Department of Internal Medicine, Roy J and Lucille A Carver College of Medicine, University of Iowa and Veterans Administration Medical Center, Iowa City, IA 52242, USA.
J Clin Gastroenterol. 2010 May-Jun;44(5):381-3. doi: 10.1097/MCG.0b013e3181aae51b.
Hepatic encephalopathy most commonly occurs in patients with cirrhosis and end-stage liver disease, however, the disorder can also occur in the presence of intrahepatic or extrahepatic shunts when the intrahepatic circulation is effectively bypassed. The majority of extrahepatic shunts described to date develop between a mesenteric vein and inferior vena cava. Herein we report a novel case of a superior mesenteric vein to left internal iliac vein shunt that led to hepatic encephalopathy in a 57-year-old woman with no apparent underlying liver disorder. The patient presented with confusion, disorientation, and hyperammonemia. Workup for parenchymal liver disease was negative and liver biopsy findings did not show significant liver disease. Magnetic resonance imaging revealed a serpiginous 1-cm-wide shunt that diverted superior mesenteric vein blood from the portal confluence to the left internal iliac vein. Surgical closure of the shunt led to marked improvement of the patient with the resolution of hepatic encephalopathy. This report is the first description of a portosystemic shunt, likely congenital, linking these 2 vessels resulting in clinically significant hepatic encephalopathy. The findings emphasize that abdominal and pelvic imaging should be considered in patients with signs of hepatic encephalopathy that have none to minimal hepatic disease.
肝性脑病最常发生于肝硬化和终末期肝病患者，但当肝内循环被有效旁路时，肝内或肝外分流也可发生该疾病。迄今为止描述的大多数肝外分流发生在肠系膜静脉和下腔静脉之间。在此，我们报告了一例新的肠系膜上静脉至左侧髂内静脉分流病例，该分流导致一名 57 岁女性发生肝性脑病，而该女性无明显潜在肝脏疾病。患者表现为意识模糊、定向障碍和血氨升高。针对实质性肝病的检查结果为阴性，肝活检结果未显示明显的肝病。磁共振成像显示一条蜿蜒 1 厘米宽的分流，将肠系膜上静脉血液从门静脉汇流引导至左侧髂内静脉。分流的手术闭合导致患者明显改善，肝性脑病得到缓解。本报告首次描述了一种可能为先天性的连接这两条血管的门体分流，导致临床上显著的肝性脑病。这些发现强调，对于无明显或最小肝脏疾病但有肝性脑病迹象的患者，应考虑进行腹部和盆腔影像学检查。
