Shnayder Michelle M, Dervishi Mario, Jo Alexandria, Pomerantz Benjamin
Department of Radiology; Division of Vascular and Interventional Radiology, University of Michigan Health System, 1500 E. Medical Center Dr, B1D502, 48109-5030, Ann Arbor, MI, USA.
American University of the Caribbean School of Medicine, Cupecoy, St. Maarten.
CVIR Endovasc. 2021 Jan 11;4(1):14. doi: 10.1186/s42155-021-00204-y.
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.
先天性门体分流是一种胚胎发育畸形，其中门静脉血流被分流至体循环。并发肝性脑病、肝肺综合征和肺动脉高压的患者具有较高的发病率和死亡率。对于合适的患者群体，血管内治疗提供了一种侵入性较小的治疗方法，可迅速缓解症状。
在本报告中，我们讨论了一名两岁男性患者的治疗情况，该患者出现异常的舞蹈样动作、精神状态改变、瞳孔不等大和高氨血症，诊断为肝内先天性下腔静脉与右门静脉之间的门体分流。鉴于患者合适的解剖结构和分流类型，使用18毫米的Amplatzer卵圆孔未闭封堵器进行了栓塞治疗。
门体分流是一种罕见的先天性异常，没有通用的治疗指南。对于合适的分流类型、大小和解剖结构，Amplatzer卵圆孔未闭封堵器可提供一种新型的分流闭合方法。
