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• W2977499340 abstract "Introduction: Sarcoidosis is a multisystem disease of unknown etiology characterized by the presence of non-caseating granulomas. Portal hypertension is a rare manifestation of sarcoidosis, first reported in 1949. Here we report the first case of extrahepatic sarcoidosis with granulomatous lymph node involvement at the porta hepatis, compression of portal vein leading to portal hypertension and recurrent episodes of gastrointestinal bleeding due to jejunal varices. A 57-year-old black female presented with pruritus, jaundice, elevated liver enzymes, and thrombocytopenia. Diagnosed with ophthalmic and pulmonary sarcoidosis in 1998, a mass at the porta hepatis causing intrahepatic dilation was now seen on CT abdomen. ERCP revealed a stricture at the proximal hepatic duct below the bifurcation, relieved by placing a biliary stent. Diagnostic exploratory laparotomy showed unresectable massive lymphadenopathy, extending from porta hepatis to the inferior aspect of liver, encroaching the vessels and bile ducts. Intrahepatic right bile duct choledoco-Roux-en-Y-jejunostomy was completed. Biopsy showed marked fibrosis and noncaseating granulomas of lymph nodes. Liver biopsy was free of sarcoidosis. Repeat episodes of pruritis and jaundice were treated with high-dose steroids and mycophenolate mofetil. Patient was readmitted with melena and severe anemia. Imaging showed slight decrease in size of the mass but effacement of extrahepatic portal vein, non-visualization of the left portal vein, and severe narrowing of right portal vein. Double balloon enteroscopy revealed large, bleeding perianastomotic jejunal varices. Endoscopic therapy failed and varices were surgically oversawn. Mycophenalate mofetil was restarted along with steroids. A year later recurrent episodes of severe bleeding developed. High-dose pulse steroid therapy was initiated and mycophenalte was increased. Mesocaval shunting was completed with temporary control of bleeding before occlusion. Patient was kept on high-dose steroids, mycophenalate, octreotide and nadolol. Later, CT abdomen revealed reduction in size of mass with partial visualization of the right branch of the portal vein. TIPS was completed via cannulation of the right portal vein, leading to resolution of portal hypertension and further episodes of GI bleed. Extrahepatic portal hypertension in the setting of porta hepatis sarcoidosis can be life threatening and very challenging to treat. Here we described how a combination immunosuppressant and radiologically placed shunt can be used successfully to treat recurrent episodes of GI bleed in such a patient." @default.
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• W2977499340 date "2014-10-01" @default.
• W2977499340 modified "2023-09-23" @default.
• W2977499340 title "A Unique Case of Noncirrhotic Portal Hypertension Secondary to Extrahepatic Sarcoidosis" @default.
• W2977499340 doi "https://doi.org/10.14309/00000434-201410002-00581" @default.
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