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Vol. 45, No. 5, 2009
Issue release date: December 2009
Pediatr Neurosurg 2009;45:384–389

Chiari Drop Attacks: Surgical Decompression and the Role of Tilt Table Testing

Straus D. · Foster K. · Zimmerman F. · Frim D.
aPritzker School of Medicine and Sections of bPediatric Cardiology and cNeurosurgery, University of Chicago, Chicago, Ill., USA

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Background: Chiari I malformation (CM1) is characterized by impaired CSF flow through the foramen magnum. Dysfunctional autonomic cardiovascular regulation may result in syncope. Syncope may be the primary presenting symptom of CM1: a syndrome termed Chiari drop attack. It has been postulated that Chiari drop attack is secondary to dysautonomia caused by hindbrain compression. There has been recent debate regarding the association between CM1, dysautonomia and Chiari drop attack. Methods: We selected patients with Chiari drop attacks who had negative workups for cardiac syncope, followed by tilt table testing and subsequent surgical decompression. We report test results and clinical outcomes following CM1 decompression. Results: Ten patients met the inclusion criteria: 5 patients had positive and 5 negative tilt table tests. Following decompression, 7 had symptomatic improvement or resolution and 3 failed to improve. The sensitivity and specificity of the tilt table test for detecting clinical improvement with surgical decompression was 43 and 33%, respectively. Tilt table testing had 40% accuracy in predicting clinical response to decompression. Conclusions: In this short series, surgical decompression of CM1 has a high success rate (70%) for patients with Chiari drop attacks. Tilt table testing has poor predictive value in judging the clinical response to surgical decompression and is not a useful test to guide surgical decision- making.

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  1. Chumas PP, Amstrong DC, Drake JM, Kulkarni AV, Hoffman HJ, Humphreys RP, Rutka JT, Hendrick EB: Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population. J Neurosurg 1993;78:568–573.
  2. Atkinson JLD, Weinshenker BG, Miller GM, Piepgras DG, Mokri B: Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial syndrome in seven cases. J Neurosurg 1998;88:237–242.
  3. Johnston I, Jacobson E, Besser M: The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management. Acta Neurochir (Wien) 1998;140:417–428.
  4. Sathi S, Stieg PE: ‘Acquired’ Chiari I malformation after multiple lumbar punctures: case report. Neurosurgery 1993;32:306–309.
  5. Paul KS, Lye RH, Strang FA, et al: Arnold-Chiari malformation: review of 71 cases. J Neurosurg 1983;58:183–187.
  6. Levy WJ, Mason L, Hahn JF: Chiari malformation in adults: a surgical experience in 127 cases. Neurosurgery 1983;12:377–390.
  7. Meadows J, Kraut M, Guarnieri M, et al: Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg 2000;92:920–926.
  8. Freeman R: Neurogenic orthostatic hypotension. N Engl J Med 2008;358:615–624.
  9. Linzer M, Yang EH, Estes M, Wang P, Vorperian V, Kapoor WN: Diagnosing syncope. 1. Value of history, physical exam and electrocardiography. Ann Intern Med 1997;126:989–996.
  10. European Society of Cardiology Taskforce on Syncope: Guidelines on the management (diagnosis and treatment) of syncope – update 2004. Europace 2004;6:467–537.
  11. American College of Cardiology expert consensus document: tilt table testing for assessing syncope. J Am Coll Cardiol 1996;28:263–275.
  12. Kapoor WN: Evaluation and outcome of patients with syncope. Medicine (Baltimore) 1990;69:160–175.
  13. Prilipko O, Dehdashti AR, Zaim S, Seeck M: Orthostatic intolerance and syncope associated with Chiari type I malformation. J Neurol Neurosurg Psychiatry 2005;76:1034–1036.
  14. Pandey A, Robinson S, Cohen AR: Cerebellar fits in children with Chiari I malformation. Neurosurg Focus 2001;11:E4.
  15. Hampton F, Williams B, Loizou LA: Syncope as a presenting feature of hindbrain herniation with syringomyelia. J Neurol Neurosurg Psychiatry 1982;45:919–922.
  16. Larson SJ, Sances A, Baker JB, Reigel DH: Herniated cerebellar tonsils and cough syncope. J Neurosurg 1974;40:524–528.
  17. Ireland PD, Mickelsen D, Rodenhouse TG, et al: Evaluation of the autonomic cardiovascular response in Arnold-Chiari deformities and cough syncope syndrome. Arch Neurol 1996;53:526–531.
  18. Weig S, Buckthal P, Choi S, Zellem R: Recurrent syncope as the presenting symptom of Arnold-Chiari malformation. Neurology 1991;41:1673–1674.
  19. Cirignotta F, Coccagna G, Zucconi M, et al: Sleep apneas, convulsive syncopes and autonomic impairment in type I Arnold-Chiari malformation. Eur Neurol 1991;31:36–40.
  20. Garland EM, Robertson D: Chiari I malformation as a cause of orthostatic intolerance symptoms: a media myth? Am J Med 2001;111:546–552.
  21. Garland EM, Anderson JC, Black BK, Kessler RM, Konrad PE, Robertson D: No increased herniation of the cerebellar tonsils in a group of patients with orthostatic intolerance. Clin Auton Res 2002;12:472–476.
  22. Schondorf R, Freeman R: The importance of orthostatic intolerance in the chronic fatigue syndrome. Am J Med Sci 1999;317:117–123.
  23. Jacob G, Biaggoni I: Idiopathic orthostatic intolerance and postural tachycardia syndromes. Am J Med Sci 1999;317:88–101.
  24. Mosqueda-Garcia R, Furlan R, Tank J, Fernandez-Violante R: The elusive pathophysiology of neurally mediated syncope. Circulation 2000;102:2898–2906.
  25. Leonelli FM, Wand K, Evans JM, Patwardhan AR, Ziegler MG, Natale A, Kim CS, Rajikovich K, Knapp CF: False positive head-up tilt: hemodynamic and neurohormonal profile. J Am Coll Cardiol 2000;35:188–193.

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