Budd-Chiari syndrome (BCS) is a rare and often fatal disorder characterized by hepatic venous outflow obstruction. The occlusion is most often caused by thrombosis of the major hepatic veins and occasionally involves the inferior vena cava (IVC). This syndrome is associated with a variety of intraabdominal as well as systemic disorders. This report presents 2 cases of BCS in which both patients had polycythemia vera and 1 had a mildly decreased level of antithrombin III. A side-to-side portacaval shunt was performed in 1 patient and a mesoatrial shunt in the other owing to concomitant obstruction of the IVC. Dramatic improvement in the status of the liver was demonstrated by a follow-up biopsy in the latter patient.
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References
1.
1. Budd G: On Diseases of the Liver, ed 1, London: John Churchill, 1845.
2.
2. Bayraktar Y, Balkanci F, Kansu E, et al: Budd-Chiari syndrome: Analysis of 30 cases. Angiology44:541-551, 1993.
3.
3. Satoh M, Katoh J, Onodera S: Leiomyosarcoma of the inferior vena cava causing Budd-Chiari syndrome -a case report. Angiology44:673-676, 1993.
4.
4. Sanfey H, Boitnott JK, Cameron JL: Surgical management of patients with the Budd-Chiari syndrome. World J Surg8:706-715, 1984.
5.
5. Hirooka M, Kimura C: Membranous obstruction of the hepatic portion of the inferior vena. Arch Surg100:656-663, 1970.
6.
6. Kimura C, Shirotani H, Hirooka M, et al: Membranous obliteration of the inferior vena cava in the hepatic portion: Review of 6 cases with 3 autopsies. J Cardiac Surg4:87-98, 1963.
7.
7. Orloff MJ, Girard B: Long term results of treatment of Budd-Chiari syndrome by side-to-side portacaval shunt. Surg Gynecol Obstet168:33-41, 1989.
8.
8. Bismuth H, Sherlock DJ: Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg214:581-589, 1991.
9.
9. Scharschmidt BF: Human liver transplantation: Analysis of data on 540 patients from four centers. Hepatology4:955-1015, 1984.
10.
10. Lois JF, Hartzman S, McGlade CT, et al: Budd-Chiari syndrome: Treatment with percutaneous transhepatic recanalization and dilation. Radiology170:791-793, 1989.
11.
11. Sparano J, Chang J, Trasl S, et al: Treatment of the Budd-Chiari syndrome with percutaneous transluminal angioplasty. Am J Med82:821-828, 1987.
12.
12. Klein AS, Cameron JL: Diagnosis and management of the Budd-Chiari syndrome. Am J Surg160:128-133, 1990.
13.
13. Ahn SS, Yellin A, Sheng FC, et al: Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management. J Vasc Surg5:28-37, 1987.
15. Orloff MJ, Johnansen KH: Treatment of Budd-Chiari syndrome by side-to-side portacaval shunt: Experimental and clinical results. Ann Surg188:494-512, 1978.
16.
16. Cameron JL, Herlong HF, Sanfey H, et al: The Budd-Chiari syndrome: Treatment by mesenteric-systemic venous shunts. Ann Surg198:335-346, 1983.
17.
17. McCarthy PM, van Heerden JA, Adson MA, et al: The Budd-Chiari syndrome: Medical and surgical management of 30 patients. Arch Surg120:657-662, 1985.
18.
18. Cameron JL, Maddrey WC: Mesoatrial shunt: A new treatment for the Budd-Chiari syndrome. Ann Surg187:402-406, 1978.
19.
19. Klein AS, Sitzmann JV, Coleman J, et al: Current management of the Budd-Chiari syndrome. Ann Surg212:144-149, 1990.
20.
20. Henderson MJ, Warren WD, Millikan WJ Jr, et al: Surgical options, hematologic evaluation, and pathologic changes in Budd-Chiari syndrome. Am J Surg159:41-50, 1990.
21.
21. Orloff MJ, Daily PO, Girard B: Treatment of Budd-Chiari syndrome due to inferior vena cava occlusion by combined portal and vena caval decompression. Am J Surg163:137-143, 1992.
23. Campbell DA, Rolles K, Jamieson N, et al: Hepatic transplantation with perioperative and long term anticoagulation as treatment for Budd-Chiari syndrome. Surg Gynecol Obstet166:511-517, 1988.
