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Abernethy Malformation
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Congenital absence of portal vein, congenital extrahepatic portosystemic shunt
              • Definitions

                • Congenital malformation characterized by diversion of portal venous blood flow around liver, more commonly into inferior vena cava (IVC) than renal, iliac, or azygous veins
                  • Type 1: Absence of portal vein with extrahepatic portosystemic shunt
                    • Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
                    • Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
                  • Type 2: Intact but hypoplastic portal vein with extrahepatic portosystemic shunt

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Hao Y et al: Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol Lett. 9(2):695-700, 2015
                                        2. Nacif LS et al: Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Med Imaging. 15:37, 2015
                                        3. Alonso-Gamarra E et al: Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 31(3):707-22, 2011
                                        4. Morgan G et al: Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 29(9):1239-41, 1994
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Congenital absence of portal vein, congenital extrahepatic portosystemic shunt
                                                    • Definitions

                                                      • Congenital malformation characterized by diversion of portal venous blood flow around liver, more commonly into inferior vena cava (IVC) than renal, iliac, or azygous veins
                                                        • Type 1: Absence of portal vein with extrahepatic portosystemic shunt
                                                          • Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
                                                          • Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
                                                        • Type 2: Intact but hypoplastic portal vein with extrahepatic portosystemic shunt

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Angiographic Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Hao Y et al: Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol Lett. 9(2):695-700, 2015
                                                                              2. Nacif LS et al: Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Med Imaging. 15:37, 2015
                                                                              3. Alonso-Gamarra E et al: Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 31(3):707-22, 2011
                                                                              4. Morgan G et al: Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 29(9):1239-41, 1994