Disappearance of recurrent pancreatitis after splenectomy in familial chylomicronemia syndrome

Recurrent pancreatitis is a severe complication of familial chylomicronemia syndrome (FCS) mainly secondary to lipoprotein lipase deficiency. The mechanism and interindividual variability of pancreatitis in FCS are not fully understood, but abnormalities in the drainage system of pancreatic veins could be involved. Two cases of typical FCS are described with a past history of recurrent pancreatitis that dramatically improved after splenectomy performed in both cases for reasons non-related to FCS. These are the first reports of the disappearance of pancreatitis after splenectomy in FCS and they should be considered of anecdotal nature at this time. The disappearance of pancreatitis following splenectomy could be in part due to subsequent improvements in pancreatic drainage. Extrahepatic portal hypertension induced by hypertriglyceridemic splenomegaly leading to pancreatic congestion could also be a contributing factor. •Pancreatitis is a frequent and serious complication in familial chylomicronemia.•We present two cases in which recurrent pancreatitis disappeared after splenectomy.•Extrahepatic portal hypertension favors the development of pancreatitis.•Improvement of portal drainage could explain this favorable evolution.