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Sudden onset of Chiari malformation type 1 in a young child after trauma

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Abstract

Introduction

Chiari 1 malformation is a rare craniovertebral junction malformation accounting up to 1 case in every 1000 newborns per year. It is characterized by herniation of cerebellar tonsils below the foramen magnum sometimes with syringomyelia. Usually, patients have a long history of slowly progressive neurological symptoms. Uncommonly, Chiari 1 malformation could present with a sudden onset, also after trauma. Few cases are reported about young children.

Methods

The authors report a case of a 6-month child with symptoms at onset after a mild trauma. The pertinent literature is reviewed.

Conclusions

Symptoms of Chiari 1 malformation are usually slowly progressive. Few cases have been reported of the sudden onset of symptoms, some of these after trauma. In young children, the clinical setting could be insidious and potentially lethal. A sudden onset of Chiari 1 malformation must be considered as a consequence of trauma, usually after performing a brain MRI. Management of these cases is still controversial, and surgery may be indicated in managing symptoms; however, it seems to not affect clinical outcome.

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References

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

    Article  PubMed  Google Scholar 

  2. Alden TD, Ojemann JG, Park TS (2001) Surgical treatment of Chiari I malformation: indications and approaches. Neurosurg Focus 11, E2

    Article  CAS  PubMed  Google Scholar 

  3. Baykal S, Çakir E, Karakuş M, Kuzeyli K, Sari A (1999) Presentation of Chiari I malformation after craniocervical trauma: a case report. Turk Neurosurg 9:129–132

    Google Scholar 

  4. Bondurant CP, Oró JJ (1993) Spinal cord injury without radiographic abnormality and Chiari malformation. J Neurosurg 79:833–838

    Article  CAS  PubMed  Google Scholar 

  5. Bonfiled CM, Tyler-Kabara E (2013) Unusual presentations of the Chiari I malformation. In: Tubbs RS, Oakes WJ (eds) The Chiari malformations. Springer Science+Business Media, New York, pp 261–264

    Chapter  Google Scholar 

  6. Bunc G, Vorsic M (2001) Presentation of a previously asymptomatic Chiari I malformation by a flexion injury to the neck. J Neurotrauma 18:645–648. doi:10.1089/089771501750291882

    Article  CAS  PubMed  Google Scholar 

  7. 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 28:1218–1220

    Article  CAS  PubMed  Google Scholar 

  8. Chiari H (1891) Ueber veränderungen des kleinhirns infolge von hydrocephalie des grosshirns. Dtsch Med Wochenschr 17:1172–1175

    Article  Google Scholar 

  9. Couldwell WT, Zhang W, Allen R, Arce D, Stillerman CB (1998) Cerebellar contusion associated with type I Chiari malformation following supratentorial head trauma: case report. Neurol Res 20:93–96

    CAS  PubMed  Google Scholar 

  10. Erlich V, Snow R, Heier L (1989) Confirmation by magnetic resonance imaging of Bell's cruciate paralysis in a young child with Chiari type I malformation and minor head trauma. Neurosurgery 25:102–105

    Article  CAS  PubMed  Google Scholar 

  11. Genitori L, Peretta P, Nurisso C, Macinante L, Mussa F (2000) Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases. Childs Nerv Syst 16:707–718

    Article  CAS  PubMed  Google Scholar 

  12. James HE (2007) Chiari malformation type I. J Neurosurg 107:184. doi:10.3171/PED-07/08/184, author reply 184

    PubMed  Google Scholar 

  13. Koehler PJ (1994) Chiari's description of cerebellar ectopy (1891). With a summary of Cleland's and Arnold's contributions and some early observations on neural-tube defects. J Neurosurg 75:823–826. doi:10.3171/jns.1991.75.5.0823

    Article  Google Scholar 

  14. Kurup H, Lawrence T, Hargreaves D (2005) Transient quadriparesis following neck injury: presentation of a Chiari 1 malformation. Eur J Orthop Surg Traumatol 15:319–321

    Article  Google Scholar 

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

    Article  PubMed  Google Scholar 

  16. Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold–Chiari malformation. J Neurol Sci 50:29–55

    Article  CAS  PubMed  Google Scholar 

  17. 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 19:73–74

    Article  CAS  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, Caldarelli M, Di Rocco C (2012) Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications. Neurosurg Rev 35:321–329. doi:10.1007/s10143-012-0391-4, discussion 329

    Article  PubMed  Google Scholar 

  20. 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 8:438–442. doi:10.3171/2011.8.PEDS11160

    Article  PubMed  Google Scholar 

  21. Massimi L, Peppucci E, Peraio S, Di Rocco C (2011) History of Chiari type I malformation. Neurol Sci 32(Suppl 3):S263–S265. doi:10.1007/s10072-011-0700-7

    Article  PubMed  Google Scholar 

  22. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920–926. doi:10.3171/jns.2000.92.6.0920

    Article  CAS  PubMed  Google Scholar 

  23. Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA (2007) Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine 7:601–609. doi:10.3171/SPI-07/12/601

    Article  PubMed  Google Scholar 

  24. 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 44:1005–1017

    Article  CAS  PubMed  Google Scholar 

  25. Olivero WC, Dinh DH (1992) Chiari I malformation with traumatic syringomyelia and spontaneous resolution: case report and literature review. Neurosurgery 30:758–760

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  27. Ramon C, Gonzales-Mandly A, Pascual J (2011) What differences exist in the appropriate treatment of congenital versus acquired adult Chiari type I malformation? Curr Pain Headache Rep 15:157–163. doi:10.1007/s11916-011-0182-6

    Article  PubMed  Google Scholar 

  28. Riviello JJ, Marks HG, Faeber EN, Steg NL (1990) Delayed cervical central cord syndrome after trivial trauma. Pediatr Emerg Care 6:113–117

    Article  PubMed  Google Scholar 

  29. Rozzelle CJ (2013) Clinical presentation of pediatric Chiari I malformations. In: Tubbs RS, Oakes WJ (eds) The Chiari malformations. Springer Science+Business Media, New York, pp 247–251

    Chapter  Google Scholar 

  30. Sgouros S, Kountouri M, Natarajan K (2006) Posterior fossa volume in children with Chiari malformation type I. J Neurosurg 105:101–106. doi:10.3171/ped.2006.105.2.101

    PubMed  Google Scholar 

  31. Tomaszek DE, Tyson GW, Bouldin T, Hansen AR (1984) Sudden death in a child with an occult hindbrain malformation. Ann Emerg Med 13:136–138

    Article  CAS  PubMed  Google Scholar 

  32. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, Rozzelle CJ, Blount JP, Oakes WJ (2011) Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr 7:248–256. doi:10.3171/2010.12.PEDS10379

    Article  PubMed  Google Scholar 

  33. Vannemreddy P, Nourbakhsh A, Willis B, Guthikonda B (2010) Congenital Chiari malformations. Neurol India 58:6–14. doi:10.4103/0028-3886.60387

    Article  PubMed  Google Scholar 

  34. Vlcek BW, Ito B (1987) Acute paraparesis secondary to Arnold‐Chiari type I malformation and neck hyperflexion. Ann Neurol 21:100–101

    Article  CAS  PubMed  Google Scholar 

  35. Wan MJ, Nomura H, Tator CH (2008) Conversion to symptomatic Chiari I malformation after minor head or neck trauma. Neurosurgery 63:748–753. doi:10.1227/01.NEU.0000325498.04975.C0, discussion 753

    Article  PubMed  Google Scholar 

  36. Wellons JC 3rd, Tubbs RS, Bui CJ, Grabb PA, Oakes WJ (2007) Urgent surgical intervention in pediatric patients with Chiari malformation type I. Report of two cases. J Neurosurg 107:49–52. doi:10.3171/PED-07/07/049

    Article  PubMed  Google Scholar 

  37. Wolf DA, Veasey SP 3rd, Wilson SK, Adame J, Korndorffer WE (1998) Death following minor head trauma in two adult individuals with the Chiari I deformity. J Forensic Sci 43:1241–1243

    Article  CAS  PubMed  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 7:244–247. doi:10.3171/2010.11.PEDS1097

    Article  PubMed  Google Scholar 

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Correspondence to Alfio Spina.

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Spina, A., Boari, N., Gagliardi, F. et al. Sudden onset of Chiari malformation type 1 in a young child after trauma. Childs Nerv Syst 31, 1589–1594 (2015). https://doi.org/10.1007/s00381-015-2736-0

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  • DOI: https://doi.org/10.1007/s00381-015-2736-0

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