Multistage closure of a congenital extrahepatic portosystemic shunt

João Facas; Manuel Cruz; João Filipe Costa; Alfredo Agostinho; Paulo Donato

doi:10.1186/s42155-021-00267-x

Multistage closure of a congenital extrahepatic portosystemic shunt

CVIR Endovasc. 2021 Nov 18;4(1):79. doi: 10.1186/s42155-021-00267-x.

Authors

João Facas¹, Manuel Cruz², João Filipe Costa², Alfredo Agostinho², Paulo Donato^{2

3}

Affiliations

¹ Medical Imaging Department, Coimbra University Hospital, Praceta Prof. Mota Pinto, Coimbra, Portugal. facasmartins@gmail.com.
² Medical Imaging Department, Coimbra University Hospital, Praceta Prof. Mota Pinto, Coimbra, Portugal.
³ Faculty of Medicine of the University of Coimbra, Azinhaga de Santa Comba, Coimbra, Portugal.

Abstract

Background: Congenital extrahepatic portosystemic shunts (CEPS) are rare shunts connecting the extrahepatic portal system with the inferior vena cava. Shunt dimensions and the risk of portal hypertension determines the closure strategy. Endovascular treatment is indicated for single stage occlusion of longer length shunts, whereas the remaining shunt types are preferentially surgically occluded. Herein we describe the technical details of a novel endovascular treatment for short length CEPS.

Case presentation: A 15-years-old male with a short length CEPS complicated with multinodular liver disease was submitted to a multistage closure, as indicated by the high portal pressure values during shunt balloon occlusion venography. Initially a transjugular intrahepatic portosystemic shunt (TIPS) was created and the CEPS occluded with an atrial septal defect occluder. In a second procedure the TIPS was embolized with a flow reductor stent and an amplatzer vascular plug II. At a 1 year follow up the liver nodules size reduced, the patient remains asymptomatic, and the shunt adequately closed.

Conclusion: This paper outlines the potential use of a TIPS and an atrial septal defect occluder combination in complex CEPS, supporting its usage as an alternative to the standard surgical treatment.

Level of evidence: Level 4, Case report.

Keywords: Abernethy; Atrial septal defect occluder; Balloon occlusion venography; Congenital extrahepatic portosystemic shunt; Transjugular intrahepatic portosystemic shunt.