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

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.

Beyond the Rib Cage: Unraveling Abernethy Syndrome as a Rare Cause of Cyanosis

“…Abernethy syndrome, also known as portal vein agenesis, is a rare cause of hepatopulmonary syndrome, characterized by a congenital extrahepatic systemic shunt [ 6 ]. Diagnosis is challenging as clinical symptoms are non-specific; patients may remain asymptomatic or present with hepatic insufficiency, cirrhosis, or isolated cyanosis [ 5 ].…”

“…In terms of treatment, various therapeutic options can be considered. These include early interventional radiology, embolization of the portosystemic shunt, surgical ligation, and, ultimately, liver transplantation as a last resort [ 6 , 7 ]. It is crucial to determine the type of shunt before proceeding with any intervention [ 3 ].…”

Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient

“…Liver transplantation is the only curative treatment for type I shunts, while patients of type II should be treated by different methods based on the development of intrahepatic portal vein branches (9). The therapeutic options for type II abernethy malformation include surgical ligation and transcatheter closure of the shunt (10,11). With a deeper understanding, interventional radiology is the preferred method of closure (12).…”