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Budd-Chiari Syndrome

  • Yukio KuniyoshiEmail author
Chapter

Abstract

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.

Keywords

Budd-Chiari syndrome Hepatic outflow occlusion Portal hypertension Esophageal varices 

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Copyright information

© 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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