Abstract
Purpose
To review the natural history of asymptomatic and symptomatic pediatric Chiari I malformations with and without syringomyelia.
Materials and methods
We reviewed the literature for case reports and case series describing the natural history of asymptomatic and symptomatic children with Chiari I malformations with and without syringomyelia. Our review included approximately 700 asymptomatic children without syringomyelia, 100 symptomatic children without syringomyelia, 22 asymptomatic children with syringomyelia, and 11 symptomatic children with syringomyelia. Symptomatic and imaging outcomes at the point of last reported follow-up were noted to describe the natural history of Chiari I malformations in children.
Results
Our review of about 700 asymptomatic children with CM-I without syrinx revealed that most children do not exhibit new-onset symptoms (5–6%) or syrinx (2–3%). The nearly 100 published cases of symptomatic CM-I without syrinx suggest that about half of children report symptomatic improvement (48%) and few report symptomatic worsening (7%). New-onset syrinx is rarely observed (2%). Few cases have been published about asymptomatic and symptomatic CM-I with syrinx as syringomyelia are generally regarded to be an indication for surgical intervention. Nevertheless, all 22 children with asymptomatic CM-I with syringomyelia included in this study were asymptomatic at follow-up, with syrinx resolution observed in 18 children and tonsillar herniation improvement observed in 16 children. Overall, our review of asymptomatic pediatric CM-I with or without syringomyelia suggests that its natural history is much more favorable than previously acknowledged and that the literature generally favors conservative management of these cases.
Conclusion
Our review of asymptomatic pediatric CM-I with or without syringomyelia suggests that its natural history is much more favorable than previously acknowledged and that the literature generally favors conservative management of these cases. Further study of symptomatic pediatric CM-I is necessary to better understand its natural history.
Similar content being viewed by others
References
Schijman E (2004) History, anatomic forms, and pathogenesis of Chiari I malformations. Child Nerv Syst : ChNS : Off J Int Soc Pediatr Neurosurg 20:323–328
Carmel PW, Markesbery WR (1972) Early descriptions of the Arnold-Chiari malformation. The contribution of John Cleland. J Neurosurg 37:543–547
Pearce JM (2000) Arnold chiari, or “Cruveilhier cleland Chiari” malformation. J Neurol Neurosurg Psychiatry 68:13
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr 8:205–213
Smith BW, Strahle J, Bapuraj JR, Muraszko KM, Garton HJ, Maher CO (2013) Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. J Neurosurg 119:812–819
Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr 2:179–187
Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, Sorel M, Wu YW (2009) Chiari type I malformation in a pediatric population. Pediatr Neurol 40:449–454
Benglis D Jr, Covington D, Bhatia R, Bhatia S, Elhammady MS, Ragheb J, Morrison G, Sandberg DI (2011) Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery. J Neurosurg Pediatr 7:375–379
Pomeraniec IJ, Ksendzovsky A, Awad AJ, Fezeu F, Jane JA Jr (2016) Natural and surgical history of Chiari malformation Type I in the pediatric population. J Neurosurg Pediatr 17:343–352
Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Child Nerv Syst : ChNS : Off J Int Soc Pediatr Neurosurg 20:341–348
Steinbok P (2004) Clinical features of Chiari I malformations. Child Nerv System : ChNS : Off J Int Soc Pediatr Neurosurg 20:329–331
Nohria V, Oakes WJ (1990) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227
Pollack IF, Pang D, Kocoshis S, Putnam P (1992) Neurogenic dysphagia resulting from Chiari malformations. Neurosurgery 30:709–719
Pollack IF, Pang D, Albright AL, Krieger D (1992) Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77:881–888
Park TS, Hoffman HJ, Hendrick EB, Humphreys RP (1983) Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele. Neurosurgery 13:147–152
Caldarelli M, Di Rocco C (2004) Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies. Child Nerv Syst : ChNS : Off J Int Soc Pediatr Neurosurg 20:332–335
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Natural history of Chiari malformation Type I following decision for conservative treatment. J Neurosurg Pediatr 8:214–221
Leon TJ, Kuhn EN, Arynchyna AA, Smith BP, Tubbs RS, Johnston JM, Blount JP, Rozzelle CJ, Oakes WJ, Rocque BG (2019) Patients with “benign” Chiari I malformations require surgical decompression at a low rate. J Neurosurg Pediatr:1–9
Avellino AM, Kim DK, Weinberger E, Roberts TS (1996) Resolution of spinal syringes and Chiari I malformation in a child. Case illustration. J Neurosurg 84:708
Sun PP, Harrop J, Sutton LN, Younkin D (2001) Complete spontaneous resolution of childhood Chiari I malformation and associated syringomyelia. Pediatrics 107:182–184
Sun JC, Steinbok P, Cochrane DD (2000) Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia. Case report. J Neurosurg 92:207–210
Jatavallabhula NS, Armstrong J, Sgouros S, Whitehouse W (2006) Spontaneous resolution of isolated Chiari I malformation. Child Nerv Syst : ChNS : Off J Int Soc Pediatr Neurosurg 22:201–203
Tokunaga M, Minami S, Isobe K, Moriya H, Kitahara H, Nakata Y (2001) Natural history of scoliosis in children with syringomyelia. J Bone Joint Surg Br Vol 83:371–376
Massimi L, Caldarelli M, Frassanito P, Di Rocco C (2011) Natural history of Chiari type I malformation in children. Neurol Sci : Off J Italian Neurol Soc Italian Soc Clin Neurophysiol 32(Suppl 3):S275–S277
Sudo K, Doi S, Maruo Y, Tashiro K, Terae S, Miyasaka K, Isu T (1990) Syringomyelia with spontaneous resolution. J Neurol Neurosurg Psychiatry 53:437–438
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflicts of interest to report.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Chatrath, A., Marino, A., Taylor, D. et al. Chiari I malformation in children—the natural history. Childs Nerv Syst 35, 1793–1799 (2019). https://doi.org/10.1007/s00381-019-04310-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-019-04310-0