Abstract
Purpose
Multiple imaging parameters have been examined to estimate the presence of syrinx and the need for surgery in Chiari I patients (CM1); however, no consistent or definitive criteria have been proposed. The objective of this study was to review existing and identify novel radiological and clinical characteristics of CM1 patients that associate syrinx development and surgical intervention.
Methods
Patients with Chiari I malformation diagnosed on imaging between 0 and 18 years were retrospectively reviewed from January 1, 2007 to February 12, 2020. Participants were included if they had a baseline MRI of the head and spine prior to surgical intervention if required. Forty age-matched controls with cranial imaging were identified for comparison. Imaging parameters and clinical symptoms were recorded.
Results
A total of 122 CM1 patients were included in this study. Of the 122 patients, 28 (23%) had syrinx, and 27 (22%) had surgery. The following imaging parameters associated with syrinx and surgical intervention were identified: midbrain length (P < 0.001; P = 0.032), the obex position (P = 0.002; P < 0.001) and medullary kinking (P = 0.041; P < 0.001). Among the clinical features, the presence of overall pain (P = 0.017; P = 0.042), neck pain (P = 0.005; P = 0.027), and sensory dysfunction (P < 0.001) were found to be strongly associated with syrinx and surgery.
Conclusion
While further investigation is needed, these specific radiological and clinical parameters should be considered when evaluating CM1 patients and may be used to guide further management.
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References
Alexander H, Tsering D, Myseros JS, Magge SN, Oluigbo C, Sanchez CE, Keating RF (2019) Management of Chiari I malformations: a paradigm in evolution. Child Nerv Syst. https://doi.org/10.1007/s00381-019-04265-2
Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol 7:795–799
Arnautovic A, Splavski B, Boop FA, Arnautovic KI (2015) Pediatric and adult Chiari malformation type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. https://doi.org/10.3171/2014.10.PEDS14295
Chatrath A, Marino A, Taylor D, Elsarrag M, Soldozy S, Jane JA (2019) Chiari I malformation in children—the natural history. Child’s Nervous System. https://doi.org/10.1007/s00381-019-04310-0
McClugage SG, Oakes WJ (2019) The Chiari I malformation. J Neurosurg Pediatr. https://doi.org/10.3171/2019.5.PEDS18382
Pepper J, Elhabal A, Tsermoulas G, Flint G (2020) Symptom outcome after craniovertebral decompression for Chiari type 1 malformation without syringomyelia. Acta Neurochir. https://doi.org/10.1007/s00701-020-04631-z
Shoja MM, Tubbs RS, Oakes WJ (2013) Embryology and pathophysiology of the Chiari I and II malformations. In: The Chiari Malformations. Springer New York, New York, NY
Steinbok P (2004) Clinical features of Chiari I malformations. Child Nerv Syst. https://doi.org/10.1007/s00381-003-0879-x
Holly LT, Batzdorf U (2019) Chiari malformation and syringomyelia. J Neurosurg Spine. https://doi.org/10.3171/2019.7.SPINE181139
Attal N (2004) Effects of surgery on the sensory deficits of syringomyelia and predictors of outcome: a long term prospective study. J Neurol Neurosurg Psychiatry. https://doi.org/10.1136/jnnp.2003.026674
Alford EN, Atchley TJ, Leon TJ, Laskay NMB, Arynchyna AA, Smith BP, Aban I, Johnston JM, Blount JP, Rozzelle CJ, Oakes WJ, Rocque BG (2021) Imaging characteristics associated with surgery in Chiari malformation type I. J Neurosurg Pediatr. https://doi.org/10.3171/2020.9.PEDS20347
Atchley TJ, Alford EN, Rocque BG (2020) Systematic review and meta-analysis of imaging characteristics in Chiari I malformation: Does anything really matter? Child Nerv Syst. https://doi.org/10.1007/s00381-019-04398-4
Gad KA, Yousem DM (2017) Syringohydromyelia in patients with Chiari i malformation: a retrospective analysis. Am J Neuroradiol 38:1833–1838. https://doi.org/10.3174/ajnr.A5290
Haller G, Sadler B, Kuensting T, Lakshman N, Greenberg JK, Strahle JM, Park TS, Dobbs MB, Gurnett CA, Limbrick DD (2020) Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation. J Neurosurg Pediatr. https://doi.org/10.3171/2020.2.PEDS19486
Stovner LJ, Rinck P (1992) Syringomyelia in Chiari malformation. Neurosurgery. https://doi.org/10.1227/00006123-199211000-00013
R Core Team (2020) R: a language and environment for statistical computing. R Foundation for Statistical Computing, Vienna, Austria. https://www.R-project.org/
Holm S (1979) A simple sequentially rejective multiple test procedure. Scand J Stat 6:65–70
Al-Habib A, al Abdulsalam H, Ahmed J, Albadr F, Alhothali W, Alzahrani A, Abojamea A, Altowim A, Ullah A, Alkubeyyer M (2020) Association between craniovertebral junction abnormalities and syringomyelia in patients with chiari malformation type-1. Neurosciences 25:308–315. https://doi.org/10.17712/nsj.2020.4.20200008
Halvorson KG, Kellogg RT, Keachie KN, Grant GA, Muh CR, Waldau B (2016) Morphometric analysis of predictors of cervical syrinx formation in the setting of Chiari I malformation. Pediatr Neurosurg. https://doi.org/10.1159/000442991
Hiremath SB, Fitsiori A, Boto J, Torres C, Zakhari N, Dietemann JL, Meling TR, Vargas MI (2020) The perplexity surrounding chiari malformations - are we any wiser now? Am J Neuroradiol 41:1975–1981
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJL, Maher CO (2011) Chiari malformation type I and syrinx in children undergoing magnetic resonance imaging: Clinical article. J Neurosurg Pediatr 8:205–213. https://doi.org/10.3171/2011.5.PEDS1121
Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM (1999) Pediatric Chiari I malformations: do clinical and radiologic features correlate? Neurology. https://doi.org/10.1212/WNL.53.6.1271
Yan H, Han X, Jin M, Liu Z, Xie D, Sha S, Qiu Y, Zhu Z (2016) Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia. Eur Spine J 25:2202–2209. https://doi.org/10.1007/s00586-016-4410-y
Milhorat TH, Chou MW, Trinidad EM, Kula RW, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients
Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ (2004) A critical analysis of the Chiari 1.5 malformation. J Neurosurg Pediatr 101. https://doi.org/10.3171/ped.2004.101.2.0179
Tubbs RS, Wellons JC, Blount JP, Grabb PA, Oakes WJ (2003) Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg Spine. https://doi.org/10.3171/spi.2003.98.1.0043
Tubbs RS, Lyerly MJ, Loukas M, Shoja MM, Oakes WJ (2007) The pediatric Chiari I malformation: a review. Child Nerv Syst. https://doi.org/10.1007/s00381-007-0428-0
Sandoval-Garcia C, Iskandar BJ (2013) Research on the pathophysiology of Chiari I-related symptoms and syringomyelia, with emphasis on dynamic MRI techniques. In: The Chiari Malformations. Springer New York, New York, NY
Koyanagi I, Houkin K (2010) Pathogenesis of syringomyelia associated with Chiari type 1 malformation: Review of evidences and proposal of a new hypothesis. Neurosurg Rev. https://doi.org/10.1007/s10143-010-0266-5
Bollo RJ, Riva-Cambrin J, Brockmeyer MM, Brockmeyer DL (2012) Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr. https://doi.org/10.3171/2012.3.PEDS11340
Strahle J, Smith BW, Martinez M, Bapuraj JR, Muraszko KM, Garton HJL, Maher CO (2015) The association between Chiari malformation type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr 15:607–611. https://doi.org/10.3171/2014.11.PEDS14135
Moore HE, Moore KR (2014) Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. https://doi.org/10.1007/s00247-014-3021-1
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. https://doi.org/10.1016/j.pediatrneurol.2009.01.003
Greenberg JK, Yarbrough CK, Radmanesh A, Godzik J, Yu M, Jeffe DB, Smyth MD, Park TS, Piccirillo JF, Limbrick DD (2015) The Chiari severity index. Neurosurgery. https://doi.org/10.1227/NEU.0000000000000608
Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Child Nerv Syst. https://doi.org/10.1007/s00381-003-0882-2
Papaker MG, Abdallah A, Cesme DH, Gönen G, Asiltürk M, Avyasov R, Sofuoğlu ÖE, Abdallah BG, Emel E (2020) Clinical and radiological evaluation of treated Chiari I adult patients: Retrospective study from two neurosurgical centers. Neurosurg Rev. https://doi.org/10.1007/s10143-020-01414-z
Holly LT, Batzdorf U (2002) Slitlike syrinx cavities: a persistent central canal. J Neurosurg Spine. https://doi.org/10.3171/spi.2002.97.2.0161
Takamura Y, Kawasaki T, Takahashi A, Nunomura K, Tiba K, Hasunuma M, Itou T (2001) A craniocervical injury—induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. J Neurosurg Spine. https://doi.org/10.3171/spi.2001.95.1.0122
Milhorat TH, Kotzen RM, Anzil AP (1994) Stenosis of central canal of spinal cord in man: Incidence and pathological findings in 232 autopsy cases. J Neurosurg. https://doi.org/10.3171/jns.1994.80.4.0716
Cai C, Oakes WJ (1997) Hindbrain herniation syndromes: the Chiari malformations (I and II)
Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-Gadol AA (2015) The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28:184–194
Hunter JV, Youl BD, Moseley IF (1992) Neuro-radiology MRI demonstration of midbrain deformity in association with Chiari malformation
el Gammal T, Mark E, Brooks B (1988) MR imaging of Chiari II malformation. Am J Roentgenol 150:163–170. https://doi.org/10.2214/ajr.150.1.163
Olszewski AM, Proctor MR (2018) Headache, Chiari I malformation and foramen magnum decompression. Curr Opin Pediatr. https://doi.org/10.1097/MOP.0000000000000679
Saletti V, Esposito S, Frittoli M, Valentini LG, Chiapparini L, Bulgheroni S, Riva D (2011) Neurological pictures in paediatric Chiari I malformation. Neurol Sci. https://doi.org/10.1007/s10072-011-0744-8
Cahan LD, Bentson JR (1982) Considerations in the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg. https://doi.org/10.3171/jns.1982.57.1.0024
Gamache FW Jr, Ducker TB (1990) Syringomyelia: a neurological and surgical spectrum. J Spinal Disord 1990 Dec;3(4):293-8. PMID: 2134442
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Dien Esquivel, M.F., Gupta, N., Wilson, N. et al. Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery. Childs Nerv Syst 38, 2119–2128 (2022). https://doi.org/10.1007/s00381-022-05644-y
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DOI: https://doi.org/10.1007/s00381-022-05644-y