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
Azahraa Haddad F et al: The newer classifications of the chiari malformations with clarifications: an anatomical review. Clin Anat. 31(3):314-22, 2018
Ivashchuk G et al: Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst. 31(11):2035-40, 2015
Cesmebasi A et al: The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat. 28(2):184-94, 2014
Jeong DH et al: Arnold-Chiari malformation type III with meningoencephalocele: a case report. Ann Rehabil Med. 38(3):401-4, 2014
Rani H et al: Chiari III malformation: a rare case with review of literature. Fetal Pediatr Pathol. 32(3):169-74, 2013
Deans AE et al: Radiology of the Chiari malformations. In Tubbs RS et al: The Chiari Malformations. New York: Springer. 153-69, 2013
Agrawal A et al: Chiari III malformation. Pediatr Neurosurg. 47(4):309-10, 2011
Ambekar S et al: Large occipito-cervical encephalocele with Chiari III malformation. J Pediatr Neurosci. 6(2):116-7, 2011
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
Garg K et al: Chiari III malformation with proatlas abnormality. Pediatr Neurosurg. 47(4):295-8, 2011
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
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
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
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
Chaudhari AM et al: Unique defect representing features of Chiari type III and IV malformations. Pediatr Neurosurg. 44(6):513-4, 2008
Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
Jaggi RS et al: Chiari malformation type III treated with primary closure. Pediatr Neurosurg. 43(5):424-7, 2007
Muzumdar D et al: Type III Chiari malformation presenting as intermittent respiratory stridor: a neurological image. Pediatr Neurosurg. 43(5):446-8, 2007
Caldarelli M et al: Chiari type III malformation. Childs Nerv Syst. 18(5):207-10, 2002
Castillo M et al: Chiari III malformation: imaging features. AJNR Am J Neuroradiol. 13(1):107-13, 1992
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
Azahraa Haddad F et al: The newer classifications of the chiari malformations with clarifications: an anatomical review. Clin Anat. 31(3):314-22, 2018
Ivashchuk G et al: Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst. 31(11):2035-40, 2015
Cesmebasi A et al: The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat. 28(2):184-94, 2014
Jeong DH et al: Arnold-Chiari malformation type III with meningoencephalocele: a case report. Ann Rehabil Med. 38(3):401-4, 2014
Rani H et al: Chiari III malformation: a rare case with review of literature. Fetal Pediatr Pathol. 32(3):169-74, 2013
Deans AE et al: Radiology of the Chiari malformations. In Tubbs RS et al: The Chiari Malformations. New York: Springer. 153-69, 2013
Agrawal A et al: Chiari III malformation. Pediatr Neurosurg. 47(4):309-10, 2011
Ambekar S et al: Large occipito-cervical encephalocele with Chiari III malformation. J Pediatr Neurosci. 6(2):116-7, 2011
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
Garg K et al: Chiari III malformation with proatlas abnormality. Pediatr Neurosurg. 47(4):295-8, 2011
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
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
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
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
Chaudhari AM et al: Unique defect representing features of Chiari type III and IV malformations. Pediatr Neurosurg. 44(6):513-4, 2008
Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
Jaggi RS et al: Chiari malformation type III treated with primary closure. Pediatr Neurosurg. 43(5):424-7, 2007
Muzumdar D et al: Type III Chiari malformation presenting as intermittent respiratory stridor: a neurological image. Pediatr Neurosurg. 43(5):446-8, 2007
Caldarelli M et al: Chiari type III malformation. Childs Nerv Syst. 18(5):207-10, 2002
Castillo M et al: Chiari III malformation: imaging features. AJNR Am J Neuroradiol. 13(1):107-13, 1992
Cohen MM Jr et al: Syndromes with cephaloceles. Teratology. 25(2):161-72, 1982
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