BACKGROUND/AIMS: Non-cirrhotic nodular liver may occasionally be associated with portal hypertension, with complications such as ascites, bleeding esophageal varices and sometimes hepatic encephalopathy. Four patients treated with transplantation are hereby described. METHODOLOGY: All 4 patients had orthotopic transplantation. Cyclosporine based immunosuppression was used in all patients. RESULTS: The first patient had nodular regenerative hyperplasia in the resected native liver and in the graft seven years after the transplant, what could be considered as recurrence of the disease. There were two more cases of nodular regenerative hyperplasia; one of them was diagnosed 9 years before transplantation, but he developed progressive liver failure. The fourth patient had chronic renal failure and a nodular liver. His general condition and fear of untreatable ascites after a portosystemic shunt, prompted us to perform a liver-kidney transplantation. CONCLUSIONS: Portal hypertension associated with non-cirrhotic nodular liver disease can be, in general, successfully treated with medical and endoscopic measures, or in certain more serious cases with portosystemic shunting. Nevertheless, there are certain cases where liver insufficiency, associated diseases or the unawareness of the pathological diagnosis determines a liver transplantation.
BACKGROUND/AIMS: Non-cirrhotic nodular liver may occasionally be associated with portal hypertension, with complications such as ascites, bleeding esophageal varices and sometimes hepatic encephalopathy. Four patients treated with transplantation are hereby described. METHODOLOGY: All 4 patients had orthotopic transplantation. Cyclosporine based immunosuppression was used in all patients. RESULTS: The first patient had nodular regenerative hyperplasia in the resected native liver and in the graft seven years after the transplant, what could be considered as recurrence of the disease. There were two more cases of nodular regenerative hyperplasia; one of them was diagnosed 9 years before transplantation, but he developed progressive liver failure. The fourth patient had chronic renal failure and a nodular liver. His general condition and fear of untreatable ascites after a portosystemic shunt, prompted us to perform a liver-kidney transplantation. CONCLUSIONS: Portal hypertension associated with non-cirrhotic nodular liver disease can be, in general, successfully treated with medical and endoscopic measures, or in certain more serious cases with portosystemic shunting. Nevertheless, there are certain cases where liver insufficiency, associated diseases or the unawareness of the pathological diagnosis determines a liver transplantation.
Authors: Berrie Meijer; Melek Simsek; Hans Blokzijl; Robert A de Man; Minneke J Coenraad; Gerard Dijkstra; Carin Mj van Nieuwkerk; Chris Jj Mulder; Nanne Kh de Boer Journal: United European Gastroenterol J Date: 2016-11-16 Impact factor: 4.623