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Dandy-Walker Malformation
Karen Y. Oh, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Dandy-Walker malformation (DWM)
            • Synonyms

              • Dandy-Walker complex, Dandy-Walker spectrum
            • Definitions

              • Classic DWM
                • Complete or partial agenesis of cerebellar vermis
                • Cystic dilation of 4th ventricle (4V)
                • Enlarged posterior fossa (PF) due to elevation of cerebellar tentorium and torcular
              • Dandy-Walker continuum used to encompass spectrum of PF malformations, including classic DWM, vermian agenesis/dysgenesis, persistent Blake pouch, mega cisterna magna
                • Important to be as precise as possible and not lump together, as these have different prognostic implications

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical 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. Ghali R et al: Perinatal and short-term neonatal outcomes of posterior fossa anomalies. Fetal Diagn Ther. 35(2):108-17, 2014
                                        3. Leibovitz Z et al: Fetal posterior fossa dimensions: normal and anomalous development assessed in mid-sagittal cranial plane by three-dimensional multiplanar sonography. Ultrasound Obstet Gynecol. 43(2):147-53, 2014
                                        4. Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
                                        5. Gandolfi Colleoni G et al: Prenatal diagnosis and outcome of fetal posterior fossa fluid collections. Ultrasound Obstet Gynecol. 39(6):625-31, 2012
                                        6. Shekdar K: Posterior fossa malformations. Semin Ultrasound CT MR. 32(3):228-41, 2011
                                        7. Malinger G et al: The fetal cerebellum. Pitfalls in diagnosis and management. Prenat Diagn. 29(4):372-80, 2009
                                        8. Kontopoulos EV et al: Dandy-Walker syndrome and monochorionic twins: insight into a possible etiological mechanism. J Matern Fetal Neonatal Med. 21(11):839-42, 2008
                                        9. Limperopoulos C et al: How accurately does current fetal imaging identify posterior fossa anomalies? AJR Am J Roentgenol. 190(6):1637-43, 2008
                                        10. Forzano F et al: Posterior fossa malformation in fetuses: a report of 56 further cases and a review of the literature. Prenat Diagn. 27(6):495-501, 2007
                                        11. Oh KY et al: The fetal posterior fossa: clinical correlation of findings on prenatal ultrasound and fetal magnetic resonance imaging. Ultrasound Q. 23(3):203-10, 2007
                                        12. Robinson AJ et al: The fetal cerebellar vermis: assessment for abnormal development by ultrasonography and magnetic resonance imaging. Ultrasound Q. 23(3):211-23, 2007
                                        13. Guibaud L et al: Plea for an anatomical approach to abnormalities of the posterior fossa in prenatal diagnosis. Ultrasound Obstet Gynecol. 27(5):477-81, 2006
                                        14. Has R et al: Dandy-walker malformation: a review of 78 cases diagnosed by prenatal sonography. Fetal Diagn Ther. 19(4):342-7, 2004
                                        15. Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
                                        16. Klein O et al: Dandy-Walker malformation: prenatal diagnosis and prognosis. Childs Nerv Syst. 19(7-8):484-9, 2003
                                        17. Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
                                        18. Malinger G et al: The fetal cerebellar vermis: normal development as shown by transvaginal ultrasound. Prenat Diagn. 21(8):687-92, 2001
                                        19. Calabro F et al: Blake's pouch cyst: an entity within the Dandy-Walker continuum. Neuroradiology. 42(4):290-5, 2000
                                        20. Ecker JL et al: The sonographic diagnosis of Dandy-Walker and Dandy-Walker variant: associated findings and outcomes. Prenat Diagn. 20(4):328-32, 2000
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Dandy-Walker malformation (DWM)
                                                  • Synonyms

                                                    • Dandy-Walker complex, Dandy-Walker spectrum
                                                  • Definitions

                                                    • Classic DWM
                                                      • Complete or partial agenesis of cerebellar vermis
                                                      • Cystic dilation of 4th ventricle (4V)
                                                      • Enlarged posterior fossa (PF) due to elevation of cerebellar tentorium and torcular
                                                    • Dandy-Walker continuum used to encompass spectrum of PF malformations, including classic DWM, vermian agenesis/dysgenesis, persistent Blake pouch, mega cisterna magna
                                                      • Important to be as precise as possible and not lump together, as these have different prognostic implications

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical 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. Ghali R et al: Perinatal and short-term neonatal outcomes of posterior fossa anomalies. Fetal Diagn Ther. 35(2):108-17, 2014
                                                                              3. Leibovitz Z et al: Fetal posterior fossa dimensions: normal and anomalous development assessed in mid-sagittal cranial plane by three-dimensional multiplanar sonography. Ultrasound Obstet Gynecol. 43(2):147-53, 2014
                                                                              4. Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
                                                                              5. Gandolfi Colleoni G et al: Prenatal diagnosis and outcome of fetal posterior fossa fluid collections. Ultrasound Obstet Gynecol. 39(6):625-31, 2012
                                                                              6. Shekdar K: Posterior fossa malformations. Semin Ultrasound CT MR. 32(3):228-41, 2011
                                                                              7. Malinger G et al: The fetal cerebellum. Pitfalls in diagnosis and management. Prenat Diagn. 29(4):372-80, 2009
                                                                              8. Kontopoulos EV et al: Dandy-Walker syndrome and monochorionic twins: insight into a possible etiological mechanism. J Matern Fetal Neonatal Med. 21(11):839-42, 2008
                                                                              9. Limperopoulos C et al: How accurately does current fetal imaging identify posterior fossa anomalies? AJR Am J Roentgenol. 190(6):1637-43, 2008
                                                                              10. Forzano F et al: Posterior fossa malformation in fetuses: a report of 56 further cases and a review of the literature. Prenat Diagn. 27(6):495-501, 2007
                                                                              11. Oh KY et al: The fetal posterior fossa: clinical correlation of findings on prenatal ultrasound and fetal magnetic resonance imaging. Ultrasound Q. 23(3):203-10, 2007
                                                                              12. Robinson AJ et al: The fetal cerebellar vermis: assessment for abnormal development by ultrasonography and magnetic resonance imaging. Ultrasound Q. 23(3):211-23, 2007
                                                                              13. Guibaud L et al: Plea for an anatomical approach to abnormalities of the posterior fossa in prenatal diagnosis. Ultrasound Obstet Gynecol. 27(5):477-81, 2006
                                                                              14. Has R et al: Dandy-walker malformation: a review of 78 cases diagnosed by prenatal sonography. Fetal Diagn Ther. 19(4):342-7, 2004
                                                                              15. Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
                                                                              16. Klein O et al: Dandy-Walker malformation: prenatal diagnosis and prognosis. Childs Nerv Syst. 19(7-8):484-9, 2003
                                                                              17. Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
                                                                              18. Malinger G et al: The fetal cerebellar vermis: normal development as shown by transvaginal ultrasound. Prenat Diagn. 21(8):687-92, 2001
                                                                              19. Calabro F et al: Blake's pouch cyst: an entity within the Dandy-Walker continuum. Neuroradiology. 42(4):290-5, 2000
                                                                              20. Ecker JL et al: The sonographic diagnosis of Dandy-Walker and Dandy-Walker variant: associated findings and outcomes. Prenat Diagn. 20(4):328-32, 2000