, Volume 25, Issue 4, pp 179–197 | Cite as

The Chiari II malformation: Part IV. The hindbrain deformity

  • T. P. Naidich
  • D. G. McLone
  • K. H. Fulling
Congenital Malformations of the Brain


Computed tomography successfully delineates the multiple components of the Chiari II malformation at the craniocervical junction, the hindbrain, and the cervical spinal cord. These include wide foramen magnum and upper cervical spinal canal; incomplete fusions of the posterior arches of C1 and lower cervical vertebrae; cascading protrusions of vermis, fourth ventricle, medulla, and cervical cord into the spinal canal; cervicomedullary “kinking”; anterior displacement and sequential sagittal compression of each protrusion by the protrusions posterior to it; compression of all protrusions by the posterior lip of foramen magnum and the posterior arch of C1; and associated cervical hydromyelia, cervical diastematomyelia, and cervical arachnoid cysts.

Key words

Brain, abnormalities cerebellum Chiari II malformation computer tomography, head computed tomography, spine hydrocephalus medulla oblongata myelography, metrizamide myelomeningocele skull, abnormalities spinal cord spine, abnormalities vermis 


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  1. 1.
    Emery JL, MacKenzie N (1973) Medullo-cervical dislocation deformity (Chiari II deformity) related to neurospinal dysraphism (meningomyelocele). Brain 96: 155–162Google Scholar
  2. 2.
    Zimmerman RD, Breckbill D, Dennis MW et al (1979) Cranial CT findings in patients with meningomyelocele. AJR 132: 623–629Google Scholar
  3. 3.
    Naidich TP, Pudlowski RM, Naidich JB, Gornish M, Rodriguez FJ (1980) Computed tomographic signs of the Chiari II malformation. Part. I. Skull and dural partitions. Radiology 134: 65–71Google Scholar
  4. 4.
    Naidich TP, Pudlowski RM, Naidich JB (1980) Computed tomographic signs of the Chiari II malformation: Part II. Midbrain and cerebellum. Radiology 134: 391–398Google Scholar
  5. 5.
    Naidich TP, Pudlowski RM, Naidich JB (1980) Computed tomographic signs of the Chiari II malformation: Part III. Ventricles and cisterns. Radiology 134: 657–663Google Scholar
  6. 6.
    Naidich TP (1981) Cranial CT signs of the Chiari II malformation. J Neuroradiol 8: 207–227Google Scholar
  7. 7.
    Bamberger-Bozo C (1982) The Chiari II malformation: Arnold Chiari of the literature. J Neuroradiol 9: 47–70Google Scholar
  8. 8.
    Babcock DS, Han BK (1981) Cranial sonographic findings in meningomyeloceles. AJR 136: 563–569Google Scholar
  9. 9.
    Naidich TP (1982) The craniovertebral junction: Chiari malformations I and II. In: Lissner J, Doppman JL (eds) Internationales Computertomographie Symposium Referate. Schnetztor, Konstanz, pp 78–84Google Scholar
  10. 10.
    Naidich TP, McLone DG, Harwood-Nash DC (1982) Malformations of the craniocervical junction. In: Newton TH, Potts DG (eds) Modern neuroradiology, Vol I. Computed tomography of the spine and spinal cord. (Chapter 18). Clavadel Press, San Anselmo, CAGoogle Scholar
  11. 11.
    Barry A, Patten BM, Stewart BH (1957) Possible factors in the development of the Arnold-Chiari malformation. J Neurosurg 14: 285–301Google Scholar
  12. 12.
    Daniel PM, Strich SJ (1958) Some observations on the congenital deformity of the central nervous system known as the Arnold-Chiari malformation. J Neuropathol Exp Neurol 17: 255–266Google Scholar
  13. 13.
    Friede RL (1975) The Arnold-Chiari malformation (Cleland-Chiari malformation). In: Friede RL (ed) Developmental neuropathology, (Chapter 25). Springer, New York Wien, pp 253–266Google Scholar
  14. 14.
    Osaka K, Matsumoto S, Tanimura T (1978) Myeloschisis in early human embryos. Childs Brain 4: 347–359Google Scholar
  15. 15.
    Osaka K, Tanimura T, Hirayama A, Matsumoto S (1978) Myelomeningocele before birth. J Neurosurg 49: 711–724Google Scholar
  16. 16.
    Cameron AH (1957) The Arnold-Chiari and other neuro-anatomical malformations associated with spina bifida. J Pathol Bacteriol 73: 195–211Google Scholar
  17. 17.
    Gardner WJ (1959) Anatomic features common to Arnold-Chiari and Dandy-Walker malformations suggest common origin. Cleve Clin Q 26: 206–222Google Scholar
  18. 18.
    Gardner WJ (1960) Myelomeningocele, the result of rupture of the embryonic neural tube. Cleve Clin Q 27: 88–100Google Scholar
  19. 19.
    Patten BM (1952) Overgrowth of the neural tube in young human embryos. Anat Rec 113: 381–393Google Scholar
  20. 20.
    Peach B (1965) Arnold-Chiari malformation: morphogenesis. Arch Neurol 12: 527–535Google Scholar
  21. 21.
    van Hoytema GJ, van den Berg R (1966) Embryological studies of the posterior fossa in connection with Arnold-Chiari malformation. Dev Med Child Neurol [Suppl] 11: 61–76Google Scholar
  22. 22.
    Brocklehurst G (1969) A quantitative study of a spina bifida foetus. J Pathol 99: 205–211Google Scholar
  23. 23.
    Williams B (1977) On the pathogenesis of the Chiari malformation. Z Kinderchir 22: 533–553Google Scholar
  24. 24.
    Peach B (1965) Arnold-Chiari malformation. Anatomic features of 10 cases. Arch Neurol 12: 613–621Google Scholar
  25. 25.
    Kruyff E, Jeffs R (1966) Skull abnormalities associated with the Arnold-Chiari malformation. Acta Radiol [Diagn] (Stockh) 5: 9–24Google Scholar
  26. 26.
    Blaauw G (1971) Defect in posterior arch of atlas in myelomeningocele. Dev Med Child Neurol [Suppl] 25: 113–115Google Scholar
  27. 27.
    Peach B (1964) The Arnold-Chiari malformation with normal spine. Arch Neurol 10: 497–501Google Scholar
  28. 28.
    Teng P, Papatheodorou C (1965) Arnold-Chiari malformation with normal spine and cranium. Arch Neurol 12: 622–624Google Scholar
  29. 29.
    MacKenzie NG, Emery JL (1971) Deformities of the cervical cord in children with neurospinal dysraphism. Dev Med Child Neurol [Suppl] 25: 58–67Google Scholar
  30. 30.
    Emery JL, Naik D (1968) Spinal cord segment lengths in children with meningomyelocele and the “Cleland Arnold Chiari” deformity. Br J Radiol 41: 287–290Google Scholar
  31. 31.
    Naik DR, Emery JL (1968) The position of the spinal cord segments related to the vertebral bodies in children with meningomyelocele and hydrocephalus. Dev Med Child Neurol [Suppl] 16: 62–68Google Scholar
  32. 32.
    Lendon RG (1968) Neuron population in the spinal cord of children with spina bifida and hydrocephalus. Dev Med Child Neurol [Suppl] 15: 50–54Google Scholar
  33. 33.
    Ogryzlo MA (1942) The Arnold-Chiari malformation. Arch Neurol Psychiatry 48: 30–46Google Scholar
  34. 34.
    Peach B (1964) Cystic prolongation of fourth ventricle. Arch Neurol 11: 609–612Google Scholar
  35. 35.
    Emery JL (1967) Kinking of the medulla in children with acute cerebral oedema and hydrocephalus and its relationship to the dentate ligaments. J Neurol Neurosurg Psychiatry 30: 267–275Google Scholar
  36. 36.
    Stoltmann HF, Blackwood W (1966) An anatomical study of the role of the dentate ligaments in the cervical spinal canal. J Neurosurg 24: 43–46Google Scholar
  37. 37.
    Howell DA (1959) Upper brain stem compression and foraminal impaction with intracranial space-occupying lesions and brain swelling. Brain 82: 525–550Google Scholar
  38. 38.
    Variend S, Emery JL (1973) The weight of the cerebellum in children with myelomeningocele. Dev Med Child Neurol [Suppl] 29: 77–83Google Scholar
  39. 39.
    Variend S (1974) Deformity of the lateral cerebellar lobes in children with meningomyelocele. Arch Dis Child 49: 495–496Google Scholar
  40. 40.
    Variend S, Emery JL (1976) Cervical dislocation of the cerebellum in children with meningomyelocele. Teratology 13: 281–290Google Scholar
  41. 41.
    Variend S, Emery JL (1979) The superior surface lesion of the cerebellum in children with myelomeningocele. Z Kinderchir Grenzgeb 28: 328–335Google Scholar
  42. 42.
    Variend S, Emery JL (1974) The pathology of the central lobes of the cerebellum in children with myelomeningocele. Dev Med Child Neurol [Suppl] 32: 99–106Google Scholar
  43. 43.
    Park TS, Hoffman HJ, Hendrick EB, Humphreys RP (1982) Posterior fossa decompression for Arnold-Chiari malformation in young infants with meningomyelocele. Presented at the 10th Annual Meeting of the International Society for Paediatric Neurosurgery, Philadelphia, PA, 12–15 September 1982Google Scholar
  44. 44.
    Emery JL, Gadsdon DR (1975) A quantitative study of the cell population of the cerebellum in children with myelomeningocele. Dev Med Child Neurol [Suppl] 35: 20–25Google Scholar
  45. 45.
    Emery JL, Lendon RG (1973) The local cord lesion in neurospinal dysraphism (meningomyelocele). J Pathol 110: 83–96Google Scholar
  46. 46.
    Batnitzky S, Hall PV, Lindseth RE, Wellman HN (1976) Meningomyelocele and syringohydromyelia: some radiological aspects. Radiology 120: 351–357Google Scholar
  47. 47.
    Hall PV, Campbell RL, Kalsbeck JE (1975) Meningomyelocele and progressive hydromyelia: progressive paresis in myelodysplasia. J Neurosurg 43: 457–463Google Scholar
  48. 48.
    Hall PV, Lindseth R, Campbell R, Kalsbeck JE, Desousa A (1979) Scoliosis and hydrocephalus in myelocele patients: the effects of ventricular shunting. J Neurosurg 50: 174–178Google Scholar
  49. 49.
    Emery JL, Levick RK (1965) The movement of the brain stem and vessels around the brain stem in children with hydrocephalus and the Arnold-Chiari deformity. Dev Med Child Neurol [Suppl] 11: 49–60Google Scholar
  50. 50.
    Gabrielsen TO, Seeger JF, Amundsen P (1975) Some new angiographic observations in patients with Chiari type I and II malformations. Radiology 115: 627–634Google Scholar
  51. 51.
    Greitz T, Sjogren SE (1963) The posterior inferior cerebellar artery. Acta Radiol [Diagn] (Stockh) 1: 284–297Google Scholar
  52. 52.
    Margolis MT, Newton TH (1971) An angiogrphic sign of cerebellar tonsillar hemiation. Neuroradiology 2: 3–8Google Scholar
  53. 53.
    Raimondi AJ (1972) Pediatric neuroradiology. Saunders, PhiladelphiaGoogle Scholar
  54. 54.
    Raimondi AJ, Cerullo LJ (1980) Pediatric cerebral angiography. A descriptive atlas. In: Nadjmi M (ed) Neuroradiological atlantes. Thieme, Stuttgart New YorkGoogle Scholar

Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • T. P. Naidich
    • 1
    • 3
  • D. G. McLone
    • 2
    • 3
  • K. H. Fulling
    • 4
  1. 1.Department of RadiologyNorthwestern University Medical SchoolChicagoUSA
  2. 2.Department of Surgery, Division of NeurosurgeryNorthwestern University Medical SchoolChicagoUSA
  3. 3.The Children's Memorial HospitalChicagoUSA
  4. 4.Department of Pathology, Division of NeuropathologyWashington University Medical SchoolSt. LouisUSA

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