Determining if a Relationship Exists Between Tonsillar Ectopia and Symptom Presentation in Chiari Malformation Patients
Author: Julia R. Saling, B.S.1, Paige Marty, B.S.2, Rebecca Fischbein, Ph.D3, Michelle Chyatte, Dr.PH., MPH4
1 Student Research Fellow, Northeast Ohio Medical University
2 Student Research Fellow, Northeast Ohio Medical University
3 Research Coordinator and Assistant Professor of Family and Community Medicine, Northeast Ohio Medical University
4 Assistant Professor of Family and Community Medicine, Northeast Ohio Medical University
[button link=”http://msrj.chm.msu.edu/wp-content/uploads/2016/08/TonsillarEctopiaepub.pdf” type=”big” color=”green” newwindow=”yes”] Full Text Article PDF[/button]
Corresponding Author: Julia R. Saling, email@example.com
Key Words: Chiari Malformation Type I, Tonsillar Ectopia, Symptom Presentation
Chiari Malformation Type I (CM I) is characterized by cerebellar tonsil ectopia and has varying symptomatology . Previous research has shown a relationship between tonsillar dominance and related conditions but few examined association with symptomatology. This study attempts to elucidate a relationship between cerebellar tonsil dominance, age, and symptomatology.
Data from CM I patients were extracted from the Conquer Chiari Patient Registry. Tonsillar dominance was determined using a ratio of right-to-left herniation length. Pearson’s correlation and one-tailed Student’s T-test were used for analysis.
Length of tonsillar descent appears to be negatively correlated to age of onset (r = -0.266; p < 0.001; n = 113) and diagnosis (r = -0.323; p < 0.001; n = 113). No correlation was found between tonsillar dominance and symptom location, nor between tonsillar dominance and symptom severity bilaterally (p > 0.05). Symptom location and severity ratios appear to be correlated (r = 0.666; p < 0.001). Tonsillar descent length appears to be strongly correlated bilaterally (r = 0.972; p < 0.001; n = 50).
Inconsistency between tonsillar dominance as related to symptomatology suggests a multifactorial contribution to clinical presentation. The inverse relationship between tonsillar herniation length and age of symptom onset and diagnosis suggests herniation length may be an important predictor for clinical outcomes. Further research is needed to elucidate additional contributing factors and tonsillar dominance and symptomatology association.
Published on date: August, 2016
Citation: Saling et al. Determining if a Relationship Exists Between Tonsillar Ectopia and Symptom Presentation in Chiari Malformation Patients Medical Student Research Journal (2016). doi:10.15404/msrj/08.2016.0007
- Siasios J, Kapsalaki EZ, Fountas KN. Surgical Management of Patients with Chiari I Malformation. Int J Pediatr. 2012;2012:1-10. doi:10.1155/2012/640127.
- Heiss J. Epidemiology of the Chiari I Malformation. In: Tubbs RS, Oakes WJ, eds. The Chiari Malformations. New York: Springer Science + Business Media; 2013:83-92.
- Milhorat TH, Chou MW, Trinidad EM, et al. Chiari I malformation redefined: Clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005-1017. doi:10.1097/00006123-199905000-00042.
- Deng X, Wang K, Wu L, et al. Asymmetry of tonsillar ectopia, syringomyelia and clinical manifestations in adult Chiari I malformation. Acta Neurochir (Wien). 2014;156(4):715-722. doi:10.1007/s00701-014-2000-5.
- Tubbs RS, Wellons JC, Oakes WJ. Asymmetry of tonsillar ectopia in Chiari I malformation. Pediatr Neurosurg. 2002;37(4):199-202. doi:10.1159/000065399.
- Wu T, Zhu Z, Sun X, et al. Is curve direction correlated with the side of dominant displacement of cerebellar tonsil and syrinx deviation in thoracic scoliosis secondary to Chiari malformation type I and syringomyelia? Stud Health Technol Inform. 2012;176(Cmi):286-290. doi:10.3233/978-1-61499-067-3-286.
- Kaplan Y, Oksuz E. Chronic migraine associated with the Chiari type 1 malformation. Clin Neurol Neurosurg. 2008;110(8):818-822. doi:10.1016/j.clineuro.2008.05.016.
- Lewis AR, Kline LB, Sharpe JA. Acquired esotropia due to Arnold-Chiari I malformation. J Neuro-Ophthalmology. 1996;16(1):49-54. <Go to ISI>://WOS:A1996UE52700012.
- Shamji MF, Ventureyra ECG, Baronia B, Nzau M, Vassilyadi M. Classification of symptomatic Chiari I malformation to guide surgical strategy. Can J Neurol Sci. 2010;37(4):482-487. doi:10.1017/S0317167100010507.
- Brandon W. Smith, M.D., M.S.C.R.1, Jennifer Strahle, M.D.1, J. Rajiv Bapuraj, M.D.2, Karin M. Muraszko, M.D.1, Hugh J. L. Garton, M.D., M.H.Sc.1, and Cormac O. Maher MD. Distribution of cerebellar tonsil position: implications for understanding Chiari malformation Clinical article. J Nerosurgery. 2013;119(3):812-819.
- Christophe C, Bernard D. Magnetic resonance imaging cranial and cerebral dimensions: Is there a relationship with Chiari I malformation? A preliminary report in children. Eur J Paediatr Neurol. 1999;3(1):15-24. doi:10.1053/ejpn.1999.0174.
- Fischbein R, Saling JR, Marty P, et al. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurological Sciences. 2015.
- Meeker J, Amerine J, Kropp D, Chyatte M, Fischbein R. The impact of Chiari malformation on daily activities: A report from the national Conquer Chiari Patient Registry database. Disabil Health J. 2015;8(4):521-526. doi:10.1016/j.dhjo.2015.01.003.
- Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, SorelM et al (2009) Chiari type I malformation in a pediatric population. Pediatr Neurol 40(6):449–454
- Speer MC, Enterline DS, Mehltretter L, Hammock P, Joseph J,Dickerson M et al (2014) Chiari type I malformation with or without syringomyelia: prevalence and genetics. J Genet Couns12(4):297–311