A rare Abernethy Ib malformation was initially misdiagnosed as chronic portal vein thrombosis in a 27-year-old female

Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report

“…Clinically, PV thrombosis is usually associated with underlying diseases or risk factors such as canalization of the umbilical vein in neonates, compression or invasion by a neoplasm, liver cirrhosis, and hypercoagulable conditions [ 1 ]. Long-term PV thrombosis can result in the development of spontaneous collateral veins and other secondary signs of portal hypertension, such as splenomegaly or ascites, which are critical to distinguish it from CEPS [ 1 , [8] , [9] , [10] ]. Patients with CEPS typically have no features of portal hypertension [ 1 ].…”

“…Although conventional angiography is considered the gold-standard diagnostic modality, it is usually not required, as CT and MRI can mostly correctly suggest the diagnosis [ 1 , 4 ]. Because of the common imaging findings, it is sometimes difficult to differentiate between CEPS and PV thrombosis and there are previously reported cases with similar diagnostic dilemmas in which CEPS was initially reported as chronic PV thrombosis [ 1 , [8] , [9] , [10] ]. However, CEPS is not usually associated with collateral vessels and findings suggestive of portal hypertension [ 6 ].…”