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Chiari 2
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Chiari 2 malformation (CM2), Chiari type II
              • Definitions

                • Complex hindbrain malformation
                  • Virtually 100% associated with neural tube closure defect (NTD), usually lumbar myelomeningocele (MMC)
                    • Rare reports in closed spinal dysraphism (probably misinterpreted Chiari 1)

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Bekiesinska-Figatowska M et al: Stenogyria - Not only in Chiari II malformation. J Neurol Sci. ePub, 2014
                                              2. Satzer D et al: Prognostic value of newborn hearing screening in patients with myelomeningocele. J Neurosurg Pediatr. 1-6, 2014
                                              3. Messing-Jünger M et al: Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 29(9):1553-62, 2013
                                              4. Citton V et al: Chiari 2 without spinal dysraphism: does it blow a hole in the pathogenesis? J Child Neurol. 27(4):536-9, 2012
                                              5. Geerdink N et al: Essential features of Chiari II malformation in MR imaging: an interobserver reliability study--part 1. Childs Nerv Syst. 28(7):977-85, 2012
                                              6. Juranek J et al: The cerebellum in children with spina bifida and Chiari II malformation: Quantitative volumetrics by region. Cerebellum. 9(2):240-8, 2010
                                              7. Luigetti M et al: Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation Type II following surgery. J Neurosurg Pediatr. 6(4):336-9, 2010
                                              8. Morota N et al: Postnatal ascent of the cerebellar tonsils in Chiari malformation Type II following surgical repair of myelomeningocele. J Neurosurg Pediatr. 2(3):188-93, 2008
                                              9. Vinck A et al: Arnold-Chiari-II malformation and cognitive functioning in spina bifida. J Neurol Neurosurg Psychiatry. 77(9):1083-6, 2006
                                              10. McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
                                              11. Tubbs RS et al: Chiari II malformation and occult spinal dysraphism. Case reports and a review of the literature. Pediatr Neurosurg. 39(2):104-7, 2003
                                              12. McDonnell GV et al: Prevalence of the Chiari/hydrosyringomyelia complex in adults with spina bifida: preliminary results. Eur J Pediatr Surg. 10 Suppl 1:18-9, 2000
                                              13. Piatt JH Jr et al: The Chiari II malformation: lesions discovered within the fourth ventricle. Pediatr Neurosurg. 30(2):79-85, 1999
                                              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

                                                          • Synonyms

                                                            • Chiari 2 malformation (CM2), Chiari type II
                                                          • Definitions

                                                            • Complex hindbrain malformation
                                                              • Virtually 100% associated with neural tube closure defect (NTD), usually lumbar myelomeningocele (MMC)
                                                                • Rare reports in closed spinal dysraphism (probably misinterpreted Chiari 1)

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Bekiesinska-Figatowska M et al: Stenogyria - Not only in Chiari II malformation. J Neurol Sci. ePub, 2014
                                                                                          2. Satzer D et al: Prognostic value of newborn hearing screening in patients with myelomeningocele. J Neurosurg Pediatr. 1-6, 2014
                                                                                          3. Messing-Jünger M et al: Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 29(9):1553-62, 2013
                                                                                          4. Citton V et al: Chiari 2 without spinal dysraphism: does it blow a hole in the pathogenesis? J Child Neurol. 27(4):536-9, 2012
                                                                                          5. Geerdink N et al: Essential features of Chiari II malformation in MR imaging: an interobserver reliability study--part 1. Childs Nerv Syst. 28(7):977-85, 2012
                                                                                          6. Juranek J et al: The cerebellum in children with spina bifida and Chiari II malformation: Quantitative volumetrics by region. Cerebellum. 9(2):240-8, 2010
                                                                                          7. Luigetti M et al: Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation Type II following surgery. J Neurosurg Pediatr. 6(4):336-9, 2010
                                                                                          8. Morota N et al: Postnatal ascent of the cerebellar tonsils in Chiari malformation Type II following surgical repair of myelomeningocele. J Neurosurg Pediatr. 2(3):188-93, 2008
                                                                                          9. Vinck A et al: Arnold-Chiari-II malformation and cognitive functioning in spina bifida. J Neurol Neurosurg Psychiatry. 77(9):1083-6, 2006
                                                                                          10. McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
                                                                                          11. Tubbs RS et al: Chiari II malformation and occult spinal dysraphism. Case reports and a review of the literature. Pediatr Neurosurg. 39(2):104-7, 2003
                                                                                          12. McDonnell GV et al: Prevalence of the Chiari/hydrosyringomyelia complex in adults with spina bifida: preliminary results. Eur J Pediatr Surg. 10 Suppl 1:18-9, 2000
                                                                                          13. Piatt JH Jr et al: The Chiari II malformation: lesions discovered within the fourth ventricle. Pediatr Neurosurg. 30(2):79-85, 1999