Malik Asad, Patel Muhammed Ebrahim, Ganger Daniel, Hohlastos Elias, Riaz Ahsun
Division of Interventional Radiology, Department of Radiology, Northwestern University, 676 N. St. Clair, Suite 800, Chicago, IL, USA.
Department of Medicine, Section of Gastroenterology/Hepatology, Northwestern University, Chicago, IL, USA.
Radiol Case Rep. 2023 Jun 25;18(9):3135-3139. doi: 10.1016/j.radcr.2023.06.019. eCollection 2023 Sep.
Abernethy malformation (congenital extrahepatic portosystemic shunt [CEPS]) is rare and is characterized by an aberrant connection between the portal and systemic veins, bypassing the liver. It can have varying presentations and can lead to severe complications if left untreated. It is usually diagnosed incidentally on abdominal imaging. Occlusion venography and measurement of portal pressures (pre- and postocclusion) is an important step in management. Complete occlusion of the malformation in cases where the portal veins in the liver are very small and the gradient is more than 10 mm Hg, can potentially lead to acute portal hypertensive complications, such as porto-mesenteric thrombosis. We report a case of Abernethy malformation diagnosed on an abdominal computed tomography scan that presented with neurological symptoms and was successfully managed by interventional radiology via endovascular closure through placement and sequential occlusion of 2 metal stents.
阿伯内西畸形（先天性肝外门体分流[CEPS]）较为罕见，其特征是门静脉与体静脉之间存在异常连接，绕过肝脏。它可有多种表现形式，若不治疗可导致严重并发症。通常在腹部影像学检查时偶然发现。闭塞性静脉造影及门静脉压力（闭塞前后）测量是治疗的重要步骤。在肝脏门静脉非常小且压力梯度超过10毫米汞柱的情况下，完全闭塞畸形可能会导致急性门静脉高压并发症，如门静脉肠系膜血栓形成。我们报告1例经腹部计算机断层扫描诊断为阿伯内西畸形的病例，该病例出现神经症状，并通过介入放射学经血管内封闭成功治疗，方法是放置并依次闭塞2枚金属支架。
