, Volume 35, Issue 2, pp 113–118 | Cite as

Posterior cranial fossa dimensions in the Chiari I malformation: Relation to pathogenesis and clinical presentation

  • L. J. Stovner
  • U. Bergan
  • G. Nilsen
  • O. Sjaastad
Diagnostic Neuroradiology


Skull dimensions were measured on lateral skull radiographs in 33 adult patients with MRI-verified Chiari I malformations and in 40 controls. The posterior cranial fossa was significantly smaller and shallower in patients than in controls. In the patients, there was a positive correlation between posterior fossa size and the degree of the cerebellar ectopia, which might indicate that a posterior cranial fossa which was originally too small had been expanded by the herniation of hindbrain structures at an early stage. Pyramidal signs and cerebellar symptoms and signs, which may be due to compression of neural structures, were associated with a large degree of ectopia and a relatively large posterior cranial fossa. Syringomyelia and headache, which may be due to the valve action of the herniated cerebellar tissue, were not associated with a particularly large posterior fossa or herniation. No special clinical presentation was associated with a very small posterior cranial fossa, which may indicate that a small posterior cranial fossa per se has little or no clinical significance, although it may be the primary developmental anomaly.

Key words

Chiari I malformation Magnetic resonance imaging Pathogenesis Posterior cranial fossa Skull radiography 


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  1. 1.
    Chiari H (1891) Über Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 17: 1172–1175Google Scholar
  2. 2.
    Chiari H (1896) Über Veränderungen des Kleinhirns, des Pons und Medulla oblongata infolge von congenitaler Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 63: 71–116Google Scholar
  3. 3.
    Spillane JD, Pallis C, Jones AM (1957) Developmental abnormalities in the region of the foramen magnum. Brain 80: 11–48Google Scholar
  4. 4.
    Mohr PD, Strang FA, Sambrook MA, Boddie HG (1977) The clinical and surgical features in 40 patients with primary cerebellar ektopia (adult Chiari malformation). Q J Med 46: 85–96Google Scholar
  5. 5.
    Dysthe GN, Menezes AH, Van Gilder JC (1989) Symptomatic Chiari malformations. J Neurosurg 71: 159–168Google Scholar
  6. 6.
    Coria F, Quintana F, Rebollo M, Combarros O, Berciano J (1983) Occipital dysplasia and Chiari type I deformity in a family. J Neurol Sci 62: 147–158Google Scholar
  7. 7.
    Vega A, Quintana F, Berciano J (1990) Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J Neurol Sci 99: 137–145Google Scholar
  8. 8.
    Schady W, Metcalfe RA, Butler P (1987) The incidence of craniocervical bony anomalies in the Chiari malformation. J Neurol Sci 82: 193–201Google Scholar
  9. 9.
    Krogness KG (1978) Posterior fossa measurements. I. The normal size of the posterior fossa. Pediatr Radiol 6: 193–197Google Scholar
  10. 10.
    Nyland H, Krogness KG (1978) Size of posterior fossa in Chiari type I malformation. Acta Neurochir (Wien) 40: 233–242Google Scholar
  11. 11.
    Levy WJ, Mason L, Hahn JF (1983) Chiari malformation presenting in adults: a surgical experience with 127 cases. Neurosurgery 12: 377–390Google Scholar
  12. 12.
    Aboulezz AO, Sartor K, Geyer CA, Gado MH (1985) Position of cerebellar tonsils in the normal population and in patients with the Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9: 1033–1036Google Scholar
  13. 13.
    Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position. AJNR 7: 795–799Google Scholar
  14. 14.
    Woolson RF (1987) Statistical methods for the analysis of biomedical data. Wiley, New York, pp 59–64Google Scholar
  15. 15.
    Margolis G, Kilham L (1969) Experimental virus-induced hydrocephalus. Relation to pathogenesis of the Arnold-Chiari malformation. J Neurosurg 31: 1–9Google Scholar
  16. 16.
    Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50: 29–55Google Scholar
  17. 17.
    Hung CF, Nakagata N, Sato K (1989) The morphogenesis of hindbrain crowding associated with lumbosacral myeloschisis. Neurol Med Chir 29: 981–988Google Scholar
  18. 18.
    Kruyff E (1965) Occipital dysplasia in infancy. The early recognition of craniovertebral abnormalities. Radiology 85: 501–507Google Scholar
  19. 19.
    Stovner LJ, Cappelen J, Nilsen G, Sjaastad O (1992) The Chiari type I malformation in two MZ twins and first degree relatives. Ann Neurol 31: 220–222Google Scholar
  20. 20.
    Jacobson RI (1985) Abnormalities of the skull in children. Neurol Clin 3: 117–145Google Scholar
  21. 21.
    Welch K, Shillito J, Strand R (1981) Chiari I “malformation”: an acquired disorder? J Neurosurg 55: 604–609Google Scholar
  22. 22.
    Pillay PK, Awad IA, Little JD, Hahn JF (1991) Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery 28: 639–645Google Scholar
  23. 23.
    Williams B (1986) Progress in syringomyelia. Neurol Res 8: 130–145Google Scholar
  24. 24.
    Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelomeningocele. J Neurol Neurosurg Psychiatry 28: 247–259Google Scholar
  25. 25.
    De Barros MC, Farias W, Ataide L, Lins S (1968) Basilar impression and Arnold Chiari malformation. A study of 66 cases. J Neurol Neurosurg Psychiatry 31: 596–605Google Scholar
  26. 26.
    Saez RJ, Onofrio BM, Yanagihara T (1976) Experience with the Arnold-Chiari malformation, 1960–1970. J Neurosurg 45: 416–422Google Scholar
  27. 27.
    Paul KS, Lye RH, Strang FA, Dutton J (1983) Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58: 183–187Google Scholar
  28. 28.
    Khurana RK (1991) Headache spectrum in Arnold-Chiari malformation. Headache 31: 151–155Google Scholar
  29. 29.
    Thomas M, Boyle R (1979) A possible connection between basilar migraine and the Arnold-Chiari malformation. Neurology 29: 527–528Google Scholar
  30. 30.
    Williams B (1981) Simultaneous cerebral and spinal fluid recordings. 2. Cerebrospinal dissociation with lesion at the foramen magnum. Acta Neurochir (Wien) 59: 123–142Google Scholar
  31. 31.
    Williams B (1980) Cough headache due to craniospinal pressure dissociation. Arch Neurol 37: 226–230Google Scholar

Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • L. J. Stovner
    • 1
  • U. Bergan
    • 2
  • G. Nilsen
    • 3
  • O. Sjaastad
    • 1
  1. 1.Department of NeurologyTrondheim University HospitalTrondheimNorway
  2. 2.Department of RadiologyTrondheim University HospitalTrondheimNorway
  3. 3.MP-CentréUniversity of TrondheimTrondheimNorway

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