Der kongenitale portosystemische Shunt

Abstract

The Abernethy malformation is a rare congenital portosystemic shunt in which the blood directly drains into the systemic vein bypassing the liver either through a complete (type 1) or a partial shunt (type 2). The diagnosis is most frequently established primarily with ultrasound. CT and MRI are used for further classification of the shunt and assessment of accompanying liver tumors and malformations. There is a wide spectrum of therapeutic options ranging from noninvasive conservative treatment to liver transplantation. The main prognostic factors are the occurrence of concomitant hepatic neoplasms and hepatic encephalopathy. We report two cases diagnosed with a type 1 shunt, hepatic encephalopathy, and associated liver tumors who underwent successful liver transplantation after having considered all therapeutic options.

Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature

“…However, focal nodular hyperplasia(FNH) was the most common hepatic tumor in patients with CAPV. Fifteen of the thirty-four reported cases with hepatic tumors involved FNH (Table 1 ), with four comprising solitary lesions [ 31 - 34 ], nine with multiple lesions [ 4 , 5 , 35 - 40 ], and one with an FNH lesion accompanied by additional tumors (HCC and adenoma) [ 41 ]. FNH and adenomas with a high risk of malignant transformation to HCC or hepatoblastoma [ 1 ].…”