Acta Neurochirurgica

, Volume 157, Issue 8, pp 1411–1423 | Cite as

Comparison of pulsatile and static pressures within the intracranial and lumbar compartments in patients with Chiari malformation type 1: a prospective observational study

  • Radek FričEmail author
  • Per Kristian Eide
Clinical Article - Spine



In Chiari malformation type 1 (CMI), the obstruction of cerebrospinal fluid (CSF) flow through the foramen magnum is believed to cause alterations of intracranial pressure (ICP) pulsations. Foramen magnum decompression (FMD) is therefore considered a treatment of choice. However, the pathophysiology of CMI is poorly understood and it remains unknown how ICP alterations relate to symptoms and radiological findings. This study was undertaken to measure pulsatile pressure and its gradient between intracranial and lumbar compartments, and to determine its relationship to clinical and radiological findings.


In patients with symptomatic CMI, we simultaneously measured ICP and lumbar CSF pressure, with particular focus on analysis of pulsatile pressure. Ventricular CSF volume (VV), intracranial volume (ICV) and posterior cranial fossa volume (PCFV) were calculated using volumetry software.


In 26 patients (median 35 years), we found clearly abnormal or borderline values of pulsatile ICP in 18/26 patients (69 %; median 4.5 mmHg) and abnormal pulsatile pressure gradient in 17/24 patients (71 %; median 2.6 mmHg). The correlation between pulsatile ICP and the pulsatile pressure gradient was significantly positive (p < 0.001). We found no significant correlation between pulsatile or static pressure and extent of tonsillar ectopy, VV, ICV or PCFV. The pulsatile pressure gradient was significantly higher in patients with syringomyelia (p = 0.02).


In this cohort, the pulsatile ICP was elevated in 69 %. The intracranial-lumbar pulsatile pressure gradient was abnormal in 71 % and significantly higher in patients with syringomyelia. The elevated pulsatile ICP significantly correlated with pulsatile pressure gradient; no similar correlation was found for static ICP. We interpret the results as providing evidence of impaired intracranial compliance as an important pathophysiological mechanism in CMI.


Chiari malformation type I Cerebrospinal fluid dynamics Foramen magnum Syringomyelia Intracranial pressure 


Conflicts of Interest

R. Frič discloses no conflicts of interests. The software used for analysis of the ICP recordings (Sensometrics Software) is manufactured by a software company (dPCom AS, Oslo, Norway) in which P.K. Eide has a financial interest.


  1. 1.
    Aiken AH, Hoots JA, Saindane AM, Hudgins PA (2012) Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol 33:1901–1906CrossRefPubMedGoogle Scholar
  2. 2.
    Aydin S, Hanimoglu H, Tanriverdi T, Yentur E, Kaynar MY (2005) Chiari type I malformations in adults: a morphometric analysis of the posterior cranial fossa. Surg Neurol 64:237–241, discussion 241 CrossRefPubMedGoogle Scholar
  3. 3.
    Bagci AM, Lee SH, Nagornaya N, Green BA, Alperin N (2013) Automated posterior cranial fossa volumetry by MRI: applications to Chiari malformation type I. AJNR Am J Neuroradiol 34:1758–1763PubMedCentralCrossRefPubMedGoogle Scholar
  4. 4.
    Banik R, Lin D, Miller NR (2006) Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 247:71–75CrossRefPubMedGoogle Scholar
  5. 5.
    Bejjani GK (2003) Association of the Adult Chiari Malformation and Idiopathic Intracranial Hypertension: more than a coincidence. Med Hypotheses 60:859–863CrossRefPubMedGoogle Scholar
  6. 6.
    Chiari H (1987) Concerning alterations in the cerebellum resulting from cerebral hydrocephalus. 1891. Pediatr Neurosci 13:3–8CrossRefPubMedGoogle Scholar
  7. 7.
    Czosnyka M, Pickard JD (2004) Monitoring and interpretation of intracranial pressure. J Neurol Neurosurg Psychiatry 75:813–821PubMedCentralCrossRefPubMedGoogle Scholar
  8. 8.
    Dagtekin A, Avci E, Kara E, Uzmansel D, Dagtekin O, Koseoglu A, Talas D, Bagdatoglu C (2011) Posterior cranial fossa morphometry in symptomatic adult Chiari I malformation patients: comparative clinical and anatomical study. Clin Neurol Neurosurg 113:399–403CrossRefPubMedGoogle Scholar
  9. 9.
    Eide PK, Bentsen G, Sorteberg AG, Marthinsen PB, Stubhaug A, Sorteberg W (2011) A randomized and blinded single-center trial comparing the effect of intracranial pressure and intracranial pressure wave amplitude-guided intensive care management on early clinical state and 12-month outcome in patients with aneurysmal subarachnoid hemorrhage. Neurosurgery 69:1105–1115PubMedGoogle Scholar
  10. 10.
    Eide PK, Brean A (2006) Lumbar cerebrospinal fluid pressure waves versus intracranial pressure waves in idiopathic normal pressure hydrocephalus. Br J Neurosurg 20:407–414CrossRefPubMedGoogle Scholar
  11. 11.
    Eide PK, Brean A (2010) Cerebrospinal fluid pulse pressure amplitude during lumbar infusion in idiopathic normal pressure hydrocephalus can predict response to shunting. Cerebrospinal Fluid Res 7:5PubMedCentralCrossRefPubMedGoogle Scholar
  12. 12.
    Eide PK, Kerty E (2011) Static and pulsatile intracranial pressure in idiopathic intracranial hypertension. Clin Neurol Neurosurg 113:123–128CrossRefPubMedGoogle Scholar
  13. 13.
    Eide PK, Sorteberg W (2007) Association among intracranial compliance, intracranial pulse pressure amplitude and intracranial pressure in patients with intracranial bleeds. Neurol Res 29:798–802CrossRefPubMedGoogle Scholar
  14. 14.
    Eide PK, Sorteberg W (2010) Diagnostic intracranial pressure monitoring and surgical management in idiopathic normal pressure hydrocephalus: a 6-year review of 214 patients. Neurosurgery 66:80–91CrossRefPubMedGoogle Scholar
  15. 15.
    Fagan LH, Ferguson S, Yassari R, Frim DM (2006) The Chiari pseudotumor cerebri syndrome: symptom recurrence after decompressive surgery for Chiari malformation type I. Pediatr Neurosurg 42:14–19CrossRefPubMedGoogle Scholar
  16. 16.
    Furtado SV, Reddy K, Hegde AS (2009) Posterior fossa morphometry in symptomatic pediatric and adult Chiari I malformation. J Clinical Neurosci 16:1449–1454CrossRefGoogle Scholar
  17. 17.
    Furtado SV, Visvanathan K, Reddy K, Hegde AS (2009) Pseudotumor cerebri: as a cause for early deterioration after Chiari I malformation surgery. Childs Nerv Syst 25:1007–1012CrossRefPubMedGoogle Scholar
  18. 18.
    Gardner WJ, Angel J (1959) The mechanism of syringomyelia and its surgical correction. Clin Neurosurg 6:131–140Google Scholar
  19. 19.
    Gonzalez-Darder JM, Barcia-Salorio JL (1989) Pulse amplitude and volume-pressure relationships in experimental hydrocephalus. Acta Neurochir (Wien) 97:166–170CrossRefGoogle Scholar
  20. 20.
    Greitz D (2006) Unraveling the riddle of syringomyelia. Neurosurg Rev 29:251–263, discussion 264 CrossRefPubMedGoogle Scholar
  21. 21.
    Häckel M, Benes V, Mohapl M (2001) Simultaneous cerebral and spinal fluid pressure recordings in surgical indications of the Chiari malformation without myelodysplasia. Acta Neurochir (Wien) 143:909–917, discussion 918 CrossRefGoogle Scholar
  22. 22.
    Heiss JDPN, DeVroom HL, Shawker T, Ennis R, Kammerer W, Eidsath A, Talbot T, Morris J, Eskioglu E, Oldfield EH (1999) Elucidating the pathophysiology of syringomyelia. J Neurosurg 91:553–562CrossRefPubMedGoogle Scholar
  23. 23.
    Hofmann E, Warmuth-Metz M, Bendszus M, Solymosi L (2000) Phase-contrast MR imaging of the cervical CSF and spinal cord: volumetric motion analysis in patients with Chiari I malformation. AJNR Am J Neuroradiol 21:151–158PubMedGoogle Scholar
  24. 24.
    Klekamp J (2002) The pathophysiology of syringomyelia - historical overview and current concept. Acta Neurochir (Wien) 144:649–664CrossRefGoogle Scholar
  25. 25.
    Klekamp J (2012) Surgical treatment of Chiari I malformation—analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. Neurosurgery 71:365–380, discussion 380 CrossRefPubMedGoogle Scholar
  26. 26.
    Krayenbuhl H (1975) Evaluation of the different surgical approaches in the treatment of syringomyelia. Clin Neurology Neurosurg 77:111–128Google Scholar
  27. 27.
    Kurschel S, Maier R, Gellner V, Eder HG (2007) Chiari I malformation and intra-cranial hypertension:a case-based review. Childs Nerv Syst 23:901–905CrossRefPubMedGoogle Scholar
  28. 28.
    Malone IB, Leung KK, Clegg S, Barnes J, Whitwell JL, Ashburner J, Fox NC, Ridgway GR (2014) Accurate automatic estimation of total intracranial volume: a nuisance variable with less nuisance. Neuroimage 104:366–372CrossRefPubMedGoogle Scholar
  29. 29.
    McGirt MJ, Nimjee SM, Fuchs HE, George TM (2006) Relationship of cine phase-contrast magnetic resonance imaging with outcome after decompression for Chiari I malformations. Neurosurgery 59:140–146, discussion 140-146 CrossRefPubMedGoogle Scholar
  30. 30.
    Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920–926CrossRefPubMedGoogle Scholar
  31. 31.
    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 44:1005–1017CrossRefPubMedGoogle Scholar
  32. 32.
    Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD (2010) Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir (Wien) 152:1117–1127CrossRefGoogle Scholar
  33. 33.
    Morris Z, Whiteley WN, Longstreth WT Jr, Weber F, Lee YC, Tsushima Y, Alphs H, Ladd SC, Warlow C, Wardlaw JM, Al-Shahi Salman R (2009) Incidental findings on brain magnetic resonance imaging: systematic review and meta-analysis. BMJ 339:b3016PubMedCentralCrossRefPubMedGoogle Scholar
  34. 34.
    Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15CrossRefPubMedGoogle Scholar
  35. 35.
    Poca MA, Sahuquillo J, Ibanez J, Amoros S, Arikan F, Rubio E (2002) Intracranial hypertension after surgery in patients with Chiari I malformation and normal or moderate increase in ventricular size. Acta Neurochir Suppl (Wien) 81:35–38Google Scholar
  36. 36.
    Poca MA, Sahuquillo J, Topczewski T, Lastra R, Font ML, Corral E (2006) Posture-induced changes in intracranial pressure: a comparative study in patients with and without a cerebrospinal fluid block at the craniovertebral junction. Neurosurgery 58:899–906, discussion 899-906 CrossRefPubMedGoogle Scholar
  37. 37.
    Schmitt M, Kiefer M, Antes S, Eymann R (2012) Detection of hidden pseudotumour cerebri behind Chiari 1 malformation: value of telemetric ICP monitoring. Child Nerv Syst 28:1811–1813CrossRefGoogle Scholar
  38. 38.
    Sinclair N, Assaad N, Johnston I (2002) Pseudotumour cerebri occurring in association with the Chiari malformation. J Clin Neurosci 9:99–101CrossRefPubMedGoogle Scholar
  39. 39.
    Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr 8:205–213CrossRefPubMedGoogle Scholar
  40. 40.
    Tisell M, Wallskog J, Linde M (2009) Long-term outcome after surgery for Chiari I malformation. Acta Neurol Scand 120:295–299CrossRefPubMedGoogle Scholar
  41. 41.
    Trigylidas T, Baronia B, Vassilyadi M, Ventureyra EC (2008) Posterior fossa dimension and volume estimates in pediatric patients with Chiari I malformations. Childs Nerv Syst 24:329–336CrossRefPubMedGoogle Scholar
  42. 42.
    Vernooij MW, Ikram MA, Tanghe HL, Vincent AJ, Hofman A, Krestin GP, Niessen WJ, Breteler MM, van der Lugt A (2007) Incidental findings on brain MRI in the general population. N Eng J Med 357:1821–1828CrossRefGoogle Scholar
  43. 43.
    Wang CS, Wang X, Fu CH, Wei LQ, Zhou DQ, Lin JK (2014) Analysis of cerebrospinal fluid flow dynamics and morphology in Chiari I malformation with cine phase-contrast magnetic resonance imaging. Acta Neurochir (Wien) 156:707–713CrossRefGoogle Scholar
  44. 44.
    Williams B (1978) A critical appraisal of posterior fossa surgery for communicating syringomyelia. Brain 101:223–250CrossRefPubMedGoogle Scholar
  45. 45.
    Williams B (1981) Simultaneous cerebral and spinal fluid pressure recordings. 2. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir (Wien) 59:123–142CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 2015

Authors and Affiliations

  1. 1.Department of NeurosurgeryOslo University Hospital—RikshospitaletOsloNorway
  2. 2.Faculty of MedicineUniversity of OsloOsloNorway

Personalised recommendations