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Brain
Diagnosis
Pathology-Based Diagnoses
Congenital Malformations
Chiari Malformations
Chiari 1 Malformation
Chiari 1 Malformation
Usha D. Nagaraj, MD; Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Chiari type 1, Chiari 1 deformity

              • Definitions

                • Chiari 1 malformation (CM1): Compressed & pointed cerebellar tonsils extending below foramen magnum with effacement of CSF spaces

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT 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

                                          Selected References

                                          1. Taylor DG et al: Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. J Neurosurg. 1-6, 2020
                                          2. Dangouloff-Ros V et al: Incidental brain MRI findings in children: a systematic review and meta-analysis. AJNR Am J Neuroradiol. 40(11):1818-23, 2019
                                          3. Saletti V et al: Chiari I malformation in defined genetic syndromes in children: are there common pathways? Childs Nerv Syst. 35(10):1727-39, 2019
                                          4. Poretti A et al: Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 47(5):293-307, 2016
                                          5. Arnautovic A et al: Pediatric and adult Chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 15(2):161-77, 2015
                                          6. Brockmeyer DL et al: Complex Chiari malformations in children: diagnosis and management. Neurosurg Clin N Am. 26(4):555-60, 2015
                                          7. Leonard JR et al: Chiari I malformation: adult and pediatric considerations. Neurosurg Clin N Am. 26(4):xiii-xiv, 2015
                                          8. Rozenfeld M et al: MRI findings after surgery for Chiari malformation type I. AJR Am J Roentgenol. 205(5):1086-93, 2015
                                          9. Strahle J et al: The association between Chiari malformation Type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr. 15(6):607-11, 2015
                                          10. Godzik J et al: Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. J Neurosurg Pediatr. 13(4):368-74, 2014
                                          11. Lee S et al: Surgical outcome of Chiari I malformation in children: clinico-radiological factors and technical aspects. Childs Nerv Syst. 30(4):613-23, 2014
                                          12. Markunas CA et al: Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann Hum Genet. 78(1):1-12, 2014
                                          13. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                          14. Moore HE et al: Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. 44(11):1403-11, 2014
                                          15. Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 491-96, 2012
                                          16. Bollo RJ et al: Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr. 10(2):134-41, 2012
                                          17. Hwang SW et al: Outcomes of Chiari I-associated scoliosis after intervention: a meta-analysis of the pediatric literature. Childs Nerv Syst. 28(8):1213-9, 2012
                                          18. Brockmeyer DL: The complex Chiari: issues and management strategies. Neurol Sci. 32 Suppl 3:S345-7, 2011
                                          19. Strahle J et al: Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 8(2):214-21, 2011
                                          20. Strahle J et al: Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 8(2):205-13, 2011
                                          21. Tubbs RS et al: Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr. 7(3):248-56, 2011
                                          22. Hofkes SK et al: Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 245(2):532-40, 2007
                                          23. Tubbs RS et al: A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 101(2 Suppl):179-83, 2004
                                          24. Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 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 type 1, Chiari 1 deformity

                                                      • Definitions

                                                        • Chiari 1 malformation (CM1): Compressed & pointed cerebellar tonsils extending below foramen magnum with effacement of CSF spaces

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT 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

                                                                                  Selected References

                                                                                  1. Taylor DG et al: Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. J Neurosurg. 1-6, 2020
                                                                                  2. Dangouloff-Ros V et al: Incidental brain MRI findings in children: a systematic review and meta-analysis. AJNR Am J Neuroradiol. 40(11):1818-23, 2019
                                                                                  3. Saletti V et al: Chiari I malformation in defined genetic syndromes in children: are there common pathways? Childs Nerv Syst. 35(10):1727-39, 2019
                                                                                  4. Poretti A et al: Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 47(5):293-307, 2016
                                                                                  5. Arnautovic A et al: Pediatric and adult Chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 15(2):161-77, 2015
                                                                                  6. Brockmeyer DL et al: Complex Chiari malformations in children: diagnosis and management. Neurosurg Clin N Am. 26(4):555-60, 2015
                                                                                  7. Leonard JR et al: Chiari I malformation: adult and pediatric considerations. Neurosurg Clin N Am. 26(4):xiii-xiv, 2015
                                                                                  8. Rozenfeld M et al: MRI findings after surgery for Chiari malformation type I. AJR Am J Roentgenol. 205(5):1086-93, 2015
                                                                                  9. Strahle J et al: The association between Chiari malformation Type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr. 15(6):607-11, 2015
                                                                                  10. Godzik J et al: Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. J Neurosurg Pediatr. 13(4):368-74, 2014
                                                                                  11. Lee S et al: Surgical outcome of Chiari I malformation in children: clinico-radiological factors and technical aspects. Childs Nerv Syst. 30(4):613-23, 2014
                                                                                  12. Markunas CA et al: Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann Hum Genet. 78(1):1-12, 2014
                                                                                  13. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                                                                  14. Moore HE et al: Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. 44(11):1403-11, 2014
                                                                                  15. Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 491-96, 2012
                                                                                  16. Bollo RJ et al: Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr. 10(2):134-41, 2012
                                                                                  17. Hwang SW et al: Outcomes of Chiari I-associated scoliosis after intervention: a meta-analysis of the pediatric literature. Childs Nerv Syst. 28(8):1213-9, 2012
                                                                                  18. Brockmeyer DL: The complex Chiari: issues and management strategies. Neurol Sci. 32 Suppl 3:S345-7, 2011
                                                                                  19. Strahle J et al: Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 8(2):214-21, 2011
                                                                                  20. Strahle J et al: Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 8(2):205-13, 2011
                                                                                  21. Tubbs RS et al: Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr. 7(3):248-56, 2011
                                                                                  22. Hofkes SK et al: Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 245(2):532-40, 2007
                                                                                  23. Tubbs RS et al: A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 101(2 Suppl):179-83, 2004
                                                                                  24. Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 1999