia rubra vera and paroxysmal nocturnal hemoglobinuria and the use of oral contraceptives. Certain tumors associated with a tendency to vascular thrombosis including hepatocellular carcinoma and renal cell carcinoma must also be considered. At the time of diagnosis the patient usually has hepatomegaly, abdominal pain and ascites. The hepatic scan may be useful in suggesting the diagnosis with marked decrease in uptake over affected areas of the liver. Occasionally the caudate lobe of the liver is not involved in the thrombotic process and may appear as an hypertrophied lobe with accumulation of tracer. The liver biopsy is characteristic with intense congestion and necrosis around the terminal hepatic venule. There is a scant inflammation in the liver biopsy and the process affects liver cells by back pressure leading to atrophy. Radiographic studies of the inferior vena cava and attempts to catheterize the hepatic vein often further establish the diagnosis. Once the hepatic veins are thrombosed little can be done to reverse the thrombosis. Only in patients with an acute thrombosis would thrombolytic therapy be expected to be useful. The peritoneojugular shunt (LeVeen) may provide relief from ascites but does little to correct the underlying pathophysiology. Some form of side to side portasystemic decompressive therapy offers the patient the best chance of a long term survival. Both mesocaval and mesoatrial shunts have been associated with dramatic results in some patients. We believe, when possible, these shunts should be used in the Budd-Chiari syndrome to prevent further progression of injury to hepatocytes by relieving back pressure.

Original languageEnglish (US)
Pages (from-to)199-218
Number of pages20
JournalMedicine
Volume61
Issue number4
StatePublished - 1982

Fingerprint

Budd-Chiari Syndrome
Liver
Ascites
Thrombosis
Hepatomegaly
Abdominal Pain
Peritoneovenous Shunt
Paroxysmal Hemoglobinuria
Biopsy
Pressure
Polycythemia Vera
Hepatic Veins
Venules
Thrombolytic Therapy
Inferior Vena Cava
Oral Contraceptives
Renal Cell Carcinoma
Venous Thrombosis
Atrophy
Blood Vessels

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Mitchell, M. C., Boitnott, J. K., Kaufman, S., Cameron, J. L., & Maddrey, W. C. (1982). Budd-Chiari syndrome: Etiology, diagnosis and management. Medicine, 61(4), 199-218.

Budd-Chiari syndrome : Etiology, diagnosis and management. / Mitchell, M. C.; Boitnott, J. K.; Kaufman, S.; Cameron, J. L.; Maddrey, W. C.

In: Medicine, Vol. 61, No. 4, 1982, p. 199-218.

Research output: Contribution to journalArticle

Mitchell, MC, Boitnott, JK, Kaufman, S, Cameron, JL & Maddrey, WC 1982, 'Budd-Chiari syndrome: Etiology, diagnosis and management', Medicine, vol. 61, no. 4, pp. 199-218.
Mitchell MC, Boitnott JK, Kaufman S, Cameron JL, Maddrey WC. Budd-Chiari syndrome: Etiology, diagnosis and management. Medicine. 1982;61(4):199-218.
Mitchell, M. C. ; Boitnott, J. K. ; Kaufman, S. ; Cameron, J. L. ; Maddrey, W. C. / Budd-Chiari syndrome : Etiology, diagnosis and management. In: Medicine. 1982 ; Vol. 61, No. 4. pp. 199-218.
@article{3cdbd09476f647fbb7808ee8ae468b19,
title = "Budd-Chiari syndrome: Etiology, diagnosis and management",
abstract = "Hepatic venous thrombosis (the Budd-Chiari syndrome) is a rare disorder characterized by right upper quadrant abdominal pain, hepatomegaly and ascites. In the past 10 years we have evaluated 12 patients with this syndrome. Most patients have an acute onset of symptoms but occasionally the disorder appears insidiously over a long period of time. The most frequent underlying conditions associated with the development of hepatic vein thrombosis are hematologic disorders including polycythemia rubra vera and paroxysmal nocturnal hemoglobinuria and the use of oral contraceptives. Certain tumors associated with a tendency to vascular thrombosis including hepatocellular carcinoma and renal cell carcinoma must also be considered. At the time of diagnosis the patient usually has hepatomegaly, abdominal pain and ascites. The hepatic scan may be useful in suggesting the diagnosis with marked decrease in uptake over affected areas of the liver. Occasionally the caudate lobe of the liver is not involved in the thrombotic process and may appear as an hypertrophied lobe with accumulation of tracer. The liver biopsy is characteristic with intense congestion and necrosis around the terminal hepatic venule. There is a scant inflammation in the liver biopsy and the process affects liver cells by back pressure leading to atrophy. Radiographic studies of the inferior vena cava and attempts to catheterize the hepatic vein often further establish the diagnosis. Once the hepatic veins are thrombosed little can be done to reverse the thrombosis. Only in patients with an acute thrombosis would thrombolytic therapy be expected to be useful. The peritoneojugular shunt (LeVeen) may provide relief from ascites but does little to correct the underlying pathophysiology. Some form of side to side portasystemic decompressive therapy offers the patient the best chance of a long term survival. Both mesocaval and mesoatrial shunts have been associated with dramatic results in some patients. We believe, when possible, these shunts should be used in the Budd-Chiari syndrome to prevent further progression of injury to hepatocytes by relieving back pressure.",
author = "Mitchell, {M. C.} and Boitnott, {J. K.} and S. Kaufman and Cameron, {J. L.} and Maddrey, {W. C.}",
year = "1982",
language = "English (US)",
volume = "61",
pages = "199--218",
journal = "Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries",
issn = "0025-7974",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Budd-Chiari syndrome

T2 - Etiology, diagnosis and management

AU - Mitchell, M. C.

AU - Boitnott, J. K.

AU - Kaufman, S.

AU - Cameron, J. L.

AU - Maddrey, W. C.

PY - 1982

Y1 - 1982

N2 - Hepatic venous thrombosis (the Budd-Chiari syndrome) is a rare disorder characterized by right upper quadrant abdominal pain, hepatomegaly and ascites. In the past 10 years we have evaluated 12 patients with this syndrome. Most patients have an acute onset of symptoms but occasionally the disorder appears insidiously over a long period of time. The most frequent underlying conditions associated with the development of hepatic vein thrombosis are hematologic disorders including polycythemia rubra vera and paroxysmal nocturnal hemoglobinuria and the use of oral contraceptives. Certain tumors associated with a tendency to vascular thrombosis including hepatocellular carcinoma and renal cell carcinoma must also be considered. At the time of diagnosis the patient usually has hepatomegaly, abdominal pain and ascites. The hepatic scan may be useful in suggesting the diagnosis with marked decrease in uptake over affected areas of the liver. Occasionally the caudate lobe of the liver is not involved in the thrombotic process and may appear as an hypertrophied lobe with accumulation of tracer. The liver biopsy is characteristic with intense congestion and necrosis around the terminal hepatic venule. There is a scant inflammation in the liver biopsy and the process affects liver cells by back pressure leading to atrophy. Radiographic studies of the inferior vena cava and attempts to catheterize the hepatic vein often further establish the diagnosis. Once the hepatic veins are thrombosed little can be done to reverse the thrombosis. Only in patients with an acute thrombosis would thrombolytic therapy be expected to be useful. The peritoneojugular shunt (LeVeen) may provide relief from ascites but does little to correct the underlying pathophysiology. Some form of side to side portasystemic decompressive therapy offers the patient the best chance of a long term survival. Both mesocaval and mesoatrial shunts have been associated with dramatic results in some patients. We believe, when possible, these shunts should be used in the Budd-Chiari syndrome to prevent further progression of injury to hepatocytes by relieving back pressure.

AB - Hepatic venous thrombosis (the Budd-Chiari syndrome) is a rare disorder characterized by right upper quadrant abdominal pain, hepatomegaly and ascites. In the past 10 years we have evaluated 12 patients with this syndrome. Most patients have an acute onset of symptoms but occasionally the disorder appears insidiously over a long period of time. The most frequent underlying conditions associated with the development of hepatic vein thrombosis are hematologic disorders including polycythemia rubra vera and paroxysmal nocturnal hemoglobinuria and the use of oral contraceptives. Certain tumors associated with a tendency to vascular thrombosis including hepatocellular carcinoma and renal cell carcinoma must also be considered. At the time of diagnosis the patient usually has hepatomegaly, abdominal pain and ascites. The hepatic scan may be useful in suggesting the diagnosis with marked decrease in uptake over affected areas of the liver. Occasionally the caudate lobe of the liver is not involved in the thrombotic process and may appear as an hypertrophied lobe with accumulation of tracer. The liver biopsy is characteristic with intense congestion and necrosis around the terminal hepatic venule. There is a scant inflammation in the liver biopsy and the process affects liver cells by back pressure leading to atrophy. Radiographic studies of the inferior vena cava and attempts to catheterize the hepatic vein often further establish the diagnosis. Once the hepatic veins are thrombosed little can be done to reverse the thrombosis. Only in patients with an acute thrombosis would thrombolytic therapy be expected to be useful. The peritoneojugular shunt (LeVeen) may provide relief from ascites but does little to correct the underlying pathophysiology. Some form of side to side portasystemic decompressive therapy offers the patient the best chance of a long term survival. Both mesocaval and mesoatrial shunts have been associated with dramatic results in some patients. We believe, when possible, these shunts should be used in the Budd-Chiari syndrome to prevent further progression of injury to hepatocytes by relieving back pressure.

UR - http://www.scopus.com/inward/record.url?scp=0019985276&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0019985276&partnerID=8YFLogxK

M3 - Article

C2 - 7045569

AN - SCOPUS:0019985276

VL - 61

SP - 199

EP - 218

JO - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

JF - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

SN - 0025-7974

IS - 4

ER -