Congenital portosystemic shunts are embryological malformations in which portal venous flow is diverted to the systemic circulation. High morbidity and mortality are seen in patients with concurrent hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension. Endovascular therapy, in the correct patient population, offers a less invasive method of treatment with rapid relief of symptoms. In this report, we discuss the treatment of a two-year-old male with abnormal chorea-like movements, altered mental status, anisocoria and hyperammonemia diagnosed with an intrahepatic congenital portosystemic shunt between the inferior vena cava and right portal vein. Given the patient’s amenable anatomy and shunt type, embolization was performed with an 18 mm Amplatzer patent foramen ovale occlusion device. Portosystemic shunts are a rare congenital abnormality without universal treatment guidelines. An Amplatzer PFO occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy.

中文翻译：

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先天性门体系统分流闭塞与Amplatzer PFO闭塞装置：一例病例报告

先天性门体系统分流是胚胎畸形，其中门静脉血流转移至体循环。并发肝性脑病，肝肺综合征和肺动脉高压的患者发病率和死亡率较高。在正确的患者人群中，血管内治疗提供了一种侵入性较小的治疗方法，可快速缓解症状。在本报告中，我们讨论了诊断为下腔静脉和右门静脉之间存在肝内先天性门体系统分流的，患有异常舞蹈症样动作，精神状态改变，精神分裂症和高氨血症的两岁男性的治疗方法。根据患者的解剖学和分流类型，用18 mm Amplatzer专利卵圆孔闭塞装置进行栓塞。没有全身治疗指南，门体系统分流是罕见的先天性异常。给定适当的分流类型，大小和解剖结构，Amplatzer PFO闭塞装置可以提供一种新颖的分流闭合方法。