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Brain
Diagnosis
Pathology-Based Diagnoses
Congenital Malformations
Chiari Malformations
Chiari 3 Malformation
Chiari 3 Malformation
Kevin R. Moore, MD; Jeffrey S. Ross, MD; Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Chiari III, Chiari 3 malformation (CM3) rhombencephalocele

              • Definitions

                • CM3: Originally described by Dr. Chiari as herniation of cerebellum ± brainstem through posterior C1-2 dysraphic defect
                  • Strict definition suggests CM3 better considered high cervical myelocystocele
                • However, many imagers more loosely define CM3 as low occipital or high cervical meningoencephalocele

              IMAGING

              • General Features

                • CT Findings

                  • MR 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

                                        Selected References

                                        1. Azahraa Haddad F et al: The newer classifications of the chiari malformations with clarifications: an anatomical review. Clin Anat. 31(3):314-22, 2018
                                        2. Ivashchuk G et al: Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst. 31(11):2035-40, 2015
                                        3. Cesmebasi A et al: The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat. 28(2):184-94, 2014
                                        4. Jeong DH et al: Arnold-Chiari malformation type III with meningoencephalocele: a case report. Ann Rehabil Med. 38(3):401-4, 2014
                                        5. Rani H et al: Chiari III malformation: a rare case with review of literature. Fetal Pediatr Pathol. 32(3):169-74, 2013
                                        6. Deans AE et al: Radiology of the Chiari malformations. In Tubbs RS et al: The Chiari Malformations. New York: Springer. 153-69, 2013
                                        7. Agrawal A et al: Chiari III malformation. Pediatr Neurosurg. 47(4):309-10, 2011
                                        8. Ambekar S et al: Large occipito-cervical encephalocele with Chiari III malformation. J Pediatr Neurosci. 6(2):116-7, 2011
                                        9. Erol FS et al: The association of Chiari type III malformation and Klippel-Feil syndrome with mirror movement: a case report. Turk Neurosurg. 21(4):655-8, 2011
                                        10. Garg K et al: Chiari III malformation with proatlas abnormality. Pediatr Neurosurg. 47(4):295-8, 2011
                                        11. Zolal A et al: Identification of the large descending tracts using diffusion tensor imaging in Chiari III malformation. Childs Nerv Syst. 26(7):867-70, 2010
                                        12. Furtado SV et al: Repair of Chiari III malformation using cranioplasty and an occipital rotation flap: technical note and review of literature. Surg Neurol. 72(4):414-7, 2009
                                        13. Işik N et al: Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 45(1):19-28, 2009
                                        14. Işik N et al: Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 45(1):19-28, 2009
                                        15. Chaudhari AM et al: Unique defect representing features of Chiari type III and IV malformations. Pediatr Neurosurg. 44(6):513-4, 2008
                                        16. Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
                                        17. Jaggi RS et al: Chiari malformation type III treated with primary closure. Pediatr Neurosurg. 43(5):424-7, 2007
                                        18. Muzumdar D et al: Type III Chiari malformation presenting as intermittent respiratory stridor: a neurological image. Pediatr Neurosurg. 43(5):446-8, 2007
                                        19. Caldarelli M et al: Chiari type III malformation. Childs Nerv Syst. 18(5):207-10, 2002
                                        20. Castillo M et al: Chiari III malformation: imaging features. AJNR Am J Neuroradiol. 13(1):107-13, 1992
                                        21. Cohen MM Jr et al: Syndromes with cephaloceles. Teratology. 25(2):161-72, 1982
                                        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 III, Chiari 3 malformation (CM3) rhombencephalocele

                                                    • Definitions

                                                      • CM3: Originally described by Dr. Chiari as herniation of cerebellum ± brainstem through posterior C1-2 dysraphic defect
                                                        • Strict definition suggests CM3 better considered high cervical myelocystocele
                                                      • However, many imagers more loosely define CM3 as low occipital or high cervical meningoencephalocele

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR 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

                                                                              Selected References

                                                                              1. Azahraa Haddad F et al: The newer classifications of the chiari malformations with clarifications: an anatomical review. Clin Anat. 31(3):314-22, 2018
                                                                              2. Ivashchuk G et al: Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst. 31(11):2035-40, 2015
                                                                              3. Cesmebasi A et al: The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat. 28(2):184-94, 2014
                                                                              4. Jeong DH et al: Arnold-Chiari malformation type III with meningoencephalocele: a case report. Ann Rehabil Med. 38(3):401-4, 2014
                                                                              5. Rani H et al: Chiari III malformation: a rare case with review of literature. Fetal Pediatr Pathol. 32(3):169-74, 2013
                                                                              6. Deans AE et al: Radiology of the Chiari malformations. In Tubbs RS et al: The Chiari Malformations. New York: Springer. 153-69, 2013
                                                                              7. Agrawal A et al: Chiari III malformation. Pediatr Neurosurg. 47(4):309-10, 2011
                                                                              8. Ambekar S et al: Large occipito-cervical encephalocele with Chiari III malformation. J Pediatr Neurosci. 6(2):116-7, 2011
                                                                              9. Erol FS et al: The association of Chiari type III malformation and Klippel-Feil syndrome with mirror movement: a case report. Turk Neurosurg. 21(4):655-8, 2011
                                                                              10. Garg K et al: Chiari III malformation with proatlas abnormality. Pediatr Neurosurg. 47(4):295-8, 2011
                                                                              11. Zolal A et al: Identification of the large descending tracts using diffusion tensor imaging in Chiari III malformation. Childs Nerv Syst. 26(7):867-70, 2010
                                                                              12. Furtado SV et al: Repair of Chiari III malformation using cranioplasty and an occipital rotation flap: technical note and review of literature. Surg Neurol. 72(4):414-7, 2009
                                                                              13. Işik N et al: Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 45(1):19-28, 2009
                                                                              14. Işik N et al: Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 45(1):19-28, 2009
                                                                              15. Chaudhari AM et al: Unique defect representing features of Chiari type III and IV malformations. Pediatr Neurosurg. 44(6):513-4, 2008
                                                                              16. Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
                                                                              17. Jaggi RS et al: Chiari malformation type III treated with primary closure. Pediatr Neurosurg. 43(5):424-7, 2007
                                                                              18. Muzumdar D et al: Type III Chiari malformation presenting as intermittent respiratory stridor: a neurological image. Pediatr Neurosurg. 43(5):446-8, 2007
                                                                              19. Caldarelli M et al: Chiari type III malformation. Childs Nerv Syst. 18(5):207-10, 2002
                                                                              20. Castillo M et al: Chiari III malformation: imaging features. AJNR Am J Neuroradiol. 13(1):107-13, 1992
                                                                              21. Cohen MM Jr et al: Syndromes with cephaloceles. Teratology. 25(2):161-72, 1982
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