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      Asymptomatic Congenital Intrahepatic Portosystemic Shunt

      case-report
      Author(s): , M.D. 1 , , M.D. 1
      Publication date (Electronic):
      Journal: Radiology Case Reports
      Publisher: Elsevier
      Keywords: CT, computed tomography

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          Abstract

          Congenital (spontaneous) intrahepatic portosystemic shunt is rare in the English literature. Most cases of portosystemic shunt occur after trauma, surgery, liver biopsy or as a result of chronic portal hypertension. Chronic shunting may result in encephalopathy, bleeding or hyperinsulinism. We report a case of an asymptomatic adult female with a presumed congenital intrahepatic portosystemic shunt and discuss the pertinent imaging findings and important key concepts related to this condition.

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          Congenital extrahepatic portocaval shunts--the Abernethy malformation.

          Edward R Howard, Mark Davenport (1997)
          The first account of an absent portal vein and a congenital mesenterico-caval shunt was given by John Abernethy in 1793. Five new cases of congenital extrahepatic portocaval shunts are described in this report. One end-to-side shunt with congenital absence of the portal vein (type 1 shunt) is the 13th case in the literature. Four are side-to-side (type 2) shunts of which there have been only two previous reports. Type 1 shunts are associated with other congenital abnormalities and have only been recognized in girls. Five of these cases developed liver tumours. Type 2 shunts are rarer, and four of five have been boys. They are not usually associated with other anomalies. Encephalopathy may be associated with these shunts in adults, and surgical closure of the side-to-side shunts is therefore recommended. End-to-side shunts are not correctable but the abnormal anatomy may create a problem for hepatic transplantation, should this be necessary for associated disorders such as biliary atresia. "The Abernethy Malformation" would seem to be a suitable eponym for congenital extrahepatic portocaval shunts.
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            Intrahepatic portosystemic venous shunt.

            Rita S. Cha, M. Han, Hay-Oak Park … (1990)
            Article 0 comments Cited 42 times – based on 0 reviews     Review now
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              The clinical anatomy of congenital portosystemic venous shunts.

              Mark Stringer (2008)
              Congenital portosystemic venous shunts are rare. Their gross anatomy has not been well defined. Four different varieties of congenital portosystemic venous shunts are described in six children seen during a 10-year period, focusing on the anatomy of the shunt as determined by imaging studies and surgery. A detailed review of the literature indicates that congenital portosystemic venous shunts are best classified as: extrahepatic or intrahepatic. Extrahepatic shunts may be further subdivided into portocaval shunts (type 1 end-to-side and type 2 side-to-side) and others. Intrahepatic shunts are due to an abnormal intrahepatic connection between the portal vein and hepatic vein/inferior vena cava or a persistent patent ductus venosus. Additional congenital anomalies, particularly cardiac malformations, may be associated with any type. Some congenital intrahepatic portosystemic venous shunts close spontaneously in infancy; all other congenital portosystemic venous shunts tend to remain patent. To a variable extent, depending largely on the volume and duration of the shunt, affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor. The key to understanding the pathogenesis of these shunts lies in the normal developmental mechanisms underlying the formation of the portal vein and inferior vena cava in the embryo. Copyright 2008 Wiley-Liss, Inc.
              Article 0 comments Cited 36 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Karen M. Brown
                Journal
                Journal ID (nlm-ta): Radiol Case Rep
                Journal ID (iso-abbrev): Radiol Case Rep
                Title: Radiology Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 1930-0433
                Publication date PMC-release: 04 October 2016
                Publication date Collection: 2009
                Publication date (Electronic): 04 October 2016
                Volume: 4
                Issue: 1
                Page: 255
                Author notes
                [1]

                Karen M. Brown, M.D., and Hassan Hal, M.D., are in the Department of Radiology, Milton S. Hershey Medical Center, Hershey, PA, United States of America.

                Article
                Publisher ID: S1930-0433(16)30265-5 Publisher ID: 255
                DOI: 10.2484/rcr.v4i1.255
                PMC ID: 5106540
                PubMed ID: 27843522
                SO-VID: f9456c3b-3be8-4eaf-b1fe-935137e01509
                Copyright © © 2009 The Authors.
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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                Subject: Article

                Keywords: ct, computed tomography
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                Keywords: ct, computed tomography

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