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Abstract

Chiari malformation represents a heterogeneous group of hindbrain malformations with the Chiari type 1 being the most common form. Herein, we attempt a comprehensive overview of the subject aiming to provide the pediatrician with a working knowledge that will help: (1) have an appropriate level of suspicion for the pathology during a workup for headaches or other neurologic symptoms, (2) understand the rationale for operative or conservative treatment, and (3) follow up confidently with pediatric Chiari patients that have been managed either surgically or conservatively.

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References

  1. Tubbs RS, Cohen-Gadol AA. Hans Chiari (1851–1916). J Neurol. 2010;257(7):1218–20.

    Article  PubMed  Google Scholar 

  2. Hans C. Über Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wsch. 1891;17:1172–5.

    Article  Google Scholar 

  3. Miller E, Widjaja E, Blaser S, Dennis M, Raybaud C. The old and the new: supratentorial MR findings in Chiari II malformation. Childs Nerv Syst. 2008;24(5):563–75.

    Article  PubMed  Google Scholar 

  4. Citton V, Toldo I, Balao L, Pettenazzo A, Emanuelli E, Sartori S, et al. Chiari 2 without spinal dysraphism: does it blow a hole in the pathogenesis? J Child Neurol. 2012;27(4):536–9.

    Article  PubMed  Google Scholar 

  5. Juranek J, Salman MS. Anomalous development of brain structure and function in spina bifida myelomeningocele. Dev Disabil Res Rev. 2010;16(1):23–30.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Chern JJ, Gordon AJ, Mortazavi MM, Tubbs RS, Oakes WJ. Pediatric Chiari malformation Type 0: a 12-year institutional experience. J Neurosurg Pediatr. 2011;8(1):1–5.

    Article  PubMed  Google Scholar 

  7. Tubbs RS, Muhleman M, Loukas M, Oakes WJ. A new form of herniation: the Chiari V malformation. Childs Nerv Syst. 2012;28(2):305–7.

    Article  PubMed  Google Scholar 

  8. Tubbs RS, Shoja MM, Ardalan MR, Shokouhi G, Loukas M. Hindbrain herniation: a review of embryological theories. Ital J Anat Embryol. 2008;113(1):37–46.

    PubMed  Google Scholar 

  9. Waldau B, Grant G, Fuchs H. Development of an acquired Chiari malformation Type I in the setting of an untreated lipomyelomeningocele. Case report. J Neurosurg Pediatr. 2008;1(2):164–6.

    Article  PubMed  Google Scholar 

  10. Massimi L, Novegno F, di Rocco C. Chiari type I malformation in children. Adv Tech Stand Neurosurg. 2011;37:143–211.

    PubMed  Google Scholar 

  11. Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir (Wien). 2010;152(7):1117–27.

    Article  Google Scholar 

  12. Linge SO, Mardal KA, Haughton V, Helgeland A. Simulating CSF flow dynamics in the normal and the Chiari I subarachnoid space during rest and exertion. AJNR Am J Neuroradiol. 2013;34(1):41–5.

    Article  CAS  PubMed  Google Scholar 

  13. Dagtekin A, Avci E, Kara E, Uzmansel D, Dagtekin O, Koseoglu A, et al. Posterior cranial fossa morphometry in symptomatic adult Chiari I malformation patients: comparative clinical and anatomical study. Clin Neurol Neurosurg. 2011;113(5):399–403.

    Article  PubMed  Google Scholar 

  14. Chang HS, Tsuchiya T, Fujisawa N, Oya S, Matsui T. Syringomyelia associated with arachnoid septum at the craniovertebral junction, contradicting the currently prevailing theory of syringomyelia formation. Acta Neurochir (Wien). 2012;154(1):141–5. discussion 5.

    Article  Google Scholar 

  15. Heiss JD, Suffredini G, Bakhtian KD, Sarntinoranont M, Oldfield EH. Normalization of hindbrain morphology after decompression of Chiari malformation Type I. J Neurosurg. 2012;117(5):942–6.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Markunas CA, Tubbs RS, Moftakhar R, Ashley-Koch AE, Gregory SG, Oakes WJ, et al. Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations. J Neurosurg Pediatr. 2012;9(4):372–8.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005–17.

    Article  CAS  PubMed  Google Scholar 

  18. Schanker BD, Walcott BP, Nahed BV, Kahle KT, Li YM, Coumans JV. Familial Chiari malformation: case series. Neurosurg Focus. 2011;31(3):E1.

    Article  PubMed  Google Scholar 

  19. Urbizu A, Toma C, Poca MA, Sahuquillo J, Cuenca-Leon E, Cormand B, et al. Chiari malformation type I: a case-control association study of 58 developmental genes. PLoS One. 2013;8(2):e57241.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, Sorel M, et al. Chiari type I malformation in a pediatric population. Pediatr Neurol. 2009;40(6):449–54.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS. Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92(6):920–6.

    Article  CAS  PubMed  Google Scholar 

  22. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons III JC, Rozzelle CJ, et al. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I. J Neurosurg Pediatr. 2011;7(3):248–56.

    Article  PubMed  Google Scholar 

  23. Headache Classification Subcommittee of the International Headache Society. The International classification of headache disorders: 2nd edition. Cephalalgia. 2004;24 Suppl 1:9–160.

    Google Scholar 

  24. Pandey A, Robinson S, Cohen AR. Cerebellar fits in children with Chiari I malformation. Neurosurg Focus. 2001;11(1):E4.

    Article  CAS  PubMed  Google Scholar 

  25. Greenlee JD, Donovan KA, Hasan DM, Menezes AH. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics. 2002;110(6):1212–9.

    Article  PubMed  Google Scholar 

  26. Carew CL, Prasad A, Tay KY, de Ribaupierre S. Unusual presentation of Chiari I in toddlers: case reports and review of the literature. Childs Nerv Syst. 2012;28(11):1965–70.

    Article  PubMed  Google Scholar 

  27. Goodwin D, Halvorson AR. Chiari I malformation presenting as downbeat nystagmus: clinical presentation, diagnosis, and management. Optometry. 2012;83(2):80–6.

    PubMed  Google Scholar 

  28. Benglis D, Levi AD. Neurologic findings of craniovertebral junction disease. Neurosurgery. 2010;66(3 Suppl):13–21.

    Article  PubMed  Google Scholar 

  29. Baxstrom CR. Nonsurgical treatment for esotropia secondary to Arnold-Chiari I malformation: a case report. Optometry. 2009;80(9):472–8.

    Article  PubMed  Google Scholar 

  30. Gonzalez-Bonet LG, Piquer J. Trigeminal neuralgia: a symptom of Chiari I malformation. Neurosurgery. 2012;71(4):E911–2.

    Article  PubMed  Google Scholar 

  31. Than KD, Sharifpour M, Wang AC, Thompson BG, Pandey AS. Chiari I malformation manifesting as bilateral trigeminal neuralgia: case report and review of the literature. J Neurol Neurosurg Psychiatry. 2011;82(9):1058–9.

    Article  PubMed  Google Scholar 

  32. Li F, Yang Y, Liu Y, Li Q, Zhu S. Glossopharyngeal neuralgia as onset of Chiari Type I malformation. Headache. 2012;52(10):1576–8.

    Article  PubMed  Google Scholar 

  33. Laufer I, Engel M, Feldstein N, Souweidane MM. Chiari malformation presenting as a focal motor deficit. Report of two cases. J Neurosurg Pediatr. 2008;1(5):392–5.

    Article  PubMed  Google Scholar 

  34. Brockmeyer DL. Editorial. Chiari malformation Type I and scoliosis: the complexity of curves. J Neurosurg Pediatr. 2011;7(1):22–3. discussion 3–4.

    Article  PubMed  Google Scholar 

  35. Attenello FJ, McGirt MJ, Atiba A, Gathinji M, Datoo G, Weingart J, et al. Suboccipital decompression for Chiari malformation-associated scoliosis: risk factors and time course of deformity progression. J Neurosurg Pediatr. 2008;1(6):456–60.

    Article  PubMed  Google Scholar 

  36. Iber C, American Academy of Sleep Medicine. The AASM manual for the scoring of sleep and associated events: rules, terminology and technical specifications. Westchester, IL: American Academy of Sleep Medicine; 2007.

    Google Scholar 

  37. Aarts LA, Willemsen MA, Vandenbussche NL, van Gent R. Nocturnal apnea in Chiari type I malformation. Eur J Pediatr. 2011;170(10):1349–52.

    Article  PubMed  Google Scholar 

  38. Botelho RV, Bittencourt LR, Rotta JM, Tufik S. The effects of posterior fossa decompressive surgery in adult patients with Chiari malformation and sleep apnea. J Neurosurg. 2010;112(4):800–7.

    Article  PubMed  Google Scholar 

  39. Dauvilliers Y, Stal V, Abril B, Coubes P, Bobin S, Touchon J, et al. Chiari malformation and sleep related breathing disorders. J Neurol Neurosurg Psychiatry. 2007;78(12):1344–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Granata T, Valentini LG. Epilepsy in type 1 Chiari malformation. Neurol Sci. 2011;32 Suppl 3:S303–6.

    Article  PubMed  Google Scholar 

  41. Pettorini BL, Gao A, Rodrigues D. Acute deterioration of a Chiari I malformation: an uncommon neurosurgical emergency. Childs Nerv Syst. 2011;27(6):857–60.

    Article  PubMed  Google Scholar 

  42. Wellons III JC, Tubbs RS, Bui CJ, Grabb PA, Oakes WJ. Urgent surgical intervention in pediatric patients with Chiari malformation type I. Report of two cases. J Neurosurg. 2007;107(1 Suppl):49–52.

    PubMed  Google Scholar 

  43. Loukas M, Shayota BJ, Oelhafen K, Miller JH, Chern JJ, Tubbs RS, et al. Associated disorders of Chiari Type I malformations: a review. Neurosurg Focus. 2011;31(3):E3.

    Article  PubMed  Google Scholar 

  44. Welsch M, Antes S, Kiefer M, Meyer S, Eymann R. Association of Chiari malformation and vitamin B12 deficit in a family. Childs Nerv Syst. 2013.

    Google Scholar 

  45. Weaver J, Manjila S, Bahuleyan B, Bangert BA, Cohen AR. Rhombencephalosynapsis: embryopathology and management strategies of associated neurosurgical conditions with a review of the literature. J Neurosurg Pediatr. 2013;11(3):320–6.

    Article  PubMed  Google Scholar 

  46. Albert GW, Menezes AH, Hansen DR, Greenlee JD, Weinstein SL. Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome. J Neurosurg Pediatr. 2010;5(6):554–61.

    Article  PubMed  Google Scholar 

  47. Raieli V, Eliseo M, Pandolfi E, La Vecchia M, La Franca G, Puma D, et al. Recurrent and chronic headaches in children below 6 years of age. J Headache Pain. 2005;6(3):135–42.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Cinalli G, Chumas P, Arnaud E, Sainte-Rose C, Renier D. Occipital remodeling and suboccipital decompression in severe craniosynostosis associated with tonsillar herniation. Neurosurgery. 1998;42(1):66–71. discussion 71–3.

    Article  CAS  PubMed  Google Scholar 

  49. Addo NK, Javadpour S, Kandasamy J, Sillifant P, May P, Sinha A. Central sleep apnea and associated Chiari malformation in children with syndromic craniosynostosis: treatment and outcome data from a supraregional national craniofacial center. J Neurosurg Pediatr. 2013;11(3):296–301.

    Article  PubMed  Google Scholar 

  50. Spence J, Pasterkamp H, McDonald PJ. Isolated central sleep apnea in type I Chiari malformation: improvement after surgery. Pediatr Pulmonol. 2010;45(11):1141–4.

    Article  PubMed  Google Scholar 

  51. Leikola J, Koljonen V, Valanne L, Hukki J. The incidence of Chiari malformation in nonsyndromic, single suture craniosynostosis. Childs Nerv Syst. 2010;26(6):771–4.

    Article  PubMed  Google Scholar 

  52. Suzuki H, Takanashi J, Kobayashi K, Nagasawa K, Tashima K, Kohno Y. MR imaging of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2001;22(1):196–9.

    CAS  PubMed  Google Scholar 

  53. Aiken AH, Hoots JA, Saindane AM, Hudgins PA. Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol. 2012;33(10):1901–6.

    Article  CAS  PubMed  Google Scholar 

  54. Aquilina K, Merchant TE, Boop FA, Sanford RA. Chiari I malformation after cranial radiation therapy in childhood: a dynamic process associated with changes in clival growth. Childs Nerv Syst. 2009;25(11):1429–36.

    Article  PubMed  Google Scholar 

  55. Peleggi AF, Lovely TJ. Treatment of delayed Chiari malformation and syringomyelia after lumboperitoneal shunt placement: case report and treatment recommendations. Surg Neurol Int. 2012;3:101.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Caldarelli M, Novegno F, Di Rocco C. A late complication of CSF shunting: acquired Chiari I malformation. Childs Nerv Syst. 2009;25(4):443–52.

    Article  PubMed  Google Scholar 

  57. Milhorat TH, Bolognese PA, Nishikawa M, Francomano CA, McDonnell NB, Roonprapunt C, et al. Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg Neurol. 2009;72(1):20–35.

    Article  PubMed  Google Scholar 

  58. Di Rocco C, Frassanito P, Massimi L, Peraio S. Hydrocephalus and Chiari type I malformation. Childs Nerv Syst. 2011;27(10):1653–64.

    Article  PubMed  Google Scholar 

  59. Gupta A, Vitali AM, Rothstein R, Cochrane DD. Resolution of syringomyelia and Chiari malformation after growth hormone therapy. Childs Nerv Syst. 2008;24(11):1345–8.

    Article  PubMed  Google Scholar 

  60. Naftel RP, Tubbs RS, Menendez JY, Oakes WJ. Progression of Chiari I malformations while on growth hormone replacement: a report of two cases. Childs Nerv Syst. 2013;29(12):2291–4.

    Article  PubMed  Google Scholar 

  61. Manara R, Bommarito G, Rizzati S, Briani C, Della Puppa A, Citton V, et al. Herniation of cerebellar tonsils in acromegaly: prevalence, pathogenesis and clinical impact. Pituitary. 2013;16(1):122–30.

    Article  PubMed  Google Scholar 

  62. Alsaadi MM, Iqbal SM, Elgamal EA, Gozal D. Sleep-disordered breathing in children with Chiari malformation type II and myelomeningocele. Pediatr Int. 2012;54(5):623–6.

    Article  PubMed  Google Scholar 

  63. Stevenson KL. Chiari Type II malformation: past, present, and future. Neurosurg Focus. 2004;16(2):E5.

    Article  PubMed  Google Scholar 

  64. Chiapparini L, Saletti V, Solero CL, Bruzzone MG, Valentini LG. Neuroradiological diagnosis of Chiari malformations. Neurol Sci. 2011;32 Suppl 3:S283–6.

    Article  PubMed  Google Scholar 

  65. Galarza M, Martinez-Lage JF, Ham S, Sood S. Cerebral anomalies and Chiari type 1 malformation. Pediatr Neurosurg. 2010;46(6):442–9.

    Article  PubMed  Google Scholar 

  66. Goh S, Bottrell CL, Aiken AH, Dillon WP, Wu YW. Presyrinx in children with Chiari malformations. Neurology. 2008;71(5):351–6.

    Article  CAS  PubMed  Google Scholar 

  67. Silva JA, Melo LR, Araujo AF, Santos Jr AA. Resolution of syringomyelia in ten cases of “up-and-down Chiari malformation” after posterior fossa decompression. Arq Neuropsiquiatr. 2010;68(5):694–9.

    Article  PubMed  Google Scholar 

  68. Zhu Z, Wu T, Sha S, Sun X, Zhu F, Qian B, et al. Is curve direction correlated with the dominant side of tonsillar ectopia and side of syrinx deviation in patients with single thoracic scoliosis secondary to Chiari malformation and syringomyelia? Spine (Phila Pa 1976). 2013;38(8):671–7.

    Article  Google Scholar 

  69. Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA. Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine. 2007;7(6):601–9.

    Article  PubMed  Google Scholar 

  70. Brennan CM, Taylor GA. Sonographic imaging of the posterior fossa utilizing the foramen magnum. Pediatr Radiol. 2010;40(8):1411–6.

    Article  PubMed  Google Scholar 

  71. Benglis Jr D, Covington D, Bhatia R, Bhatia S, Elhammady MS, Ragheb J, et al. Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery. J Neurosurg Pediatr. 2011;7(4):375–9.

    Article  PubMed  Google Scholar 

  72. Miller JH, Limbrick DD, Callen M, Smyth MD. Spontaneous resolution of Chiari malformation Type I in monozygotic twins. J Neurosurg Pediatr. 2008;2(5):317–9.

    Article  PubMed  Google Scholar 

  73. Waldau B, Domeshek LF, Leigh FA, Lum KC, Fuchs HE, Marcus JR, et al. Spontaneous resolution of a 13-mm Chiari malformation Type I in relation to differential growth of the posterior fossa volume. J Neurosurg Pediatr. 2009;3(2):110–4.

    Article  PubMed  Google Scholar 

  74. Khatwa U, Ramgopal S, Mylavarapu A, Prabhu SP, Smith E, Proctor M, et al. MRI findings and sleep apnea in children with Chiari I malformation. Pediatr Neurol. 2013;48(4):299–307.

    Article  PubMed  Google Scholar 

  75. Grammatica A, Bonali M, Ruscitti F, Marchioni D, Pinna G, Cunsolo EM, et al. Transnasal endoscopic removal of malformation of the odontoid process in a patient with type I Arnold-Chiari malformation: a case report. Acta Otorhinolaryngol Ital. 2011;31(4):248–52.

    CAS  PubMed  PubMed Central  Google Scholar 

  76. Fenoy AJ, Menezes AH, Fenoy KA. Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia. J Neurosurg Spine. 2008;9(1):1–9.

    Article  PubMed  Google Scholar 

  77. Massimi L, Pravata E, Tamburrini G, Gaudino S, Pettorini B, Novegno F, et al. Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery. 2011;68(4):950–6.

    PubMed  Google Scholar 

  78. Colombo LF, Motta F. Consensus conference on Chiari: a malformation or an anomaly? Scoliosis and others orthopaedic deformities related to Chiari 1 malformation. Neurol Sci. 2011;32 Suppl 3:S341–3.

    Article  PubMed  Google Scholar 

  79. McGirt MJ, Attenello FJ, Atiba A, Garces-Ambrossi G, Datoo G, Weingart JD, et al. Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Childs Nerv Syst. 2008;24(11):1333–9.

    Article  PubMed  Google Scholar 

  80. Massimi L, Della Pepa GM, Tamburrini G, Di Rocco C. Sudden onset of Chiari malformation Type I in previously asymptomatic patients. J Neurosurg Pediatr. 2011;8(5):438–42.

    Article  PubMed  Google Scholar 

  81. Wan MJ, Nomura H, Tator CH. Conversion to symptomatic Chiari I malformation after minor head or neck trauma. Neurosurgery. 2008;63(4):748–53. discussion 53.

    Article  PubMed  Google Scholar 

  82. Yarbrough CK, Powers AK, Park TS, Leonard JR, Limbrick DD, Smyth MD. Patients with Chiari malformation Type I presenting with acute neurological deficits: case series. J Neurosurg Pediatr. 2011;7(3):244–7.

    Article  PubMed  Google Scholar 

  83. Callaway GH, O’Brien SJ, Tehrany AM. Chiari I malformation and spinal cord injury: cause for concern in contact athletes? Med Sci Sports Exerc. 1996;28(10):1218–20.

    Article  CAS  PubMed  Google Scholar 

  84. Wolf DA, Veasey III SP, Wilson SK, Adame J, Korndorffer WE. Death following minor head trauma in two adult individuals with the Chiari I deformity. J Forensic Sci. 1998;43(6):1241–3.

    Article  CAS  PubMed  Google Scholar 

  85. Zhang J, Shao Y, Qin Z, Liu N, Zou D, Huang P, et al. Sudden unexpected death due to Chiari Type I malformation in a road accident case. J Forensic Sci. 2013;58(2):540–3.

    Article  PubMed  Google Scholar 

  86. James DS. Significance of chronic tonsillar herniation in sudden death. Forensic Sci Int. 1995;75(2–3):217–23.

    Article  CAS  PubMed  Google Scholar 

  87. Chauvet D, Carpentier A, George B. Dura splitting decompression in Chiari type 1 malformation: clinical experience and radiological findings. Neurosurg Rev. 2009;32(4):465–70.

    Article  PubMed  Google Scholar 

  88. Chou YC, Sarkar R, Osuagwu FC, Lazareff JA. Suboccipital craniotomy in the surgical treatment of Chiari I malformation. Childs Nerv Syst. 2009;25(9):1111–4.

    Article  PubMed  PubMed Central  Google Scholar 

  89. Durham SR, Fjeld-Olenec K. Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr. 2008;2(1):42–9.

    Article  PubMed  Google Scholar 

  90. Hankinson T, Tubbs RS, Wellons JC. Duraplasty or not? An evidence-based review of the pediatric Chiari I malformation. Childs Nerv Syst. 2011;27(1):35–40.

    Article  PubMed  Google Scholar 

  91. Hoffman CE, Souweidane MM. Cerebrospinal fluid-related complications with autologous duraplasty and arachnoid sparing in type I Chiari malformation. Neurosurgery. 2008;62(3 Suppl 1):156–60. discussion 60–1.

    PubMed  Google Scholar 

  92. Abla AA, Link T, Fusco D, Wilson DA, Sonntag VK. Comparison of dural grafts in Chiari decompression surgery: review of the literature. J Craniovertebr Junction Spine. 2010;1(1):29–37.

    Article  PubMed  PubMed Central  Google Scholar 

  93. Pollack IF, Pang D, Albright AL, Krieger D. Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg. 1992;77(6):881–8.

    Article  CAS  PubMed  Google Scholar 

  94. Akbari SH, Limbrick Jr DD, Kim DH, Narayan P, Leonard JR, Smyth MD, et al. Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 2013;29(7):1143–54.

    Article  PubMed  Google Scholar 

  95. Talamonti G, Zella S. Surgical treatment of CM2 and syringomyelia in a series of 231 myelomeningocele patients. Neurol Sci. 2011;32 Suppl 3:S331–3.

    Article  PubMed  Google Scholar 

  96. Choi D. Failed foramen magnum decompression for Chiari malformation: the challenge of post-operative brainstem shift and cerebellar ptosis. World Neurosurg. 2013;81(5–6):702–5.

    PubMed  Google Scholar 

  97. Klekamp J. Surgical treatment of Chiari I malformation—analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. Neurosurgery. 2012;71(2):365–80. discussion 80.

    Article  PubMed  Google Scholar 

  98. Filis AK, Moon K, Cohen AR. Symptomatic subdural hygroma and hydrocephalus following Chiari I decompression. Pediatr Neurosurg. 2009;45(6):425–8.

    Article  PubMed  Google Scholar 

  99. Furtado SV, Visvanathan K, Reddy K, Hegde AS. Pseudotumor cerebri: as a cause for early deterioration after Chiari I malformation surgery. Childs Nerv Syst. 2009;25(8):1007–12.

    Article  PubMed  Google Scholar 

  100. Vadivelu S, Bolognese P, Milhorat TH, Mogilner AY. Occipital neuromodulation for refractory headache in the Chiari malformation population. Prog Neurol Surg. 2011;24:118–25.

    Article  PubMed  Google Scholar 

  101. Vadivelu S, Bolognese P, Milhorat TH, Mogilner AY. Occipital nerve stimulation for refractory headache in the Chiari malformation population. Neurosurgery. 2012;70(6):1430–6. discussion 6–7.

    Article  PubMed  Google Scholar 

  102. Williams B. A critical appraisal of posterior fossa surgery for communicating syringomyelia. Brain. 1978;101(2):223–50.

    Article  CAS  PubMed  Google Scholar 

  103. Udani V, Holly LT, Chow D, Batzdorf U. Posterior fossa reconstruction using titanium plate for the treatment of cerebellar ptosis after decompression for Chiari malformation. World Neurosurg. 2014;81(5–6):836–41.

    Article  PubMed  Google Scholar 

  104. Di X, Luciano MG, Benzel EC. Acute respiratory arrest following partial suboccipital cranioplasty for cerebellar ptosis from Chiari malformation decompression. Neurosurg Focus. 2008;25(6):E12.

    Article  PubMed  Google Scholar 

  105. Krieger MD, Falkinstein Y, Bowen IE, Tolo VT, McComb JG. Scoliosis and Chiari malformation Type I in children. J Neurosurg Pediatr. 2011;7(1):25–9.

    Article  PubMed  Google Scholar 

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Margetis, K., Greenfield, J.P. (2017). Chiari Malformation. In: Greenfield, J., Long, C. (eds) Common Neurosurgical Conditions in the Pediatric Practice. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-3807-0_21

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