Asian Cardiovasc Thorac Ann 2006;14:e88-e90
© 2006 Asia Publishing EXchange Ltd
Surgical Repair for Entire Inferior Vena Cava Occlusion in Budd-Chiari Syndrome
Kazuhisa Rikitake, MD,
Tsuyoshi Itoh, MD,
Masafumi Natsuaki, MD,
Yuji Katayama, MD,
Satoshi Ohtsubo, MD,
Hiroumi Kataoka, MD
Department of Thoracic Surgery, Saga Medical School, Saga, Japan
For reprint information contact: Kazuhisa Rikitake, MD Tel: 81 95 234 2345 Fax: 81 95 234 2061 Email: rikitak{at}post.saga-med.ac.jp, Department of Thoracic Surgery, Saga Medical School, Nabeshima 5-1-1, Saga city 849-0937, Saga, Japan.
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ABSTRACT
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Successful repair of thrombotic occlusion of the entire inferior vena cava, including the iliac and hepatic veins, was performed on a 51-year-old man with Budd-Chiari syndrome associated with severe liver dysfunction.
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INTRODUCTION
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Budd-Chiari syndrome (BCS) is caused by occlusion of the hepatic veins and is often associated with obstruction of the retrohepatic inferior vena cava (IVC). The common clinical features are abdominal pain, ascites, and hepatomegaly.1,2 Dilated venous channels of the abdominal wall and leg edema may be present in cases with IVC occlusion. Various treatments for BCS have been tried, such as balloon angioplasty, a shunt operation, and a radical operation that included transatrial finger disobliteration, the Senning procedure, and a lateral approach.
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CASE REPORT
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A 51-year-old man had suffered severe liver dysfunction for some years. Budd-Chiari syndrome had been recognized 6 months before admission. At that time, angiography demonstrated only membranous obstruction of the retrohepatic IVC with a patent right hepatic vein. Hematemesis developed from esophageal varices, resulting in hepatic coma despite full medication, and the patient was referred to our department for surgical treatment. Physical examination revealed vein dilatation of the abdominal wall, ascites, and leg edema. Laboratory data indicated mild hyperbilirubinemia (total bilirubin, 2.3 mg·dL–1) and poor excretion of indocyanine green (36.6%) in a clearance test. Liver enzymes were normal, but the prothrombin time was severely decreased (23%). Abdominal ultrasonography showed interruption of the left and middle hepatic veins, and long segmental obstruction of the right hepatic vein. Additionally, retrograde flow was observed in the portal veins. Computed tomography revealed narrowing of the retrohepatic IVC (Figure 1A
) and thrombotic occlusion of the entire IVC (Figure 1B) and the iliac veins (Figure 1C). Only membranous obstruction of the retrohepatic IVC had been found on angiography in the previous hospital, but preoperative angiography in our hospital demonstrated rapid progression to entire IVC obstruction (Figure 2A).
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Figure 1. (A) Computed tomography demonstrating narrowing of the retrohepatic inferior vena cava, and (B) thrombotic occlusion of the entire inferior vena cava and (C) iliac veins.
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Figure 2. Preoperative angiography demonstrating obstruction of the entire inferior vena cava; (A) Venous blood was returned to the right atrium through the hemiazygos vein; (B) Postoperative angiography showing good patency of the entire inferior vena cava and iliac veins.
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A radical operation via a lateral approach was performed using femorofemoral (F-F) bypass. The patient was placed in a left semilateral position, and a right 7th intercostal thoracotomy and pararectal laparotomy were performed. After division of the diaphragm, the intrapericardial IVC was controlled. The liver and right kidney were mobilized by incision of the retroperitoneum, helping us to visualize the whole extent of the IVC. After systemic heparinization, cannulation of the right common femoral artery and the femoral vein was carried out following thrombectomy of the right femoral and iliac veins using a Fogarty balloon catheter. The lower IVC was incised and the thrombus was removed manually after initiation of F-F bypass. The retrohepatic IVC with a membranous obstruction was incised, and complete recanalization of the hepatic veins was performed with thrombectomy by suction and partial resection of the liver parenchyma protruding into the IVC. Finally, patch enlargement of the retrohepatic IVC was completed using a pericardial patch (Xenomedica; Baxter Healthcare Corp., Chicago, IL, USA). The F-F bypass was easily discontinued. Angiography on postoperative day 28 revealed good patency of the entire IVC and iliac veins (Figure 2B
). The patient recovered uneventfully without any complications and was discharged on postoperative day 32.
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DISCUSSION
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Budd-Chiari syndrome comprises hepatomegaly, ascites, and portal hypertension due to obstruction of the hepatic vein and/or retrohepatic IVC. The etiology of IVC obstruction is described as congenital hypoplasia of the IVC or an acquired factor, such as thrombophlebitis, malignancy, trauma, inflammation of the abdominal cavity, or polycythemia. Its natural course is progression from liver fibrosis to liver cirrhosis, and it occasionally develops into hepatocellular carcinoma or viral hepatitis.1,2 Budd-Chiari syndrome was classified into 7 types according to the site of occlusion of the IVC or hepatic veins, by Hirooka in 1970.3 This case is representative of type Ib: membrane below obliterated left hepatic vein and above patent right hepatic vein, which rapidly progresses to the entire IVC obstruction on angiography.
In the treatment of BCS, a shunt operation, catheter intervention, and radical operation have been recommended, although a radical operation should be selected from the viewpoint of liver prognosis.4,5 The Senning method, which is one of the radical operations, can recanalize occluded hepatic veins through a median sternotomy under cardiopulmonary bypass, but it cannot recanalize the entire occluded IVC.6 In such cases, the lateral approach described by Koja and colleagues7 should be selected. This approach allows sufficient patch enlargement of the occluded retrohepatic IVC by mobilization of the liver and right kidney through a right spiral incision under F-F bypass. Femorofemoral bypass is very useful to control bleeding after recanalization of the IVC and hepatic veins, and to avoid intermittent occlusion of the portal vein and hepatic artery, such as in the Pringle technique, resulting in the preservation of liver function. In this case, thrombectomy of the lower IVC and iliac veins was also required before establishment of F-F bypass. Partial resection of liver parenchyma can help to completely recanalize the hepatic veins and to sufficiently enlarge the retrohepatic IVC, and these may be the most important considerations in a radical operation for BCS. Liver transplantation was not considered in this case because of the reversible nature of the liver dysfunction.
The following are our concise recommendations for surgical management of BCS with extensive IVC thrombosis. 1. A right lateral skin incision should be employed with mobilization of the liver and kidney to enable an approach to the whole extent of the IVC. 2. After sufficient thrombectomy of the lower IVC and iliac veins, F-F bypass should be established. 3. The use of F-F bypass should stabilize hemodynamics despite massive bleeding. 4. Occluded hepatic veins can be recanalized by partial resection of liver parenchyma. 5. Complete recanalization of hepatic veins will contribute to strong recovery of liver function. Some reports have proposed that hepatocellular carcinoma rarely occurs in BCS with severe liver function.6,8 Nevertheless, careful follow-up should be undertaken with hematological examination and ultrasonography every 3 months.
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REFERENCES
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- Victor S, Jayanthi V, Madanagopalan N. Coarctation of the inferior vena cava. Trop Gastroenterol 1987;8:127–42.[Medline]
- Hirooka M, Kimura C. Membranous obstruction of the hepatic portion of the inferior vena cava. Surgical correction and etiological study. Arch Surg 1970;100:656–63.[Abstract/Free Full Text]
- Cameron JL, Herlong HF, Sanfey H, Boitnott J, Kaufman SL, Gott VL, et al. The Budd-Chiari syndrome. Treatment by mesenteric-systemic venous shunts. Ann Surg 1983;198:335–46.[Medline]
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ABSTRACT
INTRODUCTION
