Hernández Alvarez G, Ayala Landa R, Hernández Adrian G, Benarroch C
Servicio de Gastroenterología y Cirugía del Hospital Carlos J. Bello de la Cruz Roja Venezolana, Caracas.
G E N. 1991 Jan-Mar;45(1):55-60.
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).
EVR是一种罕见的常染色体显性遗传性疾病，通常在脊髓、颅神经和自主神经中出现神经纤维瘤。偶尔，胃肠道中也能发现神经纤维瘤。在这种情况下，最常见的部位是空肠、胃和回肠。很少见的是，它们出现在十二指肠，可能会发生溃疡，导致出血和严重疼痛，类似急性胰腺炎。小网膜中有分别来自内脏神经和迷走神经的交感神经和副交感神经。这会在肝外胆道系统周围形成一个密集的网络，可能是神经纤维瘤的起源；当它们变大时，可能会导致胆道梗阻，需要手术治疗。在此之前，通过所有可用的检查手段（超声检查、逆行胰胆管造影、计算机断层扫描等）排除其他可能导致胆道梗阻的原因（结石、硬化性胆管炎、胆管或胰腺肿瘤）非常重要。
