Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000050.xml
Download PDF
Seminars in Neurosurgery 2002; 13(2): 119-130
DOI: 10.1055/s-2002-35809
DOI: 10.1055/s-2002-35809
Chiari I Malformations and Syringomyelia
Further Information
Publication History
Publication Date:
28 November 2002 (online)
ABSTRACT
The pathogenesis of the Chiari I malformation (CMI) as well as its associated syringomyelic cavity remains relatively vague despite significant recent advances. Multiple theories have been proposed to explain both the origin of the CMI and the development of a syrinx. This article fleshes out the major theories for both of these conditions. The indeterminate quality of this condition can also be seen in its presentation. There are myriad documented presenting signs and symptoms in patients with CMI. The major clinical syndromes and most common signs and symptoms are reviewed. Most importantly for both patient and physician, this lack of consensus extends to treatment. Because of historical unsatisfying surgical results, a multitude of treatment options have been and are currently being proposed for the treatment of both conditions. The major schools of thought with regard to treatment are also reviewed.
KEYWORDS
Chiari malformation - syringomyelia - syrinx
REFERENCES
- 1 Chiari H. Ueber veranderungen des kleinhirns infolge von hydrocephalie des grosshirns. Dtsch Med Wochenschr . 1891; 17 1172-1175
- 2 Chiari H. Ueber veranderungen des kleinhirns, des pons und der medulla oblongata infolge von congenitaler hydrocephalie des gosshirns. Denkschriften der Kais Akad Wiss Math-naturw . 1896; 63 71-116
- 3 Milhorat T H, Chou M W, Trinidad E M. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery . 1999; 44 1005-1017
- 4 Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and response to suboccipital decompression with Chiari I malformation. Neurosurgery . 1995; 37 214-218
- 5 Marin-Padilla M. Notochordal-basichondrocranium relationships: abnormalities in experimental axial skeletal (dysraphic) disorders. J Embryol Exp Morphol . 1979; 53 15-38
- 6 Marin-Padilla M, Marin-Padilla T M. Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci . 1981; 50 29-55
- 7 Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Unoue Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg . 1997; 86 40-47
- 8 Nylan H, Krogness K G. Size of posterior fossa in Chiari type I malformation in adults. Acta Neurochir (Wein) . 1978; 40 233-242
- 9 Schady W, Metcalfe R A, Butler P. The incidence of craniocervical bony abnormalities in the adult Chiari malformation. J Neurol Sci . 1987; 82 193-203
- 10 Stovner L J, Berger U, Nilsen G, Sjarstad O. Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology . 1993; 35 113-118
- 11 Vega A, Quitana F, Berciano J. Basichondrocranium anomalies in adult Chiari type I malformations: a morphometric study. J Neurol Sci . 1990; 99 137-145
- 12 Batzdorf U. Chiari I malformation with syringomyelia: evaluation of surgical therapy by magnetic resonance imaging. J Neurosurg . 1988; 68 726-730
- 13 Tator C H, Meguro K, Rowed D W. Favorable results with syringosubarachnoid shunts for treatment of syringomyelia. J Neurosurg . 1982; 56 517-523
- 14 Nohria V, Oakes W J. Chiari I malformation: a review of 43 patients. Pediatr Neurosurg . 1990; 16 222-227
- 15 Park J K, Gleason P L, Madsen J R, Goumnerova L C, Scott R M. Presentation and management of Chiari I malformation in children. Pediatr Neurosurg . 1997; 26 190-196
- 16 Brewis M, Poskanzer D C, Rolland C. Neurological disease in an English city. Acta Neurol Scand Suppl . 1966; 24 1-89
- 17 Milhorat T H, Capocelli A L, Anzil A P, Kotzen R M, Milhorat R H. Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg . 1995; 82 802-812
- 18 Gardner W J, Abdullah A F, McCormack L J. Varying expressions of embryonal atresia of the fourth ventricle in adults: Arnold-Chiari malformation, Dandy-Walker syndrome, arachnoid cyst os cerebellum, and syringomyelia. J Neurosurg . 1957; 14 591-607
- 19 Gardner W J. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry . 1965; 28 247-259
- 20 Cleland J. Contribution to the study of spina bifida, encephalocele, and anencephalus. J Anat Physiol . 1883; 17 257-292
- 21 Peach B. Arnold-Chiari malformation: morphogenesis. Arch Neurol . 1965; 12 527-535
- 22 O'Rahilly R, Muller F, Meyer D B. The human vertebral column at the end of the embryonic period proper. J Anat . 1983; 136 181-195
- 23 Williams B. The distending force in the production of ``communicating'' syringomyelia. Lancet . 1969; 2 189-193
- 24 du Boulay G, O'Connell J, Currie J C. Further investigations on the pulsatile movements in the cerebrospinal fluid pathway. Acta Radiol (Diagn) . 1972; 13 496-523
- 25 du Boulay G, Shah S, Currie J C. The mechanism of hydromyelia in Chiari type I malformations. Br J Radiol . 1974; 47 579-587
- 26 Williams B. On the pathogenesis of syringomyelia: a review. J R Soc Med . 1980; 73 798-806
- 27 Williams B. Pathogenesis of syringomyelia. In: Batzdorf U, ed. Syringomyelia: Current Concepts in Diagnosis and Treatment
Baltimore: Williams & Wilkins 1991: 59-60
MissingFormLabel
- 28 Ball M J, Dayan A D. Pathogenesis of syringomyelia. Lancet . 1972; 1 799-801
- 29 Aboulker J. Syringomyelia and intra-rachidian fluids [in French]. Neurochirurgie . 1979; 25 1-144
- 30 Rennels M L, Blaumanis O R, Grady P A. Rapid solute transport throughout the brain via paravascular fluid pathways. Adv Neurol . 1990; 52 431-439
- 31 Rennels M L, Gregory T F, Blaumanis O R. Evidence for a ``perivascular'' fluid circulation in the mammalian central nervous system, provided by the rapid distribution of tracer protein throughout the brain from the subarachnoid space. Brain Res . 1985; 326 47-63
- 32 Stoodley M A, Brown S A, Brown C J, Jones N R. Arterial pulsation-dependent perivascular cerebrospinal fluid flow into the central canal in the sheep spinal cord. J Neurosurg . 1997; 86 686-693
- 33 Oldfield E H, Muraszko K, Shawker T H, Patronas N J. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. J Neurosurg . 1994; 80 3-15
- 34 Heiss J D, Patronas N, DeVroom H L. Elucidating the pathophysiology of syringomyelia. J Neurosurg . 1999; 91 553-562
- 35 Oldfield E H. Syringomyelia. J Neurosurg (Spine) . 2001; 95 153-155
- 36 Fischbein N J, Dillon W P, Cobbs C, Weinstein. The ``presyrinx'' state: a reversible myelopathic condition that may precede syringomyelia. AJNR . 1999; 20 7-20
- 37 Levy E I, Heiss J D, Kent M S, Riedel C J, Oldfield E H. Spinal cord swelling preceding syrinx development. J Neurosurg (Spine) . 2000; 92 93-97
- 38 Milhorat T H. Is reversible enlargement of the spinal cord a presyrinx state (commentary)?. AJNR . 1999; 20 21-22
- 39 Milhorat T H, Nobandegani F, Miller J I, Rao C. Noncommunicating syringomyelia following occlusion of central canal in rats. J Neurosurg . 1993; 78 273-279
- 40 Paul K S, Lye R H, Strang F A, Dutton J. Arnold-Chiari malformation: review of 71 cases. J Neurosurg . 1983; 58 183-187
- 41 Bindal A K, Dunsker S B, Tew J M. Chiari I malformation: classification and management. Neurosurgery . 1995; 37 1069-1074
- 42 Dyste G N, Menezes A H. Presentation and management of pediatric Chiari malformations without myelodysplasia. Neurosurgery . 1988; 23 589-597
- 43 Wisoff J H, Epstein F. Management of hydromyelia. Neurosurgery . 1989; 25 562-571
- 44 Levy W J, Mason L, Hahn J F. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery . 1983; 12 377-389
- 45 Dyste G N, Menezes A H, VanGilder J C. Symptomatic Chiari malformations: an analysis of presentation, management, and long-term outcome. J Neurosurg . 1989; 71 159-168
- 46 Meadows J, Guarnieri M, Miller K, Haroun R, Kraut M, Carson B S. Type I Chiari malformation: a review of the literature. Neurosurg Quart . 2001; 11 220-229
- 47 Park J K, Gleason P L, Madsen J R, Goumnerova L C, Scott R M. Presentation and management of Chiari I malformation in children. Pediatr Neurosurg . 1997; 26 190-196
- 48 McLone D G. Pediatric Neurosurgery. 4th ed. Philadelphia: WB Saunders 2001: 221
MissingFormLabel
- 49 Aboulezz A O, Sartor K, Geyer C A, Gado M H. Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comp Assist Tomog . 1985; 9 1033-1036
- 50 Barkovich A J, Wippold F J, Sherman J L, Citrin C M. Significance of cerebellar tonsillar position on MR. AJNR . 1986; 7 795-799
- 51 Mikulis D J, Diaz O, Egglin T K, Sanchez R. Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology . 1992; 183 725-728
- 52 Elster A D, Chen M YM. Chiari I malformations: clinical and radiographic reappraisal. Neuroradiology . 1992; 183 347-353
- 53 Amer T A, el-Shmam O M. Chiari malformation type I: a new MRI classification. Magn Reson Imaging . 1997; 15 397-403
- 54 Levy L M, DiChiro G. MR phase imaging and cerebrospinal fluid flow in the head and spine. Neuroradiology . 1990; 32 399-406
- 55 Armonda R A, Citrin C M, Foley K T, Ellenbogen R G. Quantitative cine-mode magnetic resonance imaging of Chiari I malformations: an analysis of cerebrospinal fluid dynamics. Neurosurgery . 1994; 35 214-223
- 56 Grabb P A, Mapstone T B, Oakes W J. Ventral brainstem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery . 1999; 44 520-527
- 57 Tindall G T. The Practice of Neurosurgery.
1st ed. Baltimore: Williams & Wilkins 1996: 2746
MissingFormLabel
- 58 McRae D L, Standen J. Roentgenologic findings in syringomyelia and hydromyelia. Am J Roentgenol Radium Ther Nucl Med . 1966; 98 695-703
- 59 Isu T, Chono Y, Iwasaki Y. Scoliosis associated with syringomyelia presenting in children. Childs Nerv Syst . 1992; 8 97-100
- 60 Haines S J, Berger M. Current treatment of Chiari malformations I and II: a survey of the pediatric section of the American Association of Neurological Surgeons. Neurosurgery . 1991; 28 353-357
- 61 Milhorat T H, Johnson W D, Miller J I, Bergland R M, Hollenberg-Sher J. Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. Neurosurgery . 1992; 31 231-244
- 62 Williams B, Fahy G. A critical apparaisal of ``terminal ventriculostomy'' for the treatment of syringomyelia. J Neurosurg . 1983; 58 188-197
- 63 Logue V, Edwards M R. Syringomyelia and its surgical treatment-an analysis of 75 patients. J Neurol Neurosurg Psychiatry . 1981; 44 273-284
- 64 Blagodatsky M D, Larionov S N, Alexandrov Y A, Velm A I. Surgical treatment of Chiari I malformation with or without syringomyelia. Acta Neurochir (Wein) . 1999; 141 963-968
- 65 Sakamoto H, Nishikawa M, Hakuba A. Expansive suboccipital cranioplasty for the treatment of syringomyelia associated with Chiari malformation. Acta Neurochir (Wein) . 1999; 141 949-961
- 66 Klekamp J, Batzdorf U, Samii M, Bothe H W. Treatment of syringomyelia associated with arachnoid scarring caused by arachnoiditis or trauma. J Neurosurg . 1997; 86 233-240
- 67 Matsumoto T, Symon L. Surgical management of syringomyelia-current results. Surg Neurol . 1989; 32 258-265
- 68 Garcia-Uria J, Leunda G, Carrillo R, Bravo G. Syringomyelia: long-term results after posterior fossa decompression. J Neurosurg . 1981; 54 380-383
- 69 Isu T, Sasaki H, Takamura H, Kobayashi N. Foramen magnum decompression with removal of the outer layer of the dura as treatment for syringomyelia occurring with Chiari I malformation. Neurosurgery . 1993; 33 845-849
- 70 Munshi I, Frim D, Stine-Reyes R, Weir B KA, Hekmatpanah J, Brown F. Effects of posterior fossa decompression with and without duraplasty on Chiari malformation-associated hydromyelia. Neurosurgery . 2000; 46 1384-1389
- 71 Pillay P K, Awad I A, Little J R, Hahn J F. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery . 1991; 28 639-645
- 72 Rhoton A L. Microsurgery of Arnold-Chiari malformation in adults with and without hydromyelia. J Neurosurg . 1976; 45 473-483
- 73 Vaquero J, Martinez R, Arias A. Syringomyelia-Chiari complex: magnetic resonance imaging and clinical evaluation of surgical treatment. J Neurosurg . 1990; 73 64-68
- 74 Depreitere B, Van Calenbergh F, Van Loon J, Goffin J, Plets C. Posterior fossa decompression in syringomyelia associated with a Chiari malformation: a retrospective analysis of 22 patients. Clin Neurol Neurosurg . 2000; 102 91-96
- 75 Fischer E G. Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils. Childs Nerv Syst . 1995; 11 625-629
- 76 Raftopoulos C, Sanchez A, Matos C, Baleriaux D, Bank W O, Brotchi J. Hydrosyringomyelia-Chiari I complex. Prospective evaluation of a modified foramen magnum decompression procedure: preliminary results. Surg Neurol . 1993; 39 163-169
- 77 Guyotat J, Bret P, Jouanneau E, Ricci A C, Lapras C. Syringomyelia associated with type I Chiari malformation a 21 year retrospective study on 75 cases treated by foramen magnum decompression with a special emphasis on the value of tonsils resection. Acta Neurochir (Wein) . 1998; 140 745-754
- 78 Fujii K, Natori Y, Nakagaki H, Fukui M. Management of syringomyelia associated with Chiari malformation: comparative study of syrinx size and symptoms by magnetic resonance imaging. Surg Neurol . 1991; 36 281-285
- 79 Isu T, Iwasaki Y, Akino M, Abe H. Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (type I). Acta Neurochir (Wein) . 1990; 107 152-160
- 80 Hida K, Iwasaki Y, Koyanagi I, Sawamura Y, Abe H. Surgical indication and results of foramen magnum decompression versus syringosubarachnoid shunting for syringomyelia associated with Chiari I malformation. Neurosurgery . 1995; 37 673-678
- 81 Iwasaki Y, Hida K, Koyanagi I, Abe H. Reevaluation of syringosubarachnoid shunt for syringomyelia with Chiari malformation. Neurosurgery . 2000; 46 407-412