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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Chiari 2 Malformation, Chiari type 2, Arnold-Chiari malformation
Definitions
Chiari 2 malformation: Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, in setting of open spinal dysraphism can be either myelomeningocele (MMC) or myelocele/myeloschisis
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
Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-36, 2017
Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 16(6):613-20, 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 ed. 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
Diagnostic Checklist
TERMINOLOGY
Synonyms
Chiari 2 Malformation, Chiari type 2, Arnold-Chiari malformation
Definitions
Chiari 2 malformation: Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, in setting of open spinal dysraphism can be either myelomeningocele (MMC) or myelocele/myeloschisis
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
Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-36, 2017
Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 16(6):613-20, 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 ed. 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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