Describe the current classifica­tion and identify congenital extra-hepatic portosystemic shunts (CEPS) on the basis of CT imaging findings. Discuss the value of the most used imaging techniques in diagno­sis of type 1 CEPS in our experience.

Congenital extra-hepatic porto-systemic shunts (Abernethy malformations) are extremely rare vascular malformations. Their gross anatomy has not been well defined but a review of the literature indicates that are classified into two types: type 1 if the portal vein is absent and the entire portal blood drains to the vena cava, type 2 when the portal blood is diverted into vena cava through extra-hepatic communication with a hypoplastic portal vein [1-3]. Differentiation between these two variants plays an essential role in determina­tion of the definitive method...

The diagnosis of CEPS is made with US, CT, MR imaging, or sometimes angiography. US is the initial examination for patients with nonspecific liver disease, because it is non-invasive and may demonstrate the absent PV in most cases. Doppler US is also useful to determine the flow direction of the identified vessels. Both CT angiography and MR angiography depict the course of the porto-systemic shunt and allow identification of the absent portal branches. Despite MR Angiography not ex­posing patients to ionizing radiation must be considered...

Despite conventional Angiography is considered the gold standard technique for diagnosis of CEPS it is not usually required because CT imaging findings suggest the correct diagno­sis in most cases. Abernethy malformations are rare condition, usually involving children, but the possibility, even in old patient with hepatic encephalopathy and with no radiological and biochemical sign of chronic liver disease, must be taken in consideration.

1. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): A Histopathologic Evaluation. Mikhail Lisovsky, Angelos A. Konstas, Joseph Misdraji et al. Am J Surg Pathol 2011;35:1381–1390 2. The Clinical Anatomy of Congenital Portosystemic Venous Shunts. Mark. D. Stringer et al. Clinical Anatomy 2008 21:147–157 3. Congenital extrahe­patic portosystemic shunts. Murray CP, Yoo SJ, Baby PS. Pediatr Radiol 2003;33 (9):614–620. 4. Resolution of Hepatopulmonary Syndrome After Auxiliary Partial Orthotopic Liver Transplantation in Abernethy Malformation. A Case Report. Sukru Emre et al. Liver Transplantation 2007; 13:1662-1668 5. Clinical and...