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.
Congenital extrahepatic portosystemic shunts are a rare cause of lower gastrointestinal bleeding in children. They result from the connection of a systemic vessel with the portomesenteric vasculature before the division of the main portal vein. Herein, we report a case of a congenital extrahepatic portosystemic shunts type II in a 4-year-old male diagnosed by Doppler ultrasonography during the investigation of abdominal pain and recurrent hematochezia, later confirmed by computed tomography. Conventional angiography with a balloon occlusion test revealed patent intrahepatic portal branches not depicted by previous imaging techniques. Successful shunt closure was achieved by endovascular approach with an Amplatzer Septal Occluder without complications.
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