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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Chiari type II, Arnold-Chiari malformation
Definitions
Chiari 2 malformation (CM2): Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism of myelomeningocele (MMC) or myelocele/myeloschisis
Vast majority of patients with MMC have CM2
Few case reports of CM2 without MMC have other congenital CSF leaks (e.g., patent craniopharyngeal duct)
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
Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 1-8, 2015
Kobraei EM et al: A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 30(9):1535-41, 2014
Kshettry VR et al: Myelomeningocele: surgical trends and predictors of outcome in the United States, 1988-2010. J Neurosurg Pediatr. 13(6):666-78, 2014
Akbari SH et al: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 29(7):1143-54, 2013
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
Sweeney KJ et al: Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia. J Neurosurg Pediatr. 11(2):154-9, 2013
Barkovich AJ et al: Pediatric Neuroimaging, 5th edition. Philadelphia: Lippincott Williams & Wilkins. 497-501, 2012
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
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
Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
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
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
Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
McLone DG et al: The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 15(1):1-12, 1989
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
TERMINOLOGY
Synonyms
Chiari type II, Arnold-Chiari malformation
Definitions
Chiari 2 malformation (CM2): Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism of myelomeningocele (MMC) or myelocele/myeloschisis
Vast majority of patients with MMC have CM2
Few case reports of CM2 without MMC have other congenital CSF leaks (e.g., patent craniopharyngeal duct)
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
Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 1-8, 2015
Kobraei EM et al: A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 30(9):1535-41, 2014
Kshettry VR et al: Myelomeningocele: surgical trends and predictors of outcome in the United States, 1988-2010. J Neurosurg Pediatr. 13(6):666-78, 2014
Akbari SH et al: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 29(7):1143-54, 2013
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
Sweeney KJ et al: Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia. J Neurosurg Pediatr. 11(2):154-9, 2013
Barkovich AJ et al: Pediatric Neuroimaging, 5th edition. Philadelphia: Lippincott Williams & Wilkins. 497-501, 2012
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
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
Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
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
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
Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
McLone DG et al: The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 15(1):1-12, 1989
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