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Dandy-Walker Continuum
Susan I. Blaser, MD, FRCPC
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Dandy-Walker (DW) spectrum (DWS), DW complex (DWC), "classic" DW malformation (DWM)
            • Hypoplastic vermis with rotation (HVR), formerly DW variant (DWV)
            • Persistent Blake pouch cyst (BPC), mega cisterna magna (MCM)
          • Definitions

            • DWS represents heterogeneous spectrum of cystic posterior fossa (PF) malformations

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nonvascular Interventions

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              • Reporting Tips

                                                Selected References

                                                1. Chapman T et al: Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, posterior fossa disorders. Clin Imaging. 39(2):167-175, 2015
                                                2. Volpe P et al: Appearance of the fetal posterior fossa at 11-14 weeks in foetuses with Dandy-Walker complex or chromosomal anomalies. Ultrasound Obstet Gynecol. ePub, 2015
                                                3. De Cock J et al: A newborn with neurocutaneous melanocytosis and Dandy-Walker malformation. Pediatr Neurol. 50(3):276-8, 2014
                                                4. Poretti A et al: Cerebellar hypoplasia: Differential diagnosis and diagnostic approach. Am J Med Genet C Semin Med Genet. 166(2):211-26, 2014
                                                5. Guibaud L et al: Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling. Prenat Diagn. 32(2):185-93, 2012
                                                6. Patek KJ et al: Posterior fossa anomalies diagnosed with fetal MRI: associated anomalies and neurodevelopmental outcomes. Prenat Diagn. 32(1):75-82, 2012
                                                7. Aldinger KA et al: FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nat Genet. 41(9):1037-42, 2009
                                                8. Barkovich AJ et al: A developmental and genetic classification for midbrain-hindbrain malformations. Brain. 132(Pt 12):3199-230, 2009
                                                9. Millen KJ et al: Cerebellar development and disease. Curr Opin Neurobiol. 18(1):12-9, 2008
                                                10. Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
                                                11. ten Donkelaar HJ et al: Development and developmental disorders of the human cerebellum. J Neurol. 250(9):1025-36, 2003
                                                12. Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Dandy-Walker (DW) spectrum (DWS), DW complex (DWC), "classic" DW malformation (DWM)
                                                          • Hypoplastic vermis with rotation (HVR), formerly DW variant (DWV)
                                                          • Persistent Blake pouch cyst (BPC), mega cisterna magna (MCM)
                                                        • Definitions

                                                          • DWS represents heterogeneous spectrum of cystic posterior fossa (PF) malformations

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Nonvascular Interventions

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          • Image Interpretation Pearls

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Chapman T et al: Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, posterior fossa disorders. Clin Imaging. 39(2):167-175, 2015
                                                                                              2. Volpe P et al: Appearance of the fetal posterior fossa at 11-14 weeks in foetuses with Dandy-Walker complex or chromosomal anomalies. Ultrasound Obstet Gynecol. ePub, 2015
                                                                                              3. De Cock J et al: A newborn with neurocutaneous melanocytosis and Dandy-Walker malformation. Pediatr Neurol. 50(3):276-8, 2014
                                                                                              4. Poretti A et al: Cerebellar hypoplasia: Differential diagnosis and diagnostic approach. Am J Med Genet C Semin Med Genet. 166(2):211-26, 2014
                                                                                              5. Guibaud L et al: Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling. Prenat Diagn. 32(2):185-93, 2012
                                                                                              6. Patek KJ et al: Posterior fossa anomalies diagnosed with fetal MRI: associated anomalies and neurodevelopmental outcomes. Prenat Diagn. 32(1):75-82, 2012
                                                                                              7. Aldinger KA et al: FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nat Genet. 41(9):1037-42, 2009
                                                                                              8. Barkovich AJ et al: A developmental and genetic classification for midbrain-hindbrain malformations. Brain. 132(Pt 12):3199-230, 2009
                                                                                              9. Millen KJ et al: Cerebellar development and disease. Curr Opin Neurobiol. 18(1):12-9, 2008
                                                                                              10. Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
                                                                                              11. ten Donkelaar HJ et al: Development and developmental disorders of the human cerebellum. J Neurol. 250(9):1025-36, 2003
                                                                                              12. Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002