Budd-Chiari Syndrome Complicating Hepatic Sarcoidosis: Definitive Treatment by Liver Transplantation: A Case Report V. Delfosse, L. de Leval, A. De Roover, J. Delwaide, P. Honoré, J. Boniver, and O. Detry 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 ortho- topic 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. S ARCOIDOSIS IS A systemic granulomatous disease of unknown etiology. 1 Hepatic involvement is common, albeit most often asymptomatic in sarcoidosis. Sarcoidotic hepatic involvement is usually treated medically. A small minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may rarely require liver transplantation. 2 Only a few cases of Budd-Chiari syndrome (BCS) have been described so far to be due to hepatic sarcoidosis. In this report, we have described the case of a woman suffering from hepatic sarcoidosis, who developed end-stage liver failure with severe cholestasis and chronic BCS, and who underwent successful liver transplantation. CASE REPORT A 45-year-old female Jehovah’s Witness was referred to our liver unit for refractory cholestasis due to hepatic sarcoidosis compli- cated by BCS. Her past medical history revealed diabetes mellitus, minor -thalassemia, hypertension, and erythema nodosum. She had a 5-year history of systemic sarcoidosis, diagnosed in 2000 after an episode of sarcoidotic uveitis. In 2001, she developed a decrease in pulmonary function; bronchial biopsies showed multiple epithe- lioid granulomas. Abnormal liver blood tests and cholestasis were observed in 2004, at which time hepatitis B and C virus serologies were negative. The patient complained of asthenia and weight loss. Liver tests demonstrated severe hyperbilirubinemia (total bilirubin up to 409 mg/L; normal = 2.3–10 mg/L) with transaminase 3-fold above normal values and increased cholestatic enzymes. Liver synthetic function was normal as confirmed by international normalized ratio (INR). Computed tomography (CT) of the chest demonstrated multiple mediastinal and peritracheal adenopathies, and multiple interstitial pulmonary nodules (Fig 1) with preserved global pul- monary function. Abdominal CT showed multiple hepatic and splenic parenchymal micronodules, and mesenteric adenopathies. Nonhomogeneous perfusion of the liver was noticed. The supra- hepatic veins were not demonstrated (Fig 2). Collateral circulation had developed in the left gastric vein. Prominent portosystemic collateral circulation was observed with spontaneous portocaval and arterioportal shunts, but no significant splenomegaly or ascites. Chronic compensated BCS syndrome was confirmed; no other cause for BCS was demonstrated. Successful cadaveric orthotopic liver transplantation (OLT) was performed in December 2005, without the use of any blood product, according to our protocol for liver transplantation in Jehovah’s Witness patients. 3,4 Postoperative evolution after OLT was rapidly favorable; liver graft function was immediate. At 3 years follow-up, the patient enjoyed a normal life with normal liver function. In particular, there was no suspicion of sarcoidosis recurrence in the liver graft. From the Departments of Pathology (V.D., L.d.L., J.B.), Ab- dominal Surgery and Transplantation (A.D., P.H., O.D.), and Hepatogastroenterology (J.D.), CHU de Liège, University of Liège, Liège, Belgium. Address reprint requests to Olivier Detry, MD, PhD, Depart- ment of Abdominal Surgery and Transplantation, CHU de Liège, Sart Tilman B35, B4000 Liège, Belgium. E-mail: Oli.Detry@ chu.ulg.ac.be 0041-1345/09/$–see front matter © 2009 by Elsevier Inc. All rights reserved. doi:10.1016/j.transproceed.2009.09.021 360 Park Avenue South, New York, NY 10010-1710 3432 Transplantation Proceedings, 41, 3432–3434 (2009)