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Dandy-Walker Continuum
Usha D. Nagaraj, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Classic Dandy-Walker malformation (DWM)
          • Synonyms

            • Terms DW spectrum, DW variant, & DW complex have complicated clinical & imaging implications in literature
          • Definitions

            • DW continuum represents clinically & radiologically heterogeneous group of posterior fossa (PF) malformations
            • Referred to as continuum by some based on belief that DWM, vermian hypoplasia (VH), Blake pouch (BP) remnant/cyst, & mega cisterna magna (MCM) are spectrum of developmental anomalies of rhombencephalic vesicle roof
              • Terminology controversial & not uniformly accepted
              • Clinical severity ranges widely in this group

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • 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. Bosemani T et al: Congenital abnormalities of the posterior fossa. Radiographics. 35(1):200-20, 2015
                                              2. Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
                                              3. Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 477-9, 2012
                                              4. Guibaud L et al: Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling. Prenat Diagn. 32(2):185-93, 2012
                                              5. Patek KJ et al: Posterior fossa anomalies diagnosed with fetal MRI: associated anomalies and neurodevelopmental outcomes. Prenat Diagn. 32(1):75-82, 2012
                                              6. Volpe P et al: Brainstem-vermis and brainstem-tentorium angles allow accurate categorization of fetal upward rotation of cerebellar vermis. Ultrasound Obstet Gynecol. 39(6):632-5, 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(12):3199-230, 2009
                                              9. Walbert T et al: Symptomatic neurocutaneous melanosis and Dandy-Walker malformation in an adult. J Clin Oncol. 27(17):2886-7, 2009
                                              10. Elsen GE et al: Zic1 and Zic4 regulate zebrafish roof plate specification and hindbrain ventricle morphogenesis. Dev Biol. 314(2):376-92, 2008
                                              11. Millen KJ et al: Cerebellar development and disease. Curr Opin Neurobiol. 18(1):12-9, 2008
                                              12. Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
                                              13. ten Donkelaar HJ et al: Development and developmental disorders of the human cerebellum. J Neurol. 250(9):1025-36, 2003
                                              14. Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
                                              15. Tortori-Donati P et al: Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities. Childs Nerv Syst. 12(6):303-8, 1996
                                              16. Kollias SS et al: Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis. Radiographics. 13(6):1211-31, 1993
                                              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

                                                        • Classic Dandy-Walker malformation (DWM)
                                                      • Synonyms

                                                        • Terms DW spectrum, DW variant, & DW complex have complicated clinical & imaging implications in literature
                                                      • Definitions

                                                        • DW continuum represents clinically & radiologically heterogeneous group of posterior fossa (PF) malformations
                                                        • Referred to as continuum by some based on belief that DWM, vermian hypoplasia (VH), Blake pouch (BP) remnant/cyst, & mega cisterna magna (MCM) are spectrum of developmental anomalies of rhombencephalic vesicle roof
                                                          • Terminology controversial & not uniformly accepted
                                                          • Clinical severity ranges widely in this group

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • 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. Bosemani T et al: Congenital abnormalities of the posterior fossa. Radiographics. 35(1):200-20, 2015
                                                                                          2. Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
                                                                                          3. Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 477-9, 2012
                                                                                          4. Guibaud L et al: Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling. Prenat Diagn. 32(2):185-93, 2012
                                                                                          5. Patek KJ et al: Posterior fossa anomalies diagnosed with fetal MRI: associated anomalies and neurodevelopmental outcomes. Prenat Diagn. 32(1):75-82, 2012
                                                                                          6. Volpe P et al: Brainstem-vermis and brainstem-tentorium angles allow accurate categorization of fetal upward rotation of cerebellar vermis. Ultrasound Obstet Gynecol. 39(6):632-5, 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(12):3199-230, 2009
                                                                                          9. Walbert T et al: Symptomatic neurocutaneous melanosis and Dandy-Walker malformation in an adult. J Clin Oncol. 27(17):2886-7, 2009
                                                                                          10. Elsen GE et al: Zic1 and Zic4 regulate zebrafish roof plate specification and hindbrain ventricle morphogenesis. Dev Biol. 314(2):376-92, 2008
                                                                                          11. Millen KJ et al: Cerebellar development and disease. Curr Opin Neurobiol. 18(1):12-9, 2008
                                                                                          12. Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
                                                                                          13. ten Donkelaar HJ et al: Development and developmental disorders of the human cerebellum. J Neurol. 250(9):1025-36, 2003
                                                                                          14. Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
                                                                                          15. Tortori-Donati P et al: Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities. Childs Nerv Syst. 12(6):303-8, 1996
                                                                                          16. Kollias SS et al: Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis. Radiographics. 13(6):1211-31, 1993