Victoria Marco-Benedí1, Itziar Lamiquiz-Moneo1, Luis A Álvarez-Sala2, Fernando Civeira3. 1. Lipid Clinic, Hospital Universitario Miguel Servet, IIS Aragón, CIBERCV, Zaragoza, Spain. 2. Lipid Clinic, Hospital Universitario Gregorio Marañón, IIS Gregorio Marañón (IiSGM), Departmento de Medicina. School of Medicine. Universidad Complutense de Madrid, Madrid, Spain. 3. Lipid Clinic, Hospital Universitario Miguel Servet, IIS Aragón, CIBERCV, Zaragoza, Spain; Universidad de Zaragoza, Zaragoza, Spain. Electronic address: civeira@unizar.es.
Abstract
BACKGROUND AND AIMS: 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. METHODS AND RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND AND AIMS: 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. METHODS AND RESULTS: 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. CONCLUSIONS: 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.