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Chiari 2 Malformation
Roya Sohaey, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Chiari 2 (Ch2) malformation
              • Open neural tube defect (ONTD)
            • Synonyms

              • Arnold Ch2 malformation
            • Definitions

              • Anomaly of cerebellum, brainstem, and cervical cord associated with small bony posterior fossa and typically seen with ONTD

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Ben Miled S et al: Severe and progressive neuronal loss in myelomeningocele begins before 16 weeks of pregnancy. Am J Obstet Gynecol. 223(2):256.e1-9, 2020
                                        2. Donepudi R et al: Trends in ventricle size during pregnancy and its use for prediction of ventriculoperitoneal shunt in fetal myelomeningocele. Ultrasound Obstet Gynecol. 56(5):678-83, 2019
                                        3. Warner HM et al: T2 prolongation in the cerebellar vermis on prenatal MRI of fetuses with Chiari 2 malformations. Clin Radiol. 74(5):408.e19-25, 2019
                                        4. Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-6, 2017
                                        5. 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
                                        6. Kappou D et al: Towards detecting open spina bifida in the first trimester: the examination of the posterior brain. Fetal Diagn Ther. 37(4):294-300, 2015
                                        7. Mignone Philpott C et al: Diffusion-weighted imaging of the cerebellum in the fetus with Chiari II malformation. AJNR Am J Neuroradiol. 34(8):1656-60, 2013
                                        8. Fuchs I et al: Normal intracranial translucency and posterior fossa at 11-13 weeks' gestation in a fetus with closed spina bifida. Ultrasound Obstet Gynecol. 40(2):238-9, 2012
                                        9. Chaoui R et al: Prospective detection of open spina bifida at 11-13 weeks by assessing intracranial translucency and posterior brain. Ultrasound Obstet Gynecol. 38(6):722-6, 2011
                                        10. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 15(1):9-14, 2010
                                        11. Chaoui R et al: Assessment of intracranial translucency (IT) in the detection of spina bifida at the 11-13-week scan. Ultrasound Obstet Gynecol. 34(3):249-52, 2009
                                        12. Wong SK et al: Supratentorial abnormalities in the Chiari II malformationa, III: The interhemispheric cyst. J Ultrasound Med. 28(8):999-1006, 2009
                                        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

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Chiari 2 (Ch2) malformation
                                                    • Open neural tube defect (ONTD)
                                                  • Synonyms

                                                    • Arnold Ch2 malformation
                                                  • Definitions

                                                    • Anomaly of cerebellum, brainstem, and cervical cord associated with small bony posterior fossa and typically seen with ONTD

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Ben Miled S et al: Severe and progressive neuronal loss in myelomeningocele begins before 16 weeks of pregnancy. Am J Obstet Gynecol. 223(2):256.e1-9, 2020
                                                                              2. Donepudi R et al: Trends in ventricle size during pregnancy and its use for prediction of ventriculoperitoneal shunt in fetal myelomeningocele. Ultrasound Obstet Gynecol. 56(5):678-83, 2019
                                                                              3. Warner HM et al: T2 prolongation in the cerebellar vermis on prenatal MRI of fetuses with Chiari 2 malformations. Clin Radiol. 74(5):408.e19-25, 2019
                                                                              4. Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-6, 2017
                                                                              5. 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
                                                                              6. Kappou D et al: Towards detecting open spina bifida in the first trimester: the examination of the posterior brain. Fetal Diagn Ther. 37(4):294-300, 2015
                                                                              7. Mignone Philpott C et al: Diffusion-weighted imaging of the cerebellum in the fetus with Chiari II malformation. AJNR Am J Neuroradiol. 34(8):1656-60, 2013
                                                                              8. Fuchs I et al: Normal intracranial translucency and posterior fossa at 11-13 weeks' gestation in a fetus with closed spina bifida. Ultrasound Obstet Gynecol. 40(2):238-9, 2012
                                                                              9. Chaoui R et al: Prospective detection of open spina bifida at 11-13 weeks by assessing intracranial translucency and posterior brain. Ultrasound Obstet Gynecol. 38(6):722-6, 2011
                                                                              10. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 15(1):9-14, 2010
                                                                              11. Chaoui R et al: Assessment of intracranial translucency (IT) in the detection of spina bifida at the 11-13-week scan. Ultrasound Obstet Gynecol. 34(3):249-52, 2009
                                                                              12. Wong SK et al: Supratentorial abnormalities in the Chiari II malformationa, III: The interhemispheric cyst. J Ultrasound Med. 28(8):999-1006, 2009