Budd-Chiari Syndrome

  • Yukio KuniyoshiEmail author


We operated on 69 patients with Budd-Chiari syndrome (BCS) using an operative procedure we devised, 59 of whom were enrolled in this study. From their data, the characteristics of BCS in our surgical patient group were evaluated. The inferior vena cava (IVC) was occluded completely in 43 patients and severely stenotic in 16 patients. The number of patent hepatic veins (HVs) ranged from 0 to 3 per patient, and an average of 1.2 ± 0.72. Esophageal varices (EVs) were found in 52 patients (88.1%). Histologic classification of the specimen obtained in the surgery was liver cirrhosis in 35 patients, liver fibrosis in 18 patients, and liver congestion in 6 patients. Fibrous tissue in liver parenchyma increased with aging. A good correlation existed between liver histology and patient age. Hepatocellular carcinoma (HCC) was found in 12 patients (20.3%) diagnosed before or after BCS surgery and was basically treated by surgical excision. After BCS surgery, EVs disappeared in 14 patients in the early postoperative period and in 5 patients in the late postoperative period. The mean number of patent HVs increased from 1.2 ± 0.72 per patient to 2.41 ± 0.80 per patient. The pressure gradient between infraoccluded IVC and right atrium decreased from 12.9 ± 3.72 to 4.67 ± 2.97 mmHg.

In summary, occlusion of HVs irrespective of IVC occlusion induces liver congestion, resulting in increasing fibrous tissue in liver parenchyma and increasing portal pressure. By reopening the occluded HVs surgically, the EVs disappear, and the increase of fibrous tissue can be inhibited.


Budd-Chiari syndrome Hepatic outflow occlusion Portal hypertension Esophageal varices 


  1. 1.
    Budd G. On Diseases of the Liver. !st Ed. London: John Churchil; 1845.146Google Scholar
  2. 2.
    Chiari H. Ueber die silbstandinge phlebitis obliterans der hauptstamme der venae hepaticae als todesursache. Beitr Pathol Anat. 1899;26:1–18.Google Scholar
  3. 3.
    Horton JD, San Miguel FL, Membreno F, Wright F, Paima J, Foster P, Ortiz JA. Budd-Chiari syndrome: illustrated review of current management. Liver Int. 2008;28:455–66.CrossRefGoogle Scholar
  4. 4.
    Martens P, Nevens F. Budd-Chiari syndrome. Gastroenterol J. 2015;3:489–500.Google Scholar
  5. 5.
    Zeitoun G, Escolano S, Hadengue A, Azar N, El Younsi M, Mallet A, Boudet MJ, Hay JM, Erlinger S, Benhamou JP, Belghiti J, Valla D. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology. 1999;30:84–9.CrossRefGoogle Scholar
  6. 6.
    Koja K, Kusaba A, Kuniyoshi Y, Iha K, Akasaki M, Miyagi K. Radical open endvenectomy with autologous pericardial patch graft for correction of Budd-Chiari syndrome. Cardiovasc Surg. 1996;4:500–4.CrossRefGoogle Scholar
  7. 7.
    Kuniyoshi Y, Koja K, Akasaki M, Miyagi K, Shimoji M, Kudaka M, Uezu T. Improvement in esophageal varices and liver histology postoperatively in Budd-Chiari syndrome. Ann Thorac Surg. 1998;65:1711–4.CrossRefGoogle Scholar
  8. 8.
    Inafuku H, Morishima Y, Nagano T, Arakaki K, Yamashiro S, Kuniyoshi Y. A three-decade experience of radical open endvenectomy with pericardial patch graft for correction of Budd-Chiari syndrome. J Vasc Surg. 2009;50:590–3.CrossRefGoogle Scholar
  9. 9.
    Kimura C. Surgical treatment of portal hypertension of post-hepatic origin, with special reference to trans-cardiac membranotomy for membranous obstruction of the hepatic portion of the inferior vena cava. Jpn Circ J. 1964;28:181–3.CrossRefGoogle Scholar
  10. 10.
    Manabe H. Endovenectomy for the surgical treatment of Budd-Chiari syndrome. Jpn Circ J. 1964;28:183–6.CrossRefGoogle Scholar

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© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.Department of Thoracic and Cardiovascular SurgeryGraduate School of Medicine, University of the RyukyusNishihara-choJapan

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