INTRODUCTION Encephalopathy secondary to hyperammonemia due to Congenital Extra-hepatic Porto-systemic shunt (CEPS) in the absence of liver cirrhosis is an exceptionally unusual condition. We describe the case of a 54-year-old woman admitted to the Emergency Department complaining of recurrent episodes of confusion and worsening cognitive impairment. At admission, the patient displayed slowing cognitive-motor skills with marked static ataxia and impaired gait. Hyperammonemia was detected in the serum. An abdominal computed tomography (CT) excluded portal hypertension and liver cirrhosis, detecting a congenital extra-hepatic porto-systemic shunt which is a highly unusual vascular malformation. The patient was treated by interventional radiologists with a successful endovascular closure. AREAS COVERED We have performed a review of the last three decades of the literature, starting from the introduction of CT scanning in common clinical practice. Eighteen studies (case reports) described 29 patients with encephalopathy secondary to hyperammonemia due to CEPS in the absence of liver cirrhosis: They underwent treatment similar to our case report of CEPS. EXPERT COMMENTARY Encephalopathy secondary to hyperammonemia in the absence of hepatic dysfunction is an important diagnostic dilemma to many clinicians. An interventional radiologic approach is currently preferred.

中文翻译：

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血管内闭合治疗成年发作性脑病的非肝硬化肝外门-系统分流术。

引言在没有肝硬化的情况下，先天性肝外门-体分流术（CEPS）导致的高氨血症继发性脑病是一种异常情况。我们描述了一名急诊科住院的54岁妇女的案例，该患者抱怨反复发作的混乱事件和认知障碍加重。入院时，患者表现出缓慢的认知运动技能，并伴有明显的静态共济失调和步态障碍。在血清中检测到高氨血症。腹部计算机断层扫描（CT）排除了门静脉高压症和肝硬化，发现了先天性肝外门体系统分流，这是一种非常不常见的血管畸形。介入放射科医生成功治疗了患者，成功进行了血管内闭合。覆盖的区域我们从最近的临床实践中引入CT扫描开始，对文献的最后三十年进行了回顾。十八项研究（病例报告）描述了29例因CEPS而在没有肝硬化的情况下继发于高氨血症的脑病患者：他们接受的治疗与我们的CEPS病例报告相似。专家评论在没有肝功能障碍的情况下，继发于高氨血症的脑病是许多临床医生的重要诊断难题。目前优选介入放射学方法。他们接受了类似于我们CEPS病例报告的治疗。专家评论在没有肝功能障碍的情况下，继发于高氨血症的脑病是许多临床医生的重要诊断难题。目前优选介入放射学方法。他们接受了类似于我们CEPS病例报告的治疗。专家评论在没有肝功能障碍的情况下，继发于高氨血症的脑病是许多临床医生的重要诊断难题。目前优选介入放射学方法。