Budd-Chiari Syndrome Complicating Hepatic Sarcoidosis: Definitive Treatment by Liver Transplantation: A Case Report

• Published in: Transplantation proceedings Vol. 41; no. 8; pp. 3432 - 3434
• Main Authors: Delfosse, V, de Leval, L, De Roover, A, Delwaide, J, Honoré, P, Boniver, J, Detry, O
• Format: Journal Article Conference Proceeding Web Resource
• Language: English
• Published: Amsterdam Elsevier Inc 01.10.2009; Elsevier; Elsevier Science
• Subjects: Biological and medical sciences; Budd-Chiari Syndrome - complications; Budd-Chiari Syndrome - diagnostic imaging; Budd-Chiari Syndrome - surgery; Chirurgie; Female; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Gastroenterology & hepatology; Gastroenterology. Liver. Pancreas. Abdomen; Gastroentérologie & hépatologie; Hepatic Veins - diagnostic imaging; Hepatic Veins - pathology; Human health sciences; Humans; Hyperbilirubinemia - complications; Liver Diseases - complications; Liver Diseases - pathology; Liver Diseases - surgery; Liver Transplantation; Liver, biliary tract, pancreas, portal circulation, spleen; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Lung - diagnostic imaging; Lung - pathology; Medical sciences; Middle Aged; Other diseases. Semiology; Sarcoidosis - complications; Sarcoidosis - surgery; Sciences de la santé humaine; Surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the digestive system; Tissue, organ and graft immunology; Tomography, X-Ray Computed; Treatment Outcome; Weight Loss; Human; Portal circulation disease; Sarcoidosis; Digestive system; Liver; Cardiovascular disease; Homotransplantation; Venous disease; Vascular disease; Case study; Medicine; Budd-Chiari syndrome; Treatment; Surgery; Systemic disease; Digestive diseases; Complication; Graft; Liver transplantation
• ISSN: 0041-1345; 1873-2623; 1873-2623
• DOI: 10.1016/j.transproceed.2009.09.021

Abstract 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.