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
Taylor DG et al: Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. J Neurosurg. 1-6, 2020
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
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
Poretti A et al: Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 47(5):293-307, 2016
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
Brockmeyer DL et al: Complex Chiari malformations in children: diagnosis and management. Neurosurg Clin N Am. 26(4):555-60, 2015
Leonard JR et al: Chiari I malformation: adult and pediatric considerations. Neurosurg Clin N Am. 26(4):xiii-xiv, 2015
Rozenfeld M et al: MRI findings after surgery for Chiari malformation type I. AJR Am J Roentgenol. 205(5):1086-93, 2015
Strahle J et al: The association between Chiari malformation Type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr. 15(6):607-11, 2015
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
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
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
McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
Moore HE et al: Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. 44(11):1403-11, 2014
Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 491-96, 2012
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
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
Brockmeyer DL: The complex Chiari: issues and management strategies. Neurol Sci. 32 Suppl 3:S345-7, 2011
Strahle J et al: Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 8(2):214-21, 2011
Strahle J et al: Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 8(2):205-13, 2011
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
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
Tubbs RS et al: A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 101(2 Suppl):179-83, 2004
Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 1999
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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
Taylor DG et al: Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. J Neurosurg. 1-6, 2020
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
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
Poretti A et al: Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 47(5):293-307, 2016
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
Brockmeyer DL et al: Complex Chiari malformations in children: diagnosis and management. Neurosurg Clin N Am. 26(4):555-60, 2015
Leonard JR et al: Chiari I malformation: adult and pediatric considerations. Neurosurg Clin N Am. 26(4):xiii-xiv, 2015
Rozenfeld M et al: MRI findings after surgery for Chiari malformation type I. AJR Am J Roentgenol. 205(5):1086-93, 2015
Strahle J et al: The association between Chiari malformation Type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr. 15(6):607-11, 2015
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
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
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
McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
Moore HE et al: Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. 44(11):1403-11, 2014
Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 491-96, 2012
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
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
Brockmeyer DL: The complex Chiari: issues and management strategies. Neurol Sci. 32 Suppl 3:S345-7, 2011
Strahle J et al: Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 8(2):214-21, 2011
Strahle J et al: Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 8(2):205-13, 2011
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
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
Tubbs RS et al: A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 101(2 Suppl):179-83, 2004
Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 1999
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