Cinematic Rendering of Abernethy Malformation with Abdominal Aneurysms

HomeRadiologyVol. 310, No. 2 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyCinematic Rendering of Abernethy Malformation with Abdominal AneurysmsZheng Ye, Zhenlin Li Zheng Ye, Zhenlin Li Author AffiliationsFrom the Department of Radiology, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu 610041, China.Address correspondence to Z.L. (email: [email protected]).Zheng YeZhenlin Li Published Online:Feb 6 2024https://doi.org/10.1148/radiol.232202MoreSectionsPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookXLinked In A 48-year-old woman with history of recurrent cough and pulmonary hypertension of unclear origin was referred to West China Hospital and underwent CT angiography. Chest CT angiography showed that the diameter of the main pulmonary artery was enlarged to approximately 4 cm. Abdominal CT angiography with cinematic rendering revealed the absence of the portal venous trunk and its intrahepatic branches and an enlarged superior mesenteric vein and tortuous splenic vein, which drained directly into the inferior vena cava. Above the level of the renal hilum, the inferior vena cava appeared diminutive, and its hepatic segment was almost invisible, which was accompanied by a compensatory dilatation of the azygos vein. The celiac trunk and common hepatic artery were severely enlarged and showed multiple abdominal aneurysms (Figure).Images in a 48-year-old woman who was diagnosed with Abernethy malformation, also known as congenital extrahepatic portosystemic shunts. (A) Axial CT angiography image and (B) multiplanar reconstruction show portomesenteric shunts, which bypass the liver and drain into the inferior vena cava (IVC), with an enlarged superior mesenteric vein (SMV) and tortuous splenic vein (SV). (C) Cinematic rendering of the arterial phase demonstrates aneurysmal dilatation in the common hepatic artery (CHA), splenic artery (SA), and superior mesenteric artery (SMA). (D) Cinematic rendering of the venous phase depicts the confluence of the superior mesenteric vein and splenic vein, as well as the compensatory enlargement of the azygos vein (AV).Download as PowerPointThe findings were consistent with Abernethy malformation (1), also known as congenital extrahepatic portosystemic shunts, a rare vascular anomaly in which portal blood bypasses the liver and drains into a systemic vein, most frequently the inferior vena cava. In addition, Abernethy malformation is often associated with congenital and acquired abnormalities (2), such as abdominal aneurysms and pulmonary hypertension. Cinematic rendering can provide clear depiction of anatomic variations and guide treatment strategies.Disclosures of conflicts of interest: Z.Y. No relevant relationships. Z.L. No relevant relationships.Supported by the China Postdoctoral Science Foundation (grant no. 2023M732464).References1. Kumar P, Bhatia M, Garg A, Jain S, Kumar K. Abernethy malformation: a comprehensive review. Diagn Interv Radiol 2022;28(1):21–28. Crossref, Medline, Google Scholar2. Agarwal A, Bhangale D, Sawardekar VM, et al. Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature. Egypt J Intern Med 2021;33(1):1–5. Crossref, Google ScholarArticle HistoryReceived: Aug 23 2023Revision requested: Sept 29 2023Revision received: Sept 30 2023Accepted: Oct 19 2023Published online: Feb 06 2024 FiguresReferencesRelatedDetailsRecommended Articles Interventional Radiology Management of Adult Liver Transplant ComplicationsRadioGraphics2022Volume: 42Issue: 6pp. 1705-1723Vascular Anomalies of the Pediatric LiverRadioGraphics2019Volume: 39Issue: 3pp. 842-856Tumor-Vessel Relationships in Pancreatic Ductal Adenocarcinoma at Multidetector CT: Different Classification Systems and Their Influence on Treatment PlanningRadioGraphics2016Volume: 37Issue: 1pp. 93-112A Comprehensive Approach to Hepatic Vascular DiseaseRadioGraphics2017Volume: 37Issue: 3pp. 813-836Right Upper Quadrant US Basics: Anatomy, Imaging Techniques, and Differential DiagnosesRadioGraphics2024Volume: 44Issue: 2See More RSNA Education Exhibits Do Not Forget the Pipes: Inferior Vena Cava: Abnormal Finding and Their Imaging AppearanceDigital Posters2022Tortuous Path: Congenital Anomalies of the Systemic Thoracic VeinsDigital Posters2022Beyond Portal Vein: The Intra-abdominal Radiologic Venous Anatomy And Its Applicability In The Everyday Routine - Whats Every Radiologist Must Know.Digital Posters2021 RSNA Case Collection Partial anomalous pulmonary venous returnRSNA Case Collection2021Right upper PAPVR and persistent left SVCRSNA Case Collection2020Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu syndrome)RSNA Case Collection2020 Vol. 310, No. 2 Metrics Altmetric Score PDF download