[Biliary obstruction in von Recklinghausen disease]

The EVR is an uncommon autosomic dominant hereditary pathology that typically presents neurofibromas in the course of the spinal, cranial and neurovegetative nerves. Occasionally, neurofibromas can be found in the gastrointestinal tract. In this setting, the most frequent locations are jejunum, stomach and ileum. Seldom, they are in duodenum where could become ulcerated determining bleeding and severe pain simulating an acute pancreatitis. There are sympathetic and parasympathetic nerves in the minor epiploon arising from the splanchnic and vagus respectively. It results in a thick network around the extrahepatic biliary system that could be the neurofibromas origin; when they become bigger biliary obstruction can results needing chirurgical treatment. Previously, it is important to discard another probable causes of biliary obstruction (lithiasis, sclerosant cholangitis, biliary or pancreatic neoplasms) with all available proceedings (echosonography, CPRE, TAC, etc).