Chiari Malformations: Historical Background, Anatomical Forms and Treatment Approaches

Zekeriya Bulut

doi:10.7197/cmj.1411030

EN TR

Chiari Malformations: Historical Background, Anatomical Forms and Treatment Approaches

Abstract

Chiari malformations (CM) refer to a series of anomalies characterized by the descent of cerebellar tonsils into the cervical spinal canal. These malformations can be associated with abnormalities such as syringomyelia, hydrocephalus, spina bifida, and scoliosis. Additionally, cranio-cervical junction anomalies, endocrinopathies, craniosynostosis, and syndromic disorders are also linked to CM. The treatment of CM is surgical, and there is no known medical therapy. Patients diagnosed with CM are typically advised to undergo surgical treatment or follow-up. Although surgical intervention is supported in the literature, debates exist regarding which procedure is most suitable and when surgery should be performed. In this article, we will examine the historical background of CM, its anatomical forms, pathophysiology, clinical presentation, relationship with other diseases, and diagnostic procedures in the light of the literature.

Keywords

Thanks

Teşekkürler

References

Massimi L, Peppucci E, Peraio S, Di Rocco C. History of Chiari type I malformation. Neurol Sci. 2011;32 Suppl 3:S263-S265.
Güzey FK, Aycan A. Chiari Malformasyonları ve Siringomiyeli: Tarihçe ve Sınıflama. Türk Nöroşirurji Dergisi. 2015;25(2):227-233.
Brockmeyer DL. The complex Chiari: Issues and management strategies. Neurol Sci. 2011;32 Suppl 3:S345-S347.
Mortazavi MM, Tubbs RS, Hankinson TC, Pugh JA, Cohen-Gadol AA, Oakes WJ. The first posterior fossa decompression for Chiari malformation: the contributions of Cornelis Joachimus van Houweninge Graftdijk and a review of the infancy of “Chiari decompression”. Childs Nerv Syst. 2011;27(11):1851-1856.
List CF. Neurologic syndromes accompanying developmental anomalies of occipital bone, atlas, and axis. Arch Neurol. 1941;45:577–616.
Adams RD, Schatzki R, Scoville WB. The Arnold–Chiari malformation. Diagnosis, demonstration by intraspinal lipiodal, and successful surgical treatment. N Engl J Med. 1941;225:125–31.
Penfield W, Coburn DF. Arnold-Chiari Malformation And its operative treatment. Arch Neurol Psychiat. 1938;40:328–36.
Elster AD, Chen MY. Chiari 1 malformations: Clinical and radiological reappraisal. Radiology. 1992;183:347–53.

Daniel PM, Strich SJ. Some observations on the congenital deformity of the central nervous system known as the Arnold- Chiari malformation. J Neuropathol Exp Neurol. 1958;17:255–66.
Gardner WJ. Hydrodynamic factors in Dandy-Walker and Arnold Chiari malformations. Childs Brain. 1972;3:200–212.
Gardner WJ, Goodall RJ. The surgical treatment of Arnold-Chiari malformation in adults: an explanation of its mechanism and importance of encephalography in diagnosis. J Neurosurg. 1950;3:199–206.
Peach B. The Arnold-Chiari malformation: its morbid anatomy and histology. [Thesis]. Manchester; 1964.
Marin-Padilla M, Marin-Padilla TM. Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci. 1981;50:29–55.
Osaka K. Myelomeningocele before birth. J Neurosurg. 1978;49:711–24.
Batzdorf U. Chiari 1 malformation of syringomyelia. Evaluation of surgical therapy by magnetic resonance imaging. J Neurosurg. 1988;68:726–30.
Chern JJ, Gordon AJ, Mortazavi MM, Tubbs RS, Oakes WJ. Pediatric Chiari malformation Type 0: a 12-year institutional experience. J Neurosurg Pediatr. 2011;8:1–5.
Milhorat TH. Classification of syringomyelia. Neurosurg Focus. 2000;8(3):E1.
Cahan LD, Bentson JR. Considerations in the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg. 1982;57:24–31.
Castillo M, Dominguez R. Imaging of common congenital anomalies of the brain and spine. Clin Imaging. 1992;16:73–88.
Boor R, Schwarz M, Goebel B, Voth D. Somatosensory evoked potentials in Arnold-Chiari malformation. Brain Dev. 2004;26:99–104.
Oakes WJ, Tubbs RS. Chiari malformations. In: Winn HR, editor. Youmans neurological surgery. 3rd ed. Philadelphia: Elsevier; 2004. p. 3347–61.
Tavallaii A, Keykhosravi E, Rezaee H, Abouei Mehrizi MA, Ghorbanpour A, Shahriari A. Outcomes of dura-splitting technique compared to conventional duraplasty technique in the treatment of adult Chiari I malformation: a systematic review and meta-analysis. Neurosurg Rev. 2021;44(3):1313–29.
McClugage SG, Oakes WJ. The Chiari I malformation: JNSPG 75th Anniversary Invited Review Article. J Neurosurg Pediatr. 2019;24(3):217–26.
Stephany JD, Garavaglia JC, Pearl GS. Sudden death in a 27-year-old man with Chiari I malformation. Am J Forensic Med Pathol. 2008;29:249–50.
Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ. The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg. 1998;89:212–16.
Aguiar PH, Tella OI, Pereira CU, Godinho F, Simm R. Chiari type I presenting as left glossopharyngeal neuralgia with cardiac syncope. Neurosurg Rev. 2002;25:99–102.
Drayer M, Geracht J, Madikians A, Harrison R. Neurogenic stunned myocardium: An unusual postoperative complication. Pediatr Crit Care Med. 2006;7:374–76.
Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005–17.
Kumar R, Kalra SK, Vaid VK, Mahapatra AK. Chiari I malformation: Surgical experience over a decade of management. Br J Neurosurg. 2008;22:409–14.
Chavez A, Roguski M, Killeen A, Heilman C, Hwang S. Comparison of operative and non-operative outcomes based on surgical selection criteria for patients with Chiari I malformations. J Clin Neurosci. 2014;21(12):2201–06.
Langridge B, Phillips E, Choi D. Chiari malformation type 1: a systematic review of natural history and conservative management. World Neurosurg. 2017;104:213–19.
Schmahmann JD. Rediscovery of an early concept. Int Rev Neurobiol. 1997;41:3–27.
Işık N. Chiari Malformasyonları ve Siringomiyeli. Türk Nöroşirurji Dergisi. 2013;23(2):185–94.
Tubbs RS, Muhleman M, Loukas M, Oakes WJ. A new form of herniation: The Chiari V malformation. Childs Nerv Syst. 2012;28:305–07.
Bollo RJ, Riva-Cambrin J, Brockmeyer MM, Brockmeyer DL. Complex Chiari malformations in children: An analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr. 2012;10(2):134–41.
Di Lorenzo N, Cacciola F. Adult syringomyelia: Classification, pathogenesis and therapeutic approaches. J Neurosurg Sci. 2005;49:65–72.
Fernandez AA, Guerrero AI, Martinez MI, Vazquez MEA, Fernandez JB, Octovia EC, Labrado JDC, et al. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis, and treatment). BMC Musculoskelet Disord. 2009;10(Suppl 1):S1.
Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, Rozzelle CJ, Blount JP, Oakes WJ. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I. J Neurosurg Pediatr. 2011;7:248–56.
Deng X, Wu L, Yang C, Tong X, Xu Y. Surgical treatment of Chiari I malformation with ventricular dilation. Neurol Med Chir (Tokyo). 2013;53:847–52.
Joshi VP, Valsangkar A, Nivargi S, Vora N, Dekhne A, Agrawal A. Giant posterior fossa arachnoid cyst causing tonsillar herniation and cervical syringomyelia. J Craniovertebr Junction Spine. 2013;4:43–45.
Saldino RM, Steinbach HL, Epstein CJ. Familial acrocephalosyndactyly (Pfeiffer syndrome). Am J Roentgenol Radium Ther Nucl Med. 1972;116:609–22.
Loukas M, Shayota BJ, Oelhafen K, Miller JH, Chern JJ, Tubbs RS, Oakes WJ. Associated disorders of Chiari Type I malformations: A review. Neurosurg Focus. 2011;31(3):E3.
Dlouhy BJ, Menezes AH. Osteopetrosis with Chiari I malformation: Presentation and surgical management. J Neurosurg Pediatr. 2011;7:369–74.
Kuether TA, Piatt JH. Chiari malformation associated with vitamin D-resistant rickets: case report. Neurosurgery. 1998;42:1168–71.
Gupta A, Vitali AM, Rothstein R, Cochrane DD. Resolution of syringomyelia and Chiari malformation after growth hormone therapy. Childs Nerv Syst. 2008;24:1345–48.
Klekamp J. Chiari I malformation with and without basilar invagination: A comparative study. Neurosurg Focus. 2015;38(4):E12.
Zileli M, Cagli S. Combined anterior and posterior approach for managing basilar invagination associated with type I Chiari malformation. J Spinal Disord Tech. 2002;15:284–89.
Menezes AH, VanGilder JC. Transoral-transpharyngeal approach to the anterior craniocervical junction. Ten-year experience with 72 patients. J Neurosurg. 1988;69:895–903.
Behari S, Kalra SK, Kiran Kumar MV, Salunke P, Jaiswal AK, Jain VK. Chiari I malformation associated with atlantoaxial dislocation: Focussing on the anterior cervico-medullary compression. Acta Neurochir (Wien). 2007;149(1):41–50.
Mangubat EZ, Wilson T, Mitchell BA, Byrne RW. Chiari I malformation associated with atlantooccipital assimilation presenting as orthopnea and cough syncope. J Clin Neurosci. 2014;21:320–23.
Markunas CA, Soldano K, Dunlap K, Cope H, Asiimwe E, Stajich J, Enterline D, Grant G, Fuchs H, Gregory SG, Ashley-Koch AE. Stratified whole genome linkage analysis of Chiari type I malformation implicates known Klippel-Feil syndrome genes as putative disease candidates. PLoS One. 2013;8(4):e61521.
Santos-Garcia D, Cabanillas M, Suarez-Dono I, Monteagudo B, de la Fuente-Fernandez R, Suarez-Amor O. Type 1 neurofibromatosis and Arnold-Chiari malformation. Actas Dermosifiliogr. 2009;100:820–22.
Heiss JD, Suffredini G, Smith R, DeVroom HL, Patronas NJ, Butman JA, Thomas F, Oldfield EH. Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery: Clinical article. J Neurosurg Spine. 2010;13:729–42.
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Natural history of Chiari malformation Type I following decision for conservative treatment. J Neurosurg Pediatr. 2011;8:214–21.
Schijman E, Steinbok P. International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst. 2004;20:341–48.
Oral S, Yilmaz A, Kucuk A, Tumturk A, Menku A. Comparison of Dural Splitting and Duraplasty in Patients with Chiari Type I Malformation: Relationship between Tonsillo-Dural Distance and Syrinx Cavity. Turk Neurosurg. 2019;29(2):229–36.
Goel A. Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine. 2015;22(2):116–27.
Mancarella C, Delfini R, Landi A. Chiari malformations. In: New Trends in Craniovertebral Junction Surgery: Experimental and Clinical Updates for a New State of Art. 2019. p. 89-95.

Details

Primary Language

English

Subjects

Health and Community Services , Medical Education

Journal Section

Review

Authors

Zekeriya Bulut ^*
0000-0002-7663-4523
Türkiye

Publication Date

March 29, 2024

Submission Date

December 27, 2023

Acceptance Date

February 25, 2024

Published in Issue

Year 2024 Volume: 46 Number: 1

DOI

https://doi.org/10.7197/cmj.1411030

IZ

https://izlik.org/JA97ZY46AU

Cite

RIS / Bibtex

AMA

1.Bulut Z. Chiari Malformations: Historical Background, Anatomical Forms and Treatment Approaches. CMJ. 2024;46(1):1-7. doi:10.7197/cmj.1411030

Chiari Malformations: Historical Background, Anatomical Forms and Treatment Approaches

Chiari Malformations: Historical Background, Anatomical Forms and Treatment Approaches

Abstract

Keywords

Chiari Malformasyonları: Tarihsel Arka Plan, Anatomik Formlar ve Tedavi Yaklaşımları

Öz

Anahtar Kelimeler

Thanks

References

Details

Primary Language

Subjects

Journal Section

Authors

Publication Date

Submission Date

Acceptance Date

Published in Issue

DOI

IZ

Cite