Skip to main content
Log in

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

  • Diagnostic Neuroradiology
  • Published:
Neuroradiology Aims and scope Submit manuscript

Summary

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Chiari H (1891) Über Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 17: 1172–1175

    Google Scholar 

  2. Chiari H (1896) Über Veränderungen des Kleinhirns, des Pons und Medulla oblongata infolge von congenitaler Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 63: 71–116

    Google Scholar 

  3. Spillane JD, Pallis C, Jones AM (1957) Developmental abnormalities in the region of the foramen magnum. Brain 80: 11–48

    Google Scholar 

  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: 85–96

    Google Scholar 

  5. Dysthe GN, Menezes AH, Van Gilder JC (1989) Symptomatic Chiari malformations. J Neurosurg 71: 159–168

    Google Scholar 

  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: 147–158

    Google Scholar 

  7. Vega A, Quintana F, Berciano J (1990) Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J Neurol Sci 99: 137–145

    Google Scholar 

  8. Schady W, Metcalfe RA, Butler P (1987) The incidence of craniocervical bony anomalies in the Chiari malformation. J Neurol Sci 82: 193–201

    Google Scholar 

  9. Krogness KG (1978) Posterior fossa measurements. I. The normal size of the posterior fossa. Pediatr Radiol 6: 193–197

    Google Scholar 

  10. Nyland H, Krogness KG (1978) Size of posterior fossa in Chiari type I malformation. Acta Neurochir (Wien) 40: 233–242

    Google Scholar 

  11. Levy WJ, Mason L, Hahn JF (1983) Chiari malformation presenting in adults: a surgical experience with 127 cases. Neurosurgery 12: 377–390

    Google Scholar 

  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: 1033–1036

    Google Scholar 

  13. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position. AJNR 7: 795–799

    Google Scholar 

  14. Woolson RF (1987) Statistical methods for the analysis of biomedical data. Wiley, New York, pp 59–64

    Google Scholar 

  15. Margolis G, Kilham L (1969) Experimental virus-induced hydrocephalus. Relation to pathogenesis of the Arnold-Chiari malformation. J Neurosurg 31: 1–9

    Google Scholar 

  16. Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50: 29–55

    Google Scholar 

  17. Hung CF, Nakagata N, Sato K (1989) The morphogenesis of hindbrain crowding associated with lumbosacral myeloschisis. Neurol Med Chir 29: 981–988

    Google Scholar 

  18. Kruyff E (1965) Occipital dysplasia in infancy. The early recognition of craniovertebral abnormalities. Radiology 85: 501–507

    Google Scholar 

  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: 220–222

    Google Scholar 

  20. Jacobson RI (1985) Abnormalities of the skull in children. Neurol Clin 3: 117–145

    Google Scholar 

  21. Welch K, Shillito J, Strand R (1981) Chiari I “malformation”: an acquired disorder? J Neurosurg 55: 604–609

    Google Scholar 

  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: 639–645

    Google Scholar 

  23. Williams B (1986) Progress in syringomyelia. Neurol Res 8: 130–145

    Google Scholar 

  24. Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelomeningocele. J Neurol Neurosurg Psychiatry 28: 247–259

    Google Scholar 

  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: 596–605

    Google Scholar 

  26. Saez RJ, Onofrio BM, Yanagihara T (1976) Experience with the Arnold-Chiari malformation, 1960–1970. J Neurosurg 45: 416–422

    Google Scholar 

  27. Paul KS, Lye RH, Strang FA, Dutton J (1983) Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58: 183–187

    Google Scholar 

  28. Khurana RK (1991) Headache spectrum in Arnold-Chiari malformation. Headache 31: 151–155

    Google Scholar 

  29. Thomas M, Boyle R (1979) A possible connection between basilar migraine and the Arnold-Chiari malformation. Neurology 29: 527–528

    Google Scholar 

  30. Williams B (1981) Simultaneous cerebral and spinal fluid recordings. 2. Cerebrospinal dissociation with lesion at the foramen magnum. Acta Neurochir (Wien) 59: 123–142

    Google Scholar 

  31. Williams B (1980) Cough headache due to craniospinal pressure dissociation. Arch Neurol 37: 226–230

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Stovner, L.J., Bergan, U., Nilsen, G. et al. Posterior cranial fossa dimensions in the Chiari I malformation: Relation to pathogenesis and clinical presentation. Neuroradiology 35, 113–118 (1993). https://doi.org/10.1007/BF00593966

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00593966

Key words

Navigation