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Asplenia and Polysplenia
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Asplenia (ASP), polysplenia (PSP)
          • Synonyms

            • Heterotaxy syndromes
            • ASP: Asplenia syndrome, Ivemark syndrome, bilateral right-sidedness
            • PSP: Polysplenia syndrome, bilateral left-sidedness
          • Definitions

            • Complex inherited syndromes associated with absence (ASP) or multiplicity (PSP) of spleens, as well as many other anomalies
          • Associated Syndromes

            • Heterotaxy: Abnormal embryologic placement of thoracoabdominal structures across right-left axis of body
            • Situs solitus: Normal placement of thoracoabdominal organs in right-left axis
            • Situs inversus: Reversal of normal positions of thoracoabdominal organs across right-left axis (mirror-image of situs solitus)
              • Can be subdivided into situs inversus with dextrocardia or levocardia
            • Situs ambiguus (heterotaxy syndrome): Abnormal placement of thoracoabdominal structures without situs inversus
              • Situs ambiguus with polysplenia: Left isomerism or bilateral left-sidedness
              • Situs ambiguus with asplenia: Right isomerism or bilateral right-sidedness

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • Angiographic Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Tawfik AM et al: Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 35(8):647-53, 2013
                                        2. Yildiz AE et al: Splenic anomalies of shape, size, and location: pictorial essay. ScientificWorldJournal. 2013:321810, 2013
                                        3. Kim SW et al: Duplication of the spleen with a short pancreas. Br J Radiol. 82(975):e42-3, 2009
                                        4. Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
                                        5. De La Villeon B et al: Polysplenia syndrome. J Visc Surg. 148(5):e395-6, 2011
                                        6. Davit-Spraul A et al: CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr. 46(1):111-2, 2008
                                        7. Halac M et al: Appearance of situs inversus totalis and polysplenia syndrome on FDG PET/CT. Clin Nucl Med. 33(2):142-3, 2008
                                        8. Maier M et al: Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR Am J Roentgenol. 188(2):W150-3, 2007
                                        9. Elsayes KM et al: MR imaging of the spleen: spectrum of abnormalities. Radiographics. 25(4):967-82, 2005
                                        10. Combs LS et al: Evaluation of spleen in children with heterotaxia and congenital heart disease. Tenn Med. 97(4):161-3, 2004
                                        11. Fulcher AS et al: Abdominal manifestations of situs anomalies in adults. Radiographics. 22(6):1439-56, 2002
                                        12. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
                                        13. Paterson A et al: A pattern-oriented approach to splenic imaging in infants and children. Radiographics. 19(6):1465-85, 1999
                                        14. Ruscazio M et al: Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol. 81(1):111-6, 1998
                                        15. Nakada K et al: Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg. 32(1):91-4, 1997
                                        16. Freeman JL et al: CT of congenital and acquired abnormalities of the spleen. Radiographics. 13(3):597-610, 1993
                                        17. Chitayat D et al: Prenatal diagnosis of asplenia/polysplenia syndrome. Am J Obstet Gynecol. 158(5):1085-7, 1988
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Asplenia (ASP), polysplenia (PSP)
                                                • Synonyms

                                                  • Heterotaxy syndromes
                                                  • ASP: Asplenia syndrome, Ivemark syndrome, bilateral right-sidedness
                                                  • PSP: Polysplenia syndrome, bilateral left-sidedness
                                                • Definitions

                                                  • Complex inherited syndromes associated with absence (ASP) or multiplicity (PSP) of spleens, as well as many other anomalies
                                                • Associated Syndromes

                                                  • Heterotaxy: Abnormal embryologic placement of thoracoabdominal structures across right-left axis of body
                                                  • Situs solitus: Normal placement of thoracoabdominal organs in right-left axis
                                                  • Situs inversus: Reversal of normal positions of thoracoabdominal organs across right-left axis (mirror-image of situs solitus)
                                                    • Can be subdivided into situs inversus with dextrocardia or levocardia
                                                  • Situs ambiguus (heterotaxy syndrome): Abnormal placement of thoracoabdominal structures without situs inversus
                                                    • Situs ambiguus with polysplenia: Left isomerism or bilateral left-sidedness
                                                    • Situs ambiguus with asplenia: Right isomerism or bilateral right-sidedness

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • Angiographic Findings

                                                        • Nuclear Medicine Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Tawfik AM et al: Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 35(8):647-53, 2013
                                                                              2. Yildiz AE et al: Splenic anomalies of shape, size, and location: pictorial essay. ScientificWorldJournal. 2013:321810, 2013
                                                                              3. Kim SW et al: Duplication of the spleen with a short pancreas. Br J Radiol. 82(975):e42-3, 2009
                                                                              4. Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
                                                                              5. De La Villeon B et al: Polysplenia syndrome. J Visc Surg. 148(5):e395-6, 2011
                                                                              6. Davit-Spraul A et al: CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr. 46(1):111-2, 2008
                                                                              7. Halac M et al: Appearance of situs inversus totalis and polysplenia syndrome on FDG PET/CT. Clin Nucl Med. 33(2):142-3, 2008
                                                                              8. Maier M et al: Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR Am J Roentgenol. 188(2):W150-3, 2007
                                                                              9. Elsayes KM et al: MR imaging of the spleen: spectrum of abnormalities. Radiographics. 25(4):967-82, 2005
                                                                              10. Combs LS et al: Evaluation of spleen in children with heterotaxia and congenital heart disease. Tenn Med. 97(4):161-3, 2004
                                                                              11. Fulcher AS et al: Abdominal manifestations of situs anomalies in adults. Radiographics. 22(6):1439-56, 2002
                                                                              12. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
                                                                              13. Paterson A et al: A pattern-oriented approach to splenic imaging in infants and children. Radiographics. 19(6):1465-85, 1999
                                                                              14. Ruscazio M et al: Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol. 81(1):111-6, 1998
                                                                              15. Nakada K et al: Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg. 32(1):91-4, 1997
                                                                              16. Freeman JL et al: CT of congenital and acquired abnormalities of the spleen. Radiographics. 13(3):597-610, 1993
                                                                              17. Chitayat D et al: Prenatal diagnosis of asplenia/polysplenia syndrome. Am J Obstet Gynecol. 158(5):1085-7, 1988