Bookmarks
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)
- Chiari 2 malformation (CM2): Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism of 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
- 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
Loading...
Related Differential Diagnoses
Loading...
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)
- Chiari 2 malformation (CM2): Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism of 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
- 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
