[en] Sarcoidotic involvement of the liver is frequent, albeit uncommonly symptomatic. Severe complications are rare, but may seldom require liver transplantation. Budd-Chiari syndrome has been described in a few patients with hepatic sarcoidosis. Herein we have reported the case of a young woman suffering from hepatic sarcoidosis who developed severe cholestasis and chronic Budd-Chiari syndrome. She successfully underwent orthotopic liver transplantation (OLT) and is asymptomatic with normal liver function at 3 years follow-up. Histopathological assessment of the liver explant demonstrated a florid granulomatous process, with involvement of the large intrahepatic veins, providing an anatomical basis for the vascular flow disturbances. This case adds further evidence that liver transplantation may be the curative treatment for complicated sarcoidotic liver disease.
Disciplines :
Gastroenterology & hepatology Surgery
Author, co-author :
Delfosse, Vincent ; Université de Liège - ULiège > Département Argenco : Secteur TLU+C > Lucid - Lab for User Cognition & Innovative Design
De Leval, Laurence ; Centre Hospitalier Universitaire de Liège - CHU > Anatomie pathologique
De Roover, Arnaud ; Centre Hospitalier Universitaire de Liège - CHU > Chirurgie abdominale- endocrinienne et de transplantation
Delwaide, Jean ; Centre Hospitalier Universitaire de Liège - CHU > Gastro-Entérologie-Hépatologie
Honore, Pierre ; Centre Hospitalier Universitaire de Liège - CHU > Chirurgie abdominale- endocrinienne et de transplantation
Boniver, Jacques ; Centre Hospitalier Universitaire de Liège - CHU > Anatomie pathologique
Detry, Olivier ; Centre Hospitalier Universitaire de Liège - CHU > Chirurgie abdominale- endocrinienne et de transplantation
Language :
English
Title :
Budd-Chiari Syndrome Complicating Hepatic Sarcoidosis: Definitive Treatment by Liver Transplantation: A Case Report.
Publication date :
2009
Journal title :
Transplantation Proceedings
ISSN :
0041-1345
eISSN :
1873-2623
Publisher :
Elsevier Science, New York, United States - New York
Iannuzzi M.C., Rybicki B.A., and Teirstein A.S. Sarcoidosis. N Engl J Med 357 (2007) 2153
Ayyala U.S., and Padilla M.L. Diagnosis and treatment of hepatic sarcoidosis. Curr Treat Options Gastroenterol 9 (2006) 475
Detry O., Honore P., Delwaide J., et al. Liver transplantation in a Jehovah's Witness. Lancet 353 (1999) 1680
Detry O., Roover A.D., Delwaide J., et al. Liver transplantation in Jehovah's witnesses. Transpl Int 18 (2005) 929
Lipson E.J., Fiel M.I., Florman S.S., et al. Patient and graft outcomes following liver transplantation for sarcoidosis. Clin Transplant 9 (2005) 487
Cengiz C., Rodriguez-Davalos M., deBoccardo G., et al. Recurrent hepatic sarcoidosis post-liver transplantation manifesting with severe hypercalcemia: a case report and review of the literature. Liver Transpl 11 (2005) 1611
Menon K.V., Shah V., and Kamath P.S. The Budd-Chiari syndrome. N Engl J Med 350 (2004) 578
DüsŞüceli E., Sancak T., Sezgin S., et al. Percutaneous transluminal angioplasty of the inferior right hepatic vein for the treatment of Budd-Chiari syndrome. Acta Gastroenterol Belg 67 (2004) 306
Langlet P., and Valla D. Is surgical portosystemic shunt the treatment of choice in Budd-Chiari syndrome?. Acta Gastroenterol Belg 65 (2002) 155
Nataline M.R., Goyette R.E., Owensby L.C., et al. The Budd-Chiari syndrome in sarcoidosis. JAMA 239 (1978) 2657
Russi E.W., Bansky G., Pfaltz M., et al. Budd-Chiari syndrome in sarcoidosis. Am J Gastroenterol 61 (1986) 71
Deniz K., Ward S.C., Rosen A., et al. Budd-Chiari syndrome in sarcoidosis involving liver. Liver Int 28 (2008) 580
James D.G., and Sherlock S. Sarcoidosis of the liver. Sarcoidosis 11 (1994) 2
