Abstract
Chiari malformation type 1 (CM I) is a common neurological disease in both children and adults. While CM I patients commonly present with slowly progressive pain, signs of brainstem compression, or spinal cord compression from a syrinx, a small minority of patients present with rapid onset of neurological deficits. Compared to the general CM I population, these patients are more likely to have motor deficits, respiratory dysfunction, cranial nerve abnormalities, or even sudden death. While traumatic injury is a potential cause of such abrupt presentations, many patients develop acute neurological deficits without preceding trauma. Compared to the typically elective management of CM I, patients with acute deficits should generally undergo surgical treatment in an expedited or urgent manner, with particular attention also paid to managing any respiratory concerns prior to and following surgical intervention. With appropriate management, these patients often have substantial if not complete resolution of their presenting complaints. Therefore, while CM I is typically considered a chronic condition, physicians should remain aware of the important minority of patients who present acutely, ensuring they receive timely diagnosis and management.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 2011;8(2):205–13.
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.
Greenberg JK, Yarbrough CK, Radmanesh A, Godzik J, Yu M, Jeffe DB, et al. The Chiari severity index: a preoperative grading system for Chiari malformation type 1. Neurosurgery. 2015;76(3):279–85; discussion 85.
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.
Massimi L, Della Pepa GM, Caldarelli M, Di Rocco C. Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications. Neurosurg Rev. 2012;35(3):321–9; discussion 9.
Tomaszek DE, Tyson GW, Bouldin T, Hansen AR. Sudden death in a child with an occult hindbrain malformation. Ann Emerg Med. 1984;13(2):136–8.
Bresnan MJ, Shoukimas GM, Hedley-Whyte ET. Case 3-1987. N Engl J Med. 1987;316(3):150–7.
Dong ML. Arnold-Chiari malformation type I appearing after tonsillectomy. Anesthesiology. 1987;67(1):120–2.
Vlcek BW, Ito B. Acute paraparesis secondary to Arnold-Chiari type I malformation and neck hyperflexion. Ann Neurol. 1987;21(1):100–1.
Bullock R, Todd NV, Easton J, Hadley D. Isolated central respiratory failure due to syringomyelia and Arnold-Chiari malformation. Br Med J. 1988;297(6661):1448–9.
Mampalam TJ, Andrews BT, Gelb D, Ferriero D, Pitts LH. Presentation of type I Chiari malformation after head trauma. Neurosurgery. 1988;23(6):760–2.
Riviello JJ Jr, Marks HG, Faerber EN, Steg NL. Delayed cervical central cord syndrome after trivial trauma. Pediatr Emerg Care. 1990;6(2):113–7.
Martinot A, Hue V, Leclerc F, Vallee L, Closset M, Pruvo JP. Sudden death revealing Chiari type 1 malformation in two children. Intensive Care Med. 1993;19(2):73–4.
Bondurant CP, Oro JJ. Spinal cord injury without radiographic abnormality and Chiari malformation. J Neurosurg. 1993;79(6):833–8.
Zager EL, Ojemann RG, Poletti CE. Acute presentations of syringomyelia. Report of three cases. J Neurosurg. 1990;72(1):133–8.
Kanev PM, Getch CC, Jallo J, Faerber EN. Cerebral syrinx with Chiari I malformation. Pediatr Neurosurg. 1994;20(3):214–6.
Alvarez D, Requena I, Arias M, Valdes L, Pereiro I, De la Torre R. Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia. Eur Respir J. 1995;8(4):661–3.
James DS. Significance of chronic tonsillar herniation in sudden death. Forensic Sci Int. 1995;75(2–3):217–23.
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.
Jackson RM, Penrose-Stevens A. Meningococcal meningitis with Arnold-Chiari malformation. J Infect. 1997;35(1):90–2.
Wolf DA, Veasey SP 3rd, 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.
Weeks CL, Hamed LM. Treatment of acute comitant esotropia in Chiari I malformation. Ophthalmology. 1999;106(12):2368–71.
Ziegler DK, Mallonee W. Chiari-1 malformation, migraine, and sudden death. Headache. 1999;39(1):38–41.
Bunc G, Vorsic M. Presentation of a previously asymptomatic Chiari I malformation by a flexion injury to the neck. J Neurotrauma. 2001;18(6):645–8.
Gentry JB, Gonzalez JM, Blacklock JB. Respiratory failure caused by Chiari I malformation with associated syringomyelia. Clin Neurol Neurosurg. 2001;103(1):43–5.
Defoort-Dhellemmes S, Denion E, Arndt CF, Bouvet-Drumare I, Hache JC, Dhellemmes P. Resolution of acute acquired comitant esotropia after suboccipital decompression for Chiari I malformation. Am J Ophthalmol. 2002;133(5):723–5.
Yoshikawa H. Sudden respiratory arrest and Arnold-Chiari malformation. Eur J Paediatr Neurol. 2003;7(4):191.
Kurup H, Lawrence T, Hargreaves D. Transient Quadriparesis following neck injury: presentation of a Chiari 1 malformation. Eur J Orthop Surg Traumatol. 2005;15(4):319–21.
Quebada PB, Duhaime AC. Chiari malformation Type I and a dolichoodontoid process responsible for sudden cardiorespiratory arrest. Case report. J Neurosurg. 2005;103(6 Suppl):567–70.
Tsara V, Serasli E, Kimiskidis V, Papagianopoulos S, Katsaridis V, Fylaktakis M, et al. Acute respiratory failure and sleep-disordered breathing in Arnold-Chiari malformation. Clin Neurol Neurosurg. 2005;107(6):521–4.
Bhangoo R, Sgouros S, Walsh AR, Clarke JR. Hindbrain-hernia-related syringomyelia without syringobulbia, complicated by permanent nocturnal central hypoventilation requiring non-invasive ventilation. Childs Nerv Syst. 2006;22(2):113–6.
Pilon A, Rhee P, Newman T, Messner L. Bilateral abducens palsies and facial weakness as initial manifestations of a Chiari 1 malformation. Optom Vis Sci. 2007;84(10):936–40.
Wellons JC 3rd, 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.
Kandasamy J, Kneen R, Gladstone M, Newman W, Mohamed T, Mallucci C. Chiari I malformation without hydrocephalus: acute intracranial hypertension managed with endoscopic third ventriculostomy (ETV). Childs Nerv Syst. 2008;24(12):1493–7.
Stephany JD, Garavaglia JC, Pearl GS. Sudden death in a 27-year-old man with Chiari I malformation. Am J Forensic Med Pathol. 2008;29(3):249–50.
Elliott R, Kalhorn S, Pacione D, Weiner H, Wisoff J, Harter D. Shunt malfunction causing acute neurological deterioration in 2 patients with previously asymptomatic Chiari malformation Type I. report of two cases. J Neurosurg Pediatr. 2009;4(2):170–5.
McMillan HJ, Sell E, Nzau M, Ventureyra EC. Chiari 1 malformation and holocord syringomyelia presenting as abrupt onset foot drop. Childs Nerv Syst. 2011;27(1):183–6.
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.
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.
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.
Oishi M, Hayashi Y, Kita D, Fukui I, Shinohara M, Heiss JD, et al. Rapidly progressing monoparesis caused by Chiari malformation type I without syringomyelia. Surg Neurol Int. 2013;4:79.
Schneider B, Birthi P, Salles S. Arnold-Chiari 1 malformation type 1 with syringohydromyelia presenting as acute tetraparesis: a case report. J Spinal Cord Med. 2013;36(2):161–5.
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.
Roohi F, Gropen T, Kula RW. Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics. Surg Neurol Int. 2014;5:17.
Wang H, Wang B, Normoyle K, Farahvar A, Olivero W. Chiari I malformation with acute brain stem compression syndromes requiring emergency neurosurgical intervention: report of two cases. J Neurol Disord. 2014;2(5):177.
Spina A, Boari N, Gagliardi F, Donofrio CA, Mortini P. Sudden onset of Chiari malformation type 1 in a young child after trauma. Childs Nerv Syst. 2015;31(9):1589–94.
Ulutabanca H, Kucuk A, Tumturk A, Bicer E, Oral S, Koc R. Chiari malformation Type 1 associated with syringomyelia which clinical symptoms occuring during tooth extraction. Erciyes Tıp Dergisi/Erciyes Med J. 2015;37(10).
Miranda SP, Kimmell KT, Silberstein HJ. Acute presentation of Chiari I malformation with hemiparesis in a pediatric patient. World Neurosurg. 2016;85:366.e1–4.
Woodward JA, Adler DE. Chiari I malformation with acute neurological deficit after craniocervical trauma: case report, imaging, and anatomic considerations. Surg Neurol Int. 2018;9:88.
Greenberg JK, Ladner TR, Olsen MA, Shannon CN, Liu J, Yarbrough CK, et al. Complications and resource use associated with surgery for Chiari malformation Type 1 in adults: a population perspective. Neurosurgery. 2015;77:261.
Greenberg JK, Olsen MA, Yarbrough CK, Ladner TR, Shannon CN, Piccirillo JF, et al. Chiari malformation Type I surgery in pediatric patients. Part 2: complications and the influence of comorbid disease in California, Florida, and New York. J Neurosurg Pediatr. 2016;17(5):525–32.
Kahn EN, Muraszko KM, Maher CO. Prevalence of Chiari I malformation and syringomyelia. Neurosurg Clin N Am. 2015;26(4):501–7.
Rozzelle CJ. Clinical presentation of pediatric Chiari I malformations. In: Tubbs RS, Oakes WJ, editors. The Chiari malformations. New York: Springer New York; 2013. p. 247–51.
Batzdorf U. Clinical presentation of the adult Chiari I malformation. In: Tubbs RS, Oakes WJ, editors. The Chiari malformations. New York: Springer New York; 2013. p. 253–9.
Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, 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.
OpenStax. Anatomy & Physiology: OpenStax CNX; Jul 30, 2014.
Alegre S, Garcia-Rubira JC, Patrignani G. Cardiac arrest in a 31-year-old man because of the Arnold-Chiari malformation. Int J Cardiol. 1994;46(3):286–8.
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.
Menezes AH. Chiari I malformations and hydromyelia--complications. Pediatr Neurosurg. 1991;17(3):146–54.
Milhorat TH, Bolognese PA, Black KS, Woldenberg RF. Acute syringomyelia: case report. Neurosurgery. 2003;53(5):1220–1; discussion 1-2.
Di Rocco C, Frassanito P, Massimi L, Peraio S. Hydrocephalus and Chiari type I malformation. Childs Nerv Syst. 2011;27(10):1653–64.
Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005;21(10):889–901.
Haroun RI, Guarnieri M, Meadow JJ, Kraut M, Carson BS. Current opinions for the treatment of syringomyelia and chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons. Pediatr Neurosurg. 2000;33(6):311–7.
Kirschen MP, Illes J. Ethical implications of an incidentally discovered asymptomatic Chiari malformation in a competitive athlete. Continuum (Minneapolis, Minn). 2014;20(6 Sports Neurology):1683–7.
Strahle J, Geh N, Selzer BJ, Bower R, Himedan M, Strahle M, et al. Sports participation with Chiari I malformation. J Neurosurg Pediatr. 2016;17(4):403–9.
Meehan WP 3rd, Jordaan M, Prabhu SP, Carew L, Mannix RC, Proctor MR. Risk of athletes with Chiari malformations suffering catastrophic injuries during sports participation is low. Clin J Sport Med. 2015;25(2):133–7.
Oaks WJ. Treatment of the pediatric Chiari I malformation. In: Tubbs RS, Oakes WJ, editors. The Chiari malformations. New York: Springer New York; 2013. p. 307–13.
Lee A, Yarbrough CK, Greenberg JK, Barber J, Limbrick DD, Smyth MD. Comparison of posterior fossa decompression with or without duraplasty in children with Type I Chiari malformation. Childs Nerv Syst. 2014;30(8):1419–24.
Anonymous. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 3-1987. A 17-year-old girl with numbness of the right leg and the recent onset of vertigo and right-sided weakness. N Engl J Med. 1987;316(3):150–7.
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.
Ranalli NJ, Limbrick DD, Park TS. Outcomes for the surgical management of Chiari I and Chiari II malformations. In: Tubbs RS, Oakes WJ, editors. The Chiari malformations. New York: Springer New York; 2013. p. 333–44.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Greenberg, J.K., Smyth, M.D. (2020). Acute and Sudden Presentations of the Chiari Malformations. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_33
Download citation
DOI: https://doi.org/10.1007/978-3-030-44862-2_33
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-44861-5
Online ISBN: 978-3-030-44862-2
eBook Packages: MedicineMedicine (R0)