Top

Child's Nervous System

Published in:

01-05-2004 | Focus Session

History, anatomic forms, and pathogenesis of Chiari I malformations

Author: Edgardo Schijman

Published in: Child's Nervous System | Issue 5/2004

Abstract

Introduction

Chiari I malformations constitute a group of entities of congenital or acquired etiology that have in common descent of the cerebellar tonsils into the cervical spinal canal. In recent years, since the advent of magnetic resonance imaging, an increasing number of asymptomatic, doubtfully symptomatic, and minimally symptomatic patients with Chiari I malformations have been diagnosed. This has resulted in controversy about the multiple therapeutic strategies indicated for these problems.

Object

With the intention of updating the knowledge that we currently have on the Chiari I malformation and the related syringomyelia we review the literature and discuss the historical background, pathogenesis, anatomic forms, clinical presentation, and diagnostic procedures of these abnormalities.

Abee R, Cooley WB (1892) Syringomyelia. Operation: exploration of cord, withdrawal of fluid, exhibition of patient. J Nerv Ment Dis 19:512–520

Aboulezz AO, Sartor K, Geyer CA, Gado MH (1985) Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9:1033–1036PubMed

Aboulker J (1979) La syringomyélie et les liquides intra-rachidiens. Neurochirurgie 25 [Suppl 1]:9–144

Ackermann JF (1790) Über die Kretinen, eine besondere Menschenabart in den Alpen. Gotha

Adams RD, Schatzki R, Scoville WB (1941) The Arnold-Chiari malformation. Diagnosis, demonstration by intraspinal lipiodol and successful surgical treatment. N Eng J Med 225:125–131

Aring CD (1938) Cerebellar syndrome in an adult with malformation of the cerebellum and brain stem (Arnold-Chiari deformity), with a note on the occurrence of “torpedoes” in the cerebellum. J Neurol Neurosurg Psychiatry 1:100–109

Armonda RA, Citrimin CM, Foley KT, Ellenbogen RG (1994) Quantitative cine-mode magnetic resonance imaging of Chiari I malformations: an analysis of cerebrospinal fluid dynamics. Neurosurgery 35:214–224

Arnold J (1894) Myelocyste, Transposition von Gewebskeimen und Sympodie. Beitr Path Anat 16:1–28

Atkinson JLD, Weinshenker BG, Miller GM, Piepgras DG, Mokri B (1998) Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial syndrome in seven cases. J Neurosurg 88:237–242PubMed

10.

Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218PubMed

11.

Baker HL Jr (1963) Myelographic examination of the posterior fossa with positive contrast medium. Radiology 81:791–801PubMed

12.

Ball MJ, Dayan AD (1972) Pathogenesis of syringomyelia. Lancet 2:799–801

13.

Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. Am J Neuroradiol 7:795–799PubMed

14.

Bejjani GK, Cockerham KP (2001) Adult Chiari malformation. Contemp Neurosurg 23:1–7

15.

Bloch S, van Rensburg MJ, Danziger J (1974) The Arnold-Chiari malformation. Clin Radiol 25:335–341PubMed

16.

Brill CB, Gutierrez J, Mishkin M (1998) Chiari I malformation: association with seizures and development disabilities. J Child Neurol 12:101–106

17.

Brunner JC (1700) De Hydrocephalo, sive Hydrops capitis. In: Bonneti T (ed) Sepelchretum, book I, 2nd edn. Cramer and Perachin, Geneva, p 396

18.

Cameron AH (1957) The Arnold-Chiari and other neuroanatomical malformations associated with spina bifida. J Pathol 73:195–211

19.

Cavender RK, Schmidt JH (1995) Tonsillar ectopy and Chiari malformations: monozygotic triplets. J Neurosurg 82:497–500PubMed

20.

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

21.

Chiari H (1896) Über Veränderungen des Kleinhirns, des Pons und der Medulla oblongata infolge von kongenitaler Hydrocephalie des Grosshirns. Denkschr Akad Wiss Wien 63:71–116

22.

Chiari H (1987) Concerning alterations in the cerebellum resulting from cerebral hydrocephalus (translated by A Radkowski). Pediatr Neurosci 13:3–8PubMed

23.

Chorobski J; Stephen L (1948) On the syndrome of Arnold-Chiari. Report of a case. J Neurosurg 5:495–500

24.

Chumas PP, Amstrong DC, Drake JM, Kulkarni AV, Hoffman HJ, Humphreys RP, Rutka JT, Hendrick EB (1993) Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population. J Neurosurg 78:568–573PubMed

25.

Dagniew E, Van Loveren HR, Tew JM (2002) Acute foramen magnum syndrome caused by an acquired Chiari malformation after lumbar drainage of cerebrospinal fluid: report of three cases. Neurosurgery 51:823–829PubMed

26.

Driesen W, Schmidt H (1971) Malformations in structures of the posterior fossa. In: Krayenbuhl H, Maspes PE, Sweet WH (eds) Progress in neurological surgery, vol 4. Karger, Basel, pp 102–132

27.

Dufour H, Levrier O, Bruder N, Messana T, Grisoli F (2001) Resection of a giant intracranial dural arteriovenous fistula with the use of low-flow deep hypothermic cardiopulmonary bypass after partial embolization: technical case report. Neurosurgery 48:1381–1385PubMed

28.

Ebenius B (1934) The roentgen appearance in four cases of basilar impression. Acta Radiol (Stockolm) 15:652–656

29.

Elia M, Biodi R, Sofia V, Musumeci SA, Ferri R, Capovilla G, Curatolo P (1999) Seizures in Chiari I malformation: a clinical and electroencephalographic study. J Child Neurol 14:446–450PubMed

30.

Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiological reappraisal. Radiology 183:347–353PubMed

31.

Epstein BS (1948) Pantopaque myelography in diagnosis of Arnold-Chiari malformation without concomitant skeletal or central nervous system defects. Am J Roentgenol 59:359–364

32.

Fischbein NJ, Dillon WP, Cobbs C et al (1999) The “presyrinx” state: a reversible myelopathic condition that may precede syringomyelia. Am J Neuroradiol 20:7–20PubMed

33.

Gambardella G, Caruso G, Caffo M, Germanò A, La Rosa G, Tomasello F (1998) Transverse microincisions of the outer layer of the dura mater combined with foramen magnum decompression as a treatment for syringomyelia with Chiari malformation. Acta Neurochir (Wien) 140:134–139

34.

Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia. J Neurol Neurosurg Psychiatry 28:247–259

35.

Gardner WJ, Goodhall RJ (1950) The surgical treatment of Arnold-Chiari malformation in adults. An explanation of its mechanism and importance of encephalography in diagnosis. J Neurosurg 7:199–206

36.

Girard N, Lasjaunias P, Taylor W (1994) Reversible tonsillar prolapse in vein of Galen aneurysmal malformations: report of eight cases and pathophysiological hypothesis. Childs Nerv Syst 10:141–147PubMed

37.

Grosso S, Scattolini R, Paolo G, Di Bartolo RM, Morgese G, Balestri P (2001) Association of Chiari I malformation, mental retardation, speech delay and epilepsy: a specific disorder? Neurosurgery 49:1099–1104PubMed

38.

Gustafson WA, Oldberg E (1940) Neurologic significance of platybasia. Arch Neurol Psychiatry 44:1184–1198

39.

Hoffman HJ, Neill J, Crone KR, Hendrick EB, Humphreys RP (1987) Hydrosyringomyelia and its management in childhood. Neurosurgery 21:347–351PubMed

40.

Homén EA (1901) Zur Kenntnis der rachitischen Deformationen der Schadelbasis und der Basden Scadel hyperostosen. Dtsch Z Nervenheilkd 20:3–15

41.

Iskandar BJ (1999) Chiari malformation and syringomyelia. In: Albright AL, Pollack IF, Adelson PD (eds) Principles and practice of pediatric neurosurgery. Thieme, New York, pp 165–187

42.

Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ (1998) The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 89:212–216PubMed

43.

Itoh Y, Kuwahara N, Sasajima T, Mizoi K, Hatazawa J (2002) Spinal cord edema preceding syringomyelia associated with Chiari I malformation—case report. Neurol Med Chir (Tokyo) 42:410–413

44.

Johnston I, Jacobson E, Besser M (1998) The acquired Chiari malformation and syringomyelia following spinal CSF drainage: A study of incidence and management. Acta Neurochir (Wien) 140:417–428

45.

Levy EI, Heiss JD, Kent MS et al (2000) Spinal cord swelling preceding syrinx development. Case report. J Neurosurg 92 [Suppl 1]:93–97

46.

Lichtor T, Foroohar M, Kulkarni K, Loth F, Alperin NJ (2001) Application of noninvasive MRI phase-contrast flow studies in patients with Chiari I malformations [abstract]. Neurosurgery 49:523

47.

List CF (1941) Neurologic syndromes accompanying developmental anomalies of occipital bone, atlas and axis. Arch Neurol 45:577–616

48.

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

49.

McConnell AA, Parker HL (1938) A deformity of the hind-brain associated with internal hydrocephalus. Its relation to the Arnold-Chiari malformation. Brain 61:415–429

50.

Menezes AH (1994) Comment. Neurosurgery 34:434

51.

Menezes AH (1999) Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg 23:260–269

52.

Menezes AH, Smoker WRK, Dyste GN (1990) Syringomyelia, Chiari malformations and hydromyelia. In: Youmans J (ed) Neurological surgery, 3rd edn. Saunders, Philadelphia

53.

Milhorat TH, Kotzen RM, Anzil AP (1994) Stenosis of the central canal of spinal cord in man: incidence and pathological findings in 232 autopsy cases. J Neurosurg 80:716–722PubMed

54.

Milhorat TH, Capocelli AL, Anzil AP, Kotzen RM, Milhorat RH (1995) Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg 82:802–812PubMed

55.

Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer M (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017PubMed

56.

Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47PubMed

57.

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

58.

Oldfield EH (2001) Syringomyelia. Editorial. J Neurosurg 95 [Suppl 1]:153–155

59.

Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. J Neurosurg 80:3–15PubMed

60.

Ollivier d’Ángers CP (1827) Syringomyélie ou cavite centrale dans la moelle. In: Traité de la moelle epinaire et de ses maladies. Crevot, Paris, pp 178–183

61.

Payner TD, Prenger E, Berger TS, Crone KR (1994) Acquired Chiari malformations: incidence, diagnosis and management. Neurosurgery 34:429–434

62.

Peach B (1965) The Arnold-Chiari malformation. Arch Neurol 12:613

63.

Penfield W, Coburn DF (1938) Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiatry 40:328–336

64.

Piper JG, Menezes AH (1996) Chiari malformation in the adult. In: Menezes AH, Sonntag VKH (eds) Principles of spinal surgery, vol 1. McGraw-Hill, New York, pp 379–394

65.

Raimondi AJ (1972) Pediatric neuroradiology. Saunders, Philadelphia, pp 294–297

66.

Raimondi AJ, Cerullo LJ (1980) Pediatric cerebral angiography. In: Nadjmi M (ed) A descriptive atlas. Thieme, Stuttgart, pp 140–141

67.

Rekate HL (2002) Cerebral oedema following EVD insertion for delayed hydrocephalus after foramen magnum decompression in Chiari I malformation. Commentary. Childs Nerv Syst 18:482CrossRefPubMed

68.

Russell DS (1949) Observations on the pathology of hydrocephalus. HM Stationery Office, London

69.

Russell DS, Donald C (1935) The mechanism of internal hydrocephalus in spina bifida. Brain 58:203–215

70.

Sahuquillo J, Rubio E, Poca MA, Rovira A, Rodriguez-Baeza A, Cervera C (1994) Posterior fossa reconstruction: a surgical technique for the treatment of Chiari I malformation and Chiari I/syringomyelia complex. Preliminary results and magnetic resonance imaging quantitative assessment of hindbrain migration. Neurosurgery 35:874–885PubMed

71.

Sathi S, Stieg PE (1993) “Acquired” Chiari I malformation after multiple lumbar punctures: case report. Neurosurgery 32:306–309PubMed

72.

Schady W, Metcalfe RA, Butler P (1987) The incidence of craniocervical bony anomalies in the adult Chiari malformation. J Neurol Sci 82:193–203CrossRefPubMed

73.

Schwalbe E, Gredig M (1907) Über Entwicklungsstörungen des Kleinhirns, Hirnstamms und Halsmarks bei spina bifida (Arnold’sche und Chiari’sche Missbildung). Beitr Pathol Anat 40:132–194

74.

Stovner LJ, Bergan U, Nilsen G, Sjaastad O (1993) Posterior fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentations. Neuroradiology 35:113–118

75.

Takamura Y, Kawasaki T, Takahashi A (2001) A craniocervical injury-induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. Case report. J Neurosurg 95 [Suppl 1]:122–127

76.

Tominaga T, Koshu K, Ogawa A et al (1991) Transoral decompression evaluated by cine-mode magnetic resonance imaging: a case of basilar impression accompanied by Chiari malformation. Neurosurgery 28:883–885PubMed

77.

Tubbs RS, Elton S, Grabb P, Dockery SE, Bartolucci AA, Oakes WJ (2001) Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 48:1050–1055PubMed

78.

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

79.

Walsh MN, Camp JD, Craig WM (1941) Basilar invagination of the skull (so-called platybasia): report of a case with operation. Mayo Clin Proc 16:449–452

80.

Weprin BE, Oakes WJ (2001) The Chiari malformations and associated syringohydromyelia. In: McLone DG (ed) Pediatric neurosurgery: surgery of the developing nervous system, 4th edn. Saunders, Philadelphia, pp 214–235

81.

Weprin BE, Iskandar BJ, Hedlung GL, Grabb PA, Oakes WJ (1998) The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 89:212–216PubMed

82.

Williams B (1980) On the pathogenesis of syringomyelia. A review. J R Soc Med 73:798–806PubMed

Title: History, anatomic forms, and pathogenesis of Chiari I malformations
Author: Edgardo Schijman
Publication date: 01-05-2004
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 5/2004
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-003-0878-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

History, anatomic forms, and pathogenesis of Chiari I malformations

Abstract

Introduction

Object

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Object

Please log in to get access to this content

Other articles of this Issue 5/2004

Cervical meningocele in association with spinal abnormalities

“Hydrocephalus-parkinsonism complex”: progressive hydrocephalus as a factor affecting extrapyramidal tract disorder—an experimental study

International survey on the management of Chiari I malformation and syringomyelia

Third International Course of the Chapter of Pediatric Neurosurgery of the Federación Latinoamericana de Neurocirugia (FLANC), Montevideo, Uruguay, 17–19 November 2003

Primary central nervous system sarcomas in children: clinical, radiological, and pathological features

Benign focal epilepsy with onset in infancy in a patient with 18q-syndrome