Liver & intrahepatic bile ducts

Vascular disorders

Budd-Chiari syndrome



Topic Completed: 19 February 2021

Minor changes: 25 February 2021

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PubMed Search: Budd-Chiari syndrome[TI] liver[TI] free full text[sb]


Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Budd-Chiari syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/liverbuddchiari.html. Accessed September 20th, 2021.
Definition / general
  • Venous outflow obstruction caused by occlusion of hepatic outflow
  • Either acute thrombotic occlusion (usually fatal) or subacute / chronic occlusion with hepatomegaly, ascites, abdominal pain
Essential features
  • Thrombotic outflow obstruction developing from various causes
  • Patients may experience fatal acute occlusion or may develop symptomatic chronic occlusion leading to cirrhosis
  • Sinusoidal dilation and portal tract changes are the most common findings on biopsy
Terminology
  • Also called hepatic vein thrombosis
  • Historically, Budd-Chiari syndrome technically referred to the triad of painful hepatomegaly, ascites and liver dysfunction
  • Membranous obstruction of the vena cava / obliterative hepatocavopathy likely represents recanalized thrombosis, more commonly seen in developing countries
ICD coding
  • ICD-10: I82.0 - Budd-Chiari syndrome
Epidemiology
  • Occurs in roughly 0.001% of the population
Sites
  • Occlusion may occur anywhere from hepatic venules to inferior vena cava
Pathophysiology
  • In addition to outflow obstruction, patients often have decreased portal perfusion with eventual compensatory increase in arterial inflow (Hepatology 2003;37:510)
Etiology
  • Hepatic vein thrombosis can occur for a variety of reasons (contraceptives, steroids, myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, pregnancy, postpartum state, hepatocellular carcinoma with inferior vena cava occlusion), though 30% of cases are idiopathic
  • Venous obliteration can lead to bridging fibrosis and eventually cirrhosis (Hepatology 1998;27:488)
Clinical features
  • Symptoms include painful hepatomegaly, jaundice, ascites and possibly liver failure
  • Elevation of transaminases and alkaline phosphatase (Mod Pathol 2004;17:874)
Diagnosis
  • Typically diagnosed by radiology, though some cases may be missed
Prognostic factors
  • High mortality for acute thrombotic occlusion (especially if all 3 hepatic veins are occluded)
  • 5 year survival of 50% for chronic form
Treatment
  • Anticoagulation; portosystemic venous shunt (causes reverse flow through portal vein); angiography (to dilate obstruction)
Gross description
  • Swollen, congested liver with reddish purple, tense capsule
Gross images

Images hosted on other servers:

Normal (left) versus occluded (right) + hepatic veins

Microscopic (histologic) description
  • Severe centrilobular congestion / necrosis, progressing to centrilobular fibrosis
  • Sinusoidal dilation, portal tract expansion / fibrosis and ductular reaction in zones 1 and 3 may be seen (Am J Surg Pathol 2014;38:205)
  • Large regenerative nodules, focal nodular hyperplasia and hepatocellular adenomas can develop (Histopathology 2004;44:172)
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.

Thrombus in hepatic vein

Sinusoidal dilation and congestion

Sample pathology report
  • Liver, biopsy:
    • Liver parenchyma with prominent zone 3 sinusoidal dilation (see comment)
    • Comment: The findings are most consistent with outflow obstruction (e.g. Budd-Chiari syndrome). Trichrome and iron stains are unremarkable.
Differential diagnosis
Additional references
Board review style question #1
Which of the following histologic findings is commonly seen in Budd-Chiari syndrome?

  1. Central vein thrombosis
  2. Lobular inflammation
  3. Periportal necrosis
  4. Sinusoidal dilation
Board review style answer #1
D. Sinusoidal dilation

Comment Here

Reference: Budd-Chiari syndrome
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