Skip to main content
SpringerLink
Log in

Unusual presentation of Chiari I in toddlers: case reports and review of the literature

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Toddlers with Chiari malformations can present with common symptoms, such as cough or drooling, which are associated with respiratory illnesses, delaying a correct diagnosis. Presentation does vary with age, and therefore can be different in older children when compared to infants. We present two cases of unusual presentations in toddlers and review the literature with an emphasis on acute presentation of Chiari in different age groups in children.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. 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(6):449–454. doi:10.1016/j.pediatrneurol.2009.01.003

    Article  PubMed  Google Scholar 

  2. Albert GW, Menezes AH, Hansen DR, Greenlee JD, Weinstein SL (2010) Chiari malformation Type I in children younger than age 6years: presentation and surgical outcome. J Neurosurg Pediatr 5(6):554–561. doi:10.3171/2010.3.PEDS09489

    Article  PubMed  Google Scholar 

  3. Alvarez D, Requena I, Arias M, Valdes L, Pereiro I, De la Torre R (1995) Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia. Eur Respir J 8(4):661–663

    PubMed  CAS  Google Scholar 

  4. 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(4):375–379. doi:10.3171/2011.1.PEDS10341

    Article  PubMed  Google Scholar 

  5. Callaway GH, O'Brien SJ, Tehrany AM (1996) Chiari I malformation and spinal cord injury: cause for concern in contact athletes? Med Sci Sports Exerc 28(10):1218–1220

    Article  PubMed  CAS  Google Scholar 

  6. Carlson MD, Muraszko KM (2003) Chiari I malformation with syrinx. Pediatr Neurol 29(2):167–169

    Article  PubMed  Google Scholar 

  7. Dauvilliers Y, Stal V, Abril B, Coubes P, Bobin S, Touchon J, Escourrou P, Parker F, Bourgin P (2007) Chiari malformation and sleep-related breathing disorders. J Neurol Neurosurg Psychiatry 78(12):1344–1348. doi:10.1136/jnnp.2006.108779

    Article  PubMed  CAS  Google Scholar 

  8. Dure LS, Percy AK, Cheek WR, Laurent JP (1989) Chiari type I malformation in children. J Pediatr 115(4):573–576

    Article  PubMed  CAS  Google Scholar 

  9. Dyste GN, Menezes AH, VanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71(2):159–168. doi:10.3171/jns.1989.71.2.0159

    Article  PubMed  CAS  Google Scholar 

  10. Gosalakkal JA (2008) Sleep-disordered breathing in Chiari malformation type 1. Pediatr Neurol 39(3):207–208. doi:10.1016/j.pediatrneurol.2008.05.017

    Article  PubMed  Google Scholar 

  11. Greenlee JD, Donovan KA, Hasan DM, Menezes AH (2002) Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6years. Pediatrics 110(6):1212–1219

    Article  PubMed  Google Scholar 

  12. Itoh N, Mimaki T, Tagawa T, Sugita T, Yabuuchi H, Ushio Y (1988) Chiari I malformation with quadriplegia and respiratory disturbance in an infant. Brain Dev 10(3):189–190

    Article  PubMed  CAS  Google Scholar 

  13. Laufer I, Engel M, Feldstein N, Souweidane MM (2008) Chiari malformation presenting as a focal motor deficit. Report of two cases. J Neurosurg Pediatr 1(5):392–395. doi:10.3171/PED/2008/1/5/392

    Article  PubMed  Google Scholar 

  14. Listernick R, Tomita T (1991) Persistent crying in infancy as a presentation of Chiari type I malformation. J Pediatr 118(4 Pt 1):567–569

    PubMed  CAS  Google Scholar 

  15. Mampalam TJ, Andrews BT, Gelb D, Ferriero D, Pitts LH (1988) Presentation of type I Chiari malformation after head trauma. Neurosurgery 23(6):760–762

    Article  PubMed  CAS  Google Scholar 

  16. Martinot A, Hue V, Leclerc F, Vallee L, Closset M, Pruvo JP (1993) Sudden death revealing Chiari type 1 malformation in two children. Intensive Care Med 19(2):73–74

    Article  PubMed  CAS  Google Scholar 

  17. Massey SL, Buland J, Hauber S, Piatt J Jr, Goraya J, Faerber E, Valencia I (2011) Acute VI nerve palsy in a 4year-old girl with Chiari I malformation and pontomedullary extension of syringomyelia: case report and review of the literature. Eur J Paediatr Neurol 15(4):303–309. doi:10.1016/j.ejpn.2011.04.001

    Article  PubMed  Google Scholar 

  18. Massimi L, Caldarelli M, Frassanito P, Di Rocco C (2011) Natural history of Chiari type I malformation in children. Neurol Sci 32(Suppl 3):S275–S277. doi:10.1007/s10072-011-0684-3

    Article  PubMed  Google Scholar 

  19. Massimi L, Della Pepa GM, Tamburrini G, Di Rocco C (2011) Sudden onset of Chiari malformation Type I in previously asymptomatic patients. J Neurosurg Pediatr 8(5):438–442. doi:10.3171/2011.8.PEDS11160

    Article  PubMed  Google Scholar 

  20. Massimi L, Novegno F, di Rocco C (2011) Chiari type I malformation in children. Adv Tech Stand Neurosurg 37:143–211. doi:10.1007/978-3-7091-0673-0_6

    Article  PubMed  CAS  Google Scholar 

  21. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44(5):1005–1017

    Article  PubMed  CAS  Google Scholar 

  22. Mueller DM, Oro JJ (2004) Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. J Am Acad Nurse Pract 16(3):134–138

    Article  PubMed  Google Scholar 

  23. Muhonen MG, Menezes AH, Sawin PD, Weinstein SL (1992) Scoliosis in pediatric Chiari malformations without myelodysplasia. J Neurosurg 77(1):69–77. doi:10.3171/jns.1992.77.1.0069

    Article  PubMed  CAS  Google Scholar 

  24. Nagib MG (1994) An approach to symptomatic children (ages 4-14years) with Chiari type I malformation. Pediatr Neurosurg 21(1):31–35

    Article  PubMed  CAS  Google Scholar 

  25. Nathadwarawala KM, Richards CA, Lawrie B, Thomas GO, Wiles CM (1992) Recurrent aspiration due to Arnold-Chiari type I malformation. BMJ 304(6826):565–566

    Article  PubMed  CAS  Google Scholar 

  26. Nohria V, Oakes WJ (1990) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16(4–5):222–227

    Article  PubMed  Google Scholar 

  27. 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(3):179–187. doi:10.3171/PED/2008/2/9/179

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  29. Quebada PB, Duhaime AC (2005) Chiari malformation Type I and a dolichoodontoid process responsible for sudden cardiorespiratory arrest. Case report. J Neurosurg 103(6 Suppl):567–570. doi:10.3171/ped.2005.103.6.0567

    PubMed  Google Scholar 

  30. Rekate HL, Nadkarni TD, Teaford PA, Wallace D (1999) Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism. Neurosurgery 45(2):386–391

    Article  PubMed  CAS  Google Scholar 

  31. Spence J, Pasterkamp H, McDonald PJ (2010) Isolated central sleep apnea in type I Chiari malformation: improvement after surgery. Pediatr Pulmonol 45(11):1141–1144. doi:10.1002/ppul.21294

    Article  PubMed  Google Scholar 

  32. Steinbok P (2004) Clinical features of Chiari I malformations. Childs Nerv Syst 20(5):329–331. doi:10.1007/s00381-003-0879-x

    Article  PubMed  Google Scholar 

  33. Taylor FR, Larkins MV (2002) Headache and Chiari I malformation: clinical presentation, diagnosis, and controversies in management. Curr Pain Headache Rep 6(4):331–337

    Article  PubMed  Google Scholar 

  34. Tubbs RS, Lyerly MJ, Loukas M, Shoja MM, Oakes WJ (2007) The pediatric Chiari I malformation: a review. Childs Nerv Syst 23(11):1239–1250. doi:10.1007/s00381-007-0428-0

    Article  PubMed  Google Scholar 

  35. Tubbs RS, McGirt MJ, Oakes WJ (2003) Surgical experience in 130 pediatric patients with Chiari I malformations. J Neurosurg 99(2):291–296. doi:10.3171/jns.2003.99.2.0291

    Article  PubMed  Google Scholar 

  36. Van den Broek MJ, Arbues AS, Chalard F, Pinto Da Costa NM, Trang H, Dorfmuller G, Evrard PP, Husson I, Servais LJ (2009) Chiari type I malformation causing central apnoeas in a 4-month-old boy. Eur J Paediatr Neurol 13(5):463–465. doi:10.1016/j.ejpn.2008.07.012

    Article  PubMed  Google Scholar 

  37. Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM (1999) Pediatric Chiari I malformations: do clinical and radiologic features correlate? Neurology 53(6):1271–1276

    Article  PubMed  CAS  Google Scholar 

  38. Yarbrough CK, Powers AK, Park TS, Leonard JR, Limbrick DD, Smyth MD (2011) Patients with Chiari malformation Type I presenting with acute neurological deficits: case series. J Neurosurg Pediatr 7(3):244–247. doi:10.3171/2010.11.PEDS1097

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Schulich School of Medicine and Dentistry, University of Western Ontario, Victoria Hospital, B1-181, 800 Commissionners Rd E, London, ON, N6A 5W9, Canada

    Caitlin L. Carew, Asuri Prasad, Keng Yeow Tay & Sandrine de Ribaupierre

  2. Department of Clinical Neurological Sciences, University of Western Ontario, Victoria Hospital, B1-181, 800 Commissionners Rd E, London, ON, N6A 5W9, Canada

    Sandrine de Ribaupierre

  3. Department of Pediatrics, University of Western Ontario, London, ON, N6A 5W9, Canada

    Asuri Prasad

  4. Department of Radiology, University of Western Ontario, London, ON, N6A 5W9, Canada

    Keng Yeow Tay

Authors
  1. Caitlin L. Carew
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Asuri Prasad
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Keng Yeow Tay
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sandrine de Ribaupierre
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sandrine de Ribaupierre.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Carew, C.L., Prasad, A., Tay, K.Y. et al. Unusual presentation of Chiari I in toddlers: case reports and review of the literature. Childs Nerv Syst 28, 1965–1970 (2012). https://doi.org/10.1007/s00381-012-1826-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-012-1826-5

Keywords

Search

Navigation