scholarly journals A case of two shunts in the endovascular treatment of type II Abernethy syndrome

2022 ◽  
Vol 5 (1) ◽  
Author(s):  
Brenden Bombardier ◽  
Adam Alli ◽  
Aaron Rohr ◽  
Zachary Collins ◽  
Kavi Raval
Keyword(s):  
Blood Flow ◽  
Portal Vein ◽  
Endovascular Treatment ◽  
Rare Condition ◽  
Portal System ◽  
Type Ii ◽  
Abernethy Malformation ◽  
Complete Occlusion ◽  
Post Procedure ◽  
Portosystemic Shunts

Abstract Background Abernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population. Case presentation We report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7 mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT. Conclusions This report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.

scholarly journals A Case of Two Shunts In The Endovascular Treatment of Type II Abernethy Syndrome

2021 ◽  
Author(s):  
Brenden Bombardier ◽  
Adam Alli ◽  
Aaron Rohr ◽  
Zachary Collins ◽  
Kavi Raval
Keyword(s):  
Blood Flow ◽  
Portal Vein ◽  
Endovascular Treatment ◽  
Rare Condition ◽  
Portal System ◽  
Type Ii ◽  
Abernethy Malformation ◽  
Complete Occlusion ◽  
Post Procedure ◽  
Portosystemic Shunts

Abstract Background:Abernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population.Case Presentation:We report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT.Conclusions:This report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.

Evolution of portal hypertension and mechanisms involved in its maintenance in a rat model

1985 ◽  
Vol 248 (6) ◽  
pp. G618-G625 ◽  
Author(s):  
E. Sikuler ◽  
D. Kravetz ◽  
R. J. Groszmann
Keyword(s):  
Blood Flow ◽  
Portal Hypertension ◽  
Portal Vein ◽  
Rat Model ◽  
Portal Pressure ◽  
Portal System ◽  
Hypertensive Rats ◽  
Hemodynamic Changes ◽  
Sequence Of Events ◽  
Radioactive Microsphere

In rats with portal hypertension induced by partial ligation of the portal vein, we have recently demonstrated an increased portal venous inflow that becomes an important factor in the maintenance of portal hypertension. The sequence of events that leads into this circulatory disarray is unknown. We evaluated chronologically the chain of hemodynamic changes that occurred after portal hypertension was induced by partial ligation of the portal vein. In this model it is possible to follow, from the initiation of the portal-hypertensive state, the interaction between blood flow and resistance in the portal system as well as the relation between the development of portal-systemic shunting and the elevated portal venous inflow. The study was performed in 45 portal-hypertensive rats and in 29 sham-operated rats. Blood flow and portal-systemic shunting were measured by radioactive microsphere techniques. The constriction of the portal vein was immediately followed by a resistance-induced portal hypertension characterized by increased portal resistance (9.78 +/- 0.89 vs. 4.18 +/- 0.71 dyn X s X cm-5 X 10(4), mean +/- SE, P less than 0.01), increased portal pressure (17.7 +/- 0.9 vs. 9.5 +/- 0.6 mmHg, P less than 0.001), and decreased portal venous inflow (3.93 +/- 0.26 vs. 6.82 +/- 0.49 ml X min-1 X 100 g body wt-1, P less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Rex Shunt as a Treatment of Type II Abernethy Malformation with Severe Dysplasia of Portal Vein

2018 ◽  
Vol 53 ◽  
pp. 268.e1-268.e6 ◽  
Author(s):  
Jin-Shan Zhang ◽  
Long Li ◽  
Wei Cheng ◽  
Wen-Ying Hou
Keyword(s):  
Portal Vein ◽  
Severe Dysplasia ◽  
Type Ii ◽  
Abernethy Malformation ◽  
Rex Shunt

scholarly journals Anomalies of the Portal Venous System in Dogs and Cats as Seen on Multidetector-Row CT: An Overview and Systematization Proposal

2019 ◽  
Author(s):  
Giovanna Bertolini
Keyword(s):  
Portal Vein ◽  
Venous System ◽  
Low Flow ◽  
Portal System ◽  
Multidetector Row Computed Tomography ◽  
Portal Venous System ◽  
Multidetector Row Ct ◽  
Multidetector Row ◽  
Vein Thrombosis ◽  
Portosystemic Shunts

This article offers an overview of congenital and acquired vascular anomalies involving the portal venous system in dogs and cats, as determined by multidetector-row computed tomography angiography. Congenital absence of the portal vein, portal vein hypoplasia, portal vein thrombosis and portal collaterals are described. Portal collaterals are further discussed as high- and low-flow connections, and categorized in hepatic arterioportal malformation, arteriovenous fistula, end-to-side and side-to-side congenital portosystemic shunts, acquired portosystemic shunts, cavoportal and porto-portal collaterals. Knowledge of different portal system anomalies helps understand the underlying physiopathological mechanism and is essential for surgical and interventional approaches.

Abernethy Malformation – a Rare but Important Diagnosis Prior to Liver-Directed Therapy for Hepatocellular Carcinoma

2019 ◽  
Vol 3 ◽  
pp. 9
Author(s):  
Nainesh Parikh ◽  
Kun Jiang ◽  
Kim Truong
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Portal Vein ◽  
Portosystemic Shunt ◽  
Left Hepatic Lobe ◽  
Abernethy Malformation ◽  
Hepatic Lobe ◽  
Portosystemic Shunts ◽  
Congenital Extrahepatic Portosystemic Shunt ◽  
The Right

A 45-year-old man with incidentally discovered, unresectable HCC were treated with TACE to the left hepatic lobe and TARE to the right hepatic lobe. Upon retrospective review, he was found to have a congenital extrahepatic portosystemic shunt with the absence of the portal vein (Abernethy malformation). This case report discusses variant splanchnic and portal anatomy in the setting of rare, congenital portosystemic shunts and evaluates types of liver-directed therapies for HCC in this setting.

Endovascular Treatment of Acute Posttransplant Portal Vein Thrombosis Due to Portal Steal From Mesocaval And Coronary Portosystemic Shunts

2020 ◽  
Vol 18 (5) ◽  
pp. 653-656
Author(s):  
Leonardo Centonze ◽  
Stefano Di Sandro ◽  
Marco Cereda ◽  
Andrea Lauterio ◽  
Riccardo De Carlis ◽  
...  
Keyword(s):  
Portal Vein ◽  
Endovascular Treatment ◽  
Portal Vein Thrombosis ◽  
Vein Thrombosis ◽  
Portosystemic Shunts

scholarly journals Multistage closure of a congenital extrahepatic portosystemic shunt

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
João Facas ◽  
Manuel Cruz ◽  
João Filipe Costa ◽  
Alfredo Agostinho ◽  
Paulo Donato
Keyword(s):  
Atrial Septal Defect ◽  
Endovascular Treatment ◽  
Septal Defect ◽  
Portal Pressure ◽  
Vena Cava ◽  
Portosystemic Shunt ◽  
Short Length ◽  
Portal System ◽  
Level Of Evidence ◽  
Portosystemic Shunts

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.

scholarly journals Abernethy Malformation in Children with Focal Liver Lesions

2021 ◽  
Vol 102 (3) ◽  
pp. 166-171
Author(s):  
D. E. Arutyunyants ◽  
V. V. Khovrin ◽  
T. N. Galyan ◽  
A. S. Kulichkin ◽  
A. V. Filin ◽  
...  
Keyword(s):  
Portal Vein ◽  
Focal Liver Lesions ◽  
Liver Lesions ◽  
Congenital Disease ◽  
Abernethy Malformation ◽  
Vascular Abnormality ◽  
Portosystemic Shunts ◽  
Clinical Cases

Abernethy malformation is a congenital disease characterized by complete or partial underdevelopment of the portal vein, leading to the occurrence of congenital extrahepatic portosystemic shunts. The anomaly with the absence of the portal vein is detected in 80% of cases in childhood. Patients with this pathology are very frequently examined when detecting space-occupying liver lesions and assuming the presence of a vascular abnormality. The paper describes clinical cases of benign focal liver lesions and Abernethy malformation in children.

scholarly journals Anomalies of the Portal Venous System in Dogs and Cats as Seen on Multidetector-Row Computed Tomography: An Overview and Systematization Proposal

2019 ◽  
Vol 6 (1) ◽  
pp. 10 ◽  
Author(s):  
Giovanna Bertolini
Keyword(s):  
Computed Tomography ◽  
Portal Vein ◽  
Venous System ◽  
Low Flow ◽  
Portal System ◽  
Multidetector Row Computed Tomography ◽  
Portal Venous System ◽  
Multidetector Row ◽  
Vein Thrombosis ◽  
Portosystemic Shunts

This article offers an overview of congenital and acquired vascular anomalies involving the portal venous system in dogs and cats, as determined by multidetector-row computed tomography angiography. Congenital absence of the portal vein, portal vein hypoplasia, portal vein thrombosis and portal collaterals are described. Portal collaterals are further discussed as high- and low-flow connections and categorized in hepatic arterioportal malformation, arteriovenous fistula, end-to-side and side-to-side congenital portosystemic shunts, acquired portosystemic shunts, cavoportal and porto-portal collaterals. Knowledge of different portal system anomalies helps understand the underlying physiopathological mechanism and is essential for surgical and interventional approaches.

The Clearance of 133Xenon from the Liver Intraportal Injection in Man

1965 ◽  
Vol 05 (03) ◽  
pp. 241-245 ◽  
Author(s):  
K.-F. Aronsen ◽  
B. Ericsson ◽  
A. Fajgelj ◽  
S.-E. Lindell
Keyword(s):  
Blood Flow ◽  
Portal Vein ◽  
Hepatic Blood Flow ◽  
Scintillation Detectors ◽  
Disappearance Rate ◽  
Simultaneous Measurements

Summary 133Xe dissolved in saline was injected into the portal vein in man. Hepatic blood flow was calculated from the disappearance rate of 133Xe recorded with scintillation detectors placed over the liver. The results are discussed and related to simultaneous measurements of the pressure in the portal vein.

Sign in / Sign up

Export Citation Format

Share Document