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Dandy-Walker Malformation
Anne Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Dandy-Walker malformation (DWM)
              • Definitions

                • 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 of Herophili
                • Dandy-Walker continuum: Abandoned term encompassing spectrum of PF findings
                  • Distinctions between PF abnormalities can be subtle
                  • Aim for maximum precision as very 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. AIUM practice parameter for the performance of detailed diagnostic obstetric ultrasound examinations between 12 weeks 0 days and 13 weeks 6 days. J Ultrasound Med. 40(5):E1-16, 2021
                                          2. Garcia-Rodriguez R et al: First-trimester cystic posterior fossa: reference ranges, associated findings, and pregnancy outcomes. J Matern Fetal Neonatal Med. 1-10, 2019
                                          3. Morton CC et al: The Impact of Prenatal Diagnosis of Selected Central Nervous System Anomalies for Prenatal Counselling Based on Significant Pregnancy Morbidity and Neonatal Outcomes. J Obstet Gynaecol Can. 41(2):166-173.e1, 2019
                                          4. Paladini D et al: Differentiating features of posterior fossa at 12-13 weeks' gestation in fetuses with Dandy-Walker malformation and Blake's pouch cyst. Ultrasound Obstet Gynecol. 53(6):850-852, 2019
                                          5. Paladini D et al: Hindbrain morphometry and choroid plexus position in differential diagnosis of posterior fossa cystic malformations. Ultrasound Obstet Gynecol. 54(2):207-14, 2019
                                          6. Chapman T et al: Establishment of normative values for the fetal posterior fossa by magnetic resonance imaging. Prenat Diagn. 38(13):1035-41, 2018
                                          7. Martinez-Ten P et al: Non-visualization of choroid plexus of fourth ventricle as first-trimester predictor of posterior fossa anomalies and chromosomal defects. Ultrasound Obstet Gynecol. 51(2):199-207, 2018
                                          8. Wüest A et al: Enlarged posterior fossa on prenatal imaging: differential diagnosis, associated anomalies and postnatal outcome. Acta Obstet Gynecol Scand. 96(7):837-43, 2017
                                          9. Bernardo S et al: Dandy-Walker Malformation: is the 'tail sign' the key sign? Prenat Diagn. 35(13):1358-64, 2015
                                          10. Chapman T et al: Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, posterior fossa disorders. Clin Imaging. 39(2):167-75, 2015
                                          11. Ghali R et al: Perinatal and short-term neonatal outcomes of posterior fossa anomalies. Fetal Diagn Ther. 35(2):108-17, 2014
                                          12. 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
                                          13. Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
                                          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

                                                      • Abbreviations

                                                        • Dandy-Walker malformation (DWM)
                                                      • Definitions

                                                        • 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 of Herophili
                                                        • Dandy-Walker continuum: Abandoned term encompassing spectrum of PF findings
                                                          • Distinctions between PF abnormalities can be subtle
                                                          • Aim for maximum precision as very 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. AIUM practice parameter for the performance of detailed diagnostic obstetric ultrasound examinations between 12 weeks 0 days and 13 weeks 6 days. J Ultrasound Med. 40(5):E1-16, 2021
                                                                                  2. Garcia-Rodriguez R et al: First-trimester cystic posterior fossa: reference ranges, associated findings, and pregnancy outcomes. J Matern Fetal Neonatal Med. 1-10, 2019
                                                                                  3. Morton CC et al: The Impact of Prenatal Diagnosis of Selected Central Nervous System Anomalies for Prenatal Counselling Based on Significant Pregnancy Morbidity and Neonatal Outcomes. J Obstet Gynaecol Can. 41(2):166-173.e1, 2019
                                                                                  4. Paladini D et al: Differentiating features of posterior fossa at 12-13 weeks' gestation in fetuses with Dandy-Walker malformation and Blake's pouch cyst. Ultrasound Obstet Gynecol. 53(6):850-852, 2019
                                                                                  5. Paladini D et al: Hindbrain morphometry and choroid plexus position in differential diagnosis of posterior fossa cystic malformations. Ultrasound Obstet Gynecol. 54(2):207-14, 2019
                                                                                  6. Chapman T et al: Establishment of normative values for the fetal posterior fossa by magnetic resonance imaging. Prenat Diagn. 38(13):1035-41, 2018
                                                                                  7. Martinez-Ten P et al: Non-visualization of choroid plexus of fourth ventricle as first-trimester predictor of posterior fossa anomalies and chromosomal defects. Ultrasound Obstet Gynecol. 51(2):199-207, 2018
                                                                                  8. Wüest A et al: Enlarged posterior fossa on prenatal imaging: differential diagnosis, associated anomalies and postnatal outcome. Acta Obstet Gynecol Scand. 96(7):837-43, 2017
                                                                                  9. Bernardo S et al: Dandy-Walker Malformation: is the 'tail sign' the key sign? Prenat Diagn. 35(13):1358-64, 2015
                                                                                  10. Chapman T et al: Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, posterior fossa disorders. Clin Imaging. 39(2):167-75, 2015
                                                                                  11. Ghali R et al: Perinatal and short-term neonatal outcomes of posterior fossa anomalies. Fetal Diagn Ther. 35(2):108-17, 2014
                                                                                  12. 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
                                                                                  13. Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014