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. 1990 Aug;212(2):144–149. doi: 10.1097/00000658-199008000-00005

Current management of the Budd-Chiari syndrome.

A S Klein 1, J V Sitzmann 1, J Coleman 1, F H Herlong 1, J L Cameron 1
PMCID: PMC1358048  PMID: 2375646

Abstract

Twenty-six patients with the Budd-Chiari syndrome were treated surgically at the Johns Hopkins Hospital. Twenty-one of the patients were female and five were male, with a median age at diagnosis of 37 years. Nine patients had polycythemia vera, 6 were receiving estrogen therapy, 5 had a previous hepatitis A or B infection, and 4 had cirrhosis. There was one case each of hepatic malignancy, paroxysmal nocturnal hemoglobinuria, and idiopathic thrombocytopenic purpura. In five cases no etiologic factors or associated disorders were identified. Ascites was the most common presenting feature in this group of patients. Hepatic function at the time of diagnosis, as measured by standard serum chemistries, was only minimally abnormal. The diagnosis of the Budd-Chiari syndrome was confirmed in all 26 patients by hepatic vein catheterization. Inferior vena cavography was also performed and revealed caval occlusion in 4 patients, significant caval obstruction in 13 patients, and a normal vena cava in 9 patients. Interpretation of the vena cavogram was helpful in selecting the appropriate surgical procedure for each patient. Twenty-three of the twenty-six patients underwent percutaneous liver biopsy before operation, with no morbidity or mortality. Four patients had well-established cirrhosis noted on biopsy. Thirty mesenteric-systemic venous shunts were performed on the 26 patients. In 11 patients a mesocaval shunt was performed and in one instance conversion to a mesoatrial shunt was required as a second procedure. In 15 patients a mesoatrial shunt was performed as the initial procedure. Graft thrombosis occurring in 2 of these 15 patients prompted one revision in 1 patient and 2 revisions in the second patient. After mesenteric-systemic venous shunt, eight of the patients (31%) died before discharge from the hospital. The remaining 18 patients in this series were discharged from the hospital alive and well with patent shunts. Patients were followed for a median of 43 months (range, 9 months to 13 years). Five late deaths occurred between 5 and 84 months after the operation. Three- and five-year actuarial survival rates were 65% and 59%, respectively.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ahn S. S., Goldstein L. I., Busuttil R. W. Simultaneous retrohepatic inferior vena cavoplasty and side-to-side portacaval shunt for recurrent thrombosed mesoatrial shunt in the Budd-Chiari syndrome. Surgery. 1987 Feb;101(2):165–171. [PubMed] [Google Scholar]
  2. Akita H., Sakoda K. Portopulmonary shunt by splenopneumopexy as a surgical treatment of Budd-Chiari syndrome. Surgery. 1980 Jan;87(1):85–94. [PubMed] [Google Scholar]
  3. Brems J. J., Hiatt J. R., Klein A. S., Millis J. M., Colonna J. O., Quinones-Baldrich W. J., Ramming K. P., Busuttil R. W. Effect of a prior portasystemic shunt on subsequent liver transplantation. Ann Surg. 1989 Jan;209(1):51–56. doi: 10.1097/00000658-198901000-00008. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Cameron J. L., Harrington D. P., Maddrey W. C. The mesocaval C shunt. Surg Gynecol Obstet. 1980 Mar;150(3):401–403. [PubMed] [Google Scholar]
  5. Cameron J. L., Herlong H. F., Sanfey H., Boitnott J., Kaufman S. L., Gott V. L., Maddrey W. C. The Budd-Chiari syndrome. Treatment by mesenteric-systemic venous shunts. Ann Surg. 1983 Sep;198(3):335–346. doi: 10.1097/00000658-198309000-00010. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Cameron J. L., Kadir S., Pierce W. S. Mesoatrial shunt: a prosthesis modification. Surgery. 1984 Jul;96(1):114–116. [PubMed] [Google Scholar]
  7. Cameron J. L., Maddrey W. C. Mesoatrial shunt: a new treatment for the Budd-Chiari syndrome. Ann Surg. 1978 Apr;187(4):402–406. doi: 10.1097/00000658-197804000-00010. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Fishman E. K., Siegelman S. S., Cameron J. L. The evaluation of mesoatrial shunt patency with computed tomography. Surgery. 1985 Jan;97(1):60–65. [PubMed] [Google Scholar]
  9. Huguet C., Liegeois A., Levy V. G., Caroli J. Interposition mesocaval shunt for chronic primary occlusion of the hepatic veins. Surg Gynecol Obstet. 1979 May;148(5):691–698. [PubMed] [Google Scholar]
  10. McCarthy P. M., van Heerden J. A., Adson M. A., Schafer L. W., Wiesner R. H. The Budd-Chiari syndrome. Medical and surgical management of 30 patients. Arch Surg. 1985 Jun;120(6):657–662. doi: 10.1001/archsurg.1985.01390300007001. [DOI] [PubMed] [Google Scholar]
  11. McDermott W. V., Stone M. D., Bothe A., Jr, Trey C. Budd-Chiari syndrome. Historical and clinical review with an analysis of surgical corrective procedures. Am J Surg. 1984 Apr;147(4):463–467. doi: 10.1016/0002-9610(84)90006-0. [DOI] [PubMed] [Google Scholar]
  12. Nagasue N., Ogawa Y., Yukaya H., Chang Y. C., Kaneko S. Cavo-atrial shunt with externally stented PTFE graft for Budd-Chiari syndrome with caval obstruction. Br J Surg. 1987 Dec;74(12):1177–1178. doi: 10.1002/bjs.1800741230. [DOI] [PubMed] [Google Scholar]
  13. Orloff M. J., Johansen K. H. Treatment of Budd-Chiari syndrome by side-to-side portacaval shunt: experimental and clinical results. Ann Surg. 1978 Oct;188(4):494–512. doi: 10.1097/00000658-197810000-00007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Putnam C. W., Porter K. A., Weil R., 3rd, Reid H. A., Starzl T. E. Liver transplantation of Budd-Chiari syndrome. JAMA. 1976 Sep 6;236(10):1142–1143. [PMC free article] [PubMed] [Google Scholar]
  15. Saubier E. C., Braulta A., Partensky C. Splenoazygos shunt for stenosis of the suprahepatic inferior vena cava with cirrhosis. Secondary Budd-Chiari syndrome. Int Surg. 1974 Oct;59(10):526–532. [PubMed] [Google Scholar]
  16. Stringer M. D., Howard E. R., Green D. W., Karani J., Gimson A. S., Williams R. Mesoatrial shunt: a surgical option in the management of the Budd-Chiari syndrome. Br J Surg. 1989 May;76(5):474–478. doi: 10.1002/bjs.1800760516. [DOI] [PubMed] [Google Scholar]
  17. Tavill A. S., Wood E. J., Kreel L., Jones E. A., Gregory M., Sherlock S. The Budd-Chiari syndrome: correlation between hepatic scintigraphy and the clinical, radiological, and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology. 1975 Mar;68(3):509–518. [PubMed] [Google Scholar]
  18. Vons C., Smadja C., Bourstyn E., Szekely A. M., Bonnet P., Franco D. Results of portal systemic shunts in Budd-Chiari syndrome. Ann Surg. 1986 Apr;203(4):366–370. doi: 10.1097/00000658-198604000-00005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Wang Z. G., Zhu Y., Wang S. H., Pu L. P., Du Y. H., Zhang H., Yuan C., Chen Z., Wei M. L., Pu L. Q. Recognition and management of Budd-Chiari syndrome: report of one hundred cases. J Vasc Surg. 1989 Aug;10(2):149–156. [PubMed] [Google Scholar]

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