Abstract
Objective
Multiple surgical strategies exist for the management of the symptomatic Chiari II malformation. To date, no comprehensive analysis of this medical literature in an attempt to seek out standards or guidelines has been performed, thus serving as the impetus for this present review.
Methods
A computerized search of the database of the National Library of Medicine was performed on the English-language medical literature between 1966 and 2003. Terms searched for included hindbrain hernia, Chiari II, Arnold-Chiari, surgery, decompression, syringomyelia, and CSF shunts. All literature found was reviewed with associated references from these sources. Each piece of literature reviewed was scrutinized and guidelines developed utilizing the methodology used by the Guidelines for the Management of Acute Cervical Spine and Spinal Cord Injuries.
Results
All data reviewed were deemed to be Class III in nature. No standards or guidelines could be established from the available literature. There is significant debate and variable results in the current neurosurgical literature regarding the evaluation of shunt function vs. Chiari II decompression in patients with symptomatic hindbrain herniation.
Conclusions
Multi-institutional prospective randomized trials are needed before any conclusions can be drawn regarding symptomatic Chiari II and its treatment paradigm. Until this time, clinical practice will be based on individual surgeons’ experience, training, and personal beliefs.
Similar content being viewed by others
References
Aaronson DD, Kahn RH, Canady A, Bollinger RO, Towbin R (1991) Instability of the cervical spine after decompression in patients who have Arnold-Chiari malformation. J Bone Joint Surg Am 73:898–906
Ball MJ, Dayan AD (1972) Pathogenesis of syringomyelia. Lancet 2:799–801
Barnet AB, Weiss IP, Shaer C (1993) Evoked potentials in infant brainstem syndrome associated with Arnold-Chiari malformation. Dev Med Child Neurol 35:42–48
Bell WO, Charney EB, Bruce DA, Sutton LN, Schut L (1987) Symptomatic Arnold Chiari malformation: review of experiences with 22 cases. J Neurosurg 66:812–816
Caldarelli M, Di Rocco C, Colosimo C Jr, Fariello G, Di Gennaro M (1995) Surgical treatment of late neurological deterioration in children with myelodysplasia. Acta Neurochir 137:199–206
Cameron AH (1957) The Arnold–Chiari and other neuroanatomical malformations associated with spina bifida. J Path Bact 73:195–211
Charney EB, Rorke LB, Sutton LN, Schut L (1987) Management of Chiari II complications in infants with myelomeningocele. J Pediatr 111:364–371
Cochrane DD, Adderly R, White CP, Norman M, Steinbok P (1991) Apnea in patients with myelomeningocele. Pediatr Neurosurg 16:232–239
Dyste GN, Menezes AH, Van Gilder JC (1989) Symptomatic Chiari malformations. J Neurosurg 71:159–168
Haines SJ, Berger MD (1991) Current treatment of Chiari malformations type I and II: a survey of the Pediatric Section of the American Association of Neurological Surgeons. Neurosurgery 28:353–357
Hesz N, Wolraich M (1985) Vocal-cord paralysis and brainstem dysfunction in children with spina bifida. Dev Med Child Neurol 27:528–531
Hoffman HJ, Hendrick EB, Humphreys RP (1975) Manifestations and management of Arnold Chiari malformation in patients with myelomeningocele. Childs Brain 1:255–259
Hoffman HJ, Neil J, Crone KR, Hendrick EB, Humphreys RP (1987) Hydrosyringomyelia and its management in childhood. Neurosurgery 21:347–351
Holinger PC, Holinger LD, Reichart TJ, Holinger PH (1978) Respiratory obstruction and apnea in infants with bilateral abductor cord paralysis, meningocele, hydrocephalus, and Arnold Chiari malformation. J Pediatr 92:368–373
Holliday PO, Pillsbury D, Kelly DL, Dillard R (1985) Brain stem auditory evoked potentials in Arnold-Chiari malformation: possible prognostic value and changes with surgical decompression. Neurosurgery 16:48–53
Holschneider AM, Bliesener JA, Abel M (1990) The brain stem dysfunction in Arnold-Chiari II syndrome. Z Kinderchir 45:67–71
Iskandar BJ, McLaughlin C, Mapstone TB, Grabb PA, Oakes WJ (1998) Pitfalls in the diagnosis of ventricular shunt dysfunction: radiology reports and ventricular size. Pediatrics 101:1031–1036
James HE, Brant A (2002) Treatment of the Chiari malformation with bone decompression without durotomy in children and young adults. Childs Nerv Syst 18:202–206
LaMarca F, Herman M, Grant JA, McLone DG (1997) Presentation and management of hydromyelia in children with Chiari type-II malformation. Pediatr Neurosurg 26:57–67
Logue J, Edwards MR (1981) Syringomyelia and its surgical treatment. J Neurol Neurosurg Psychiatry 44:273–284
McLone DG (1992) Continuing concepts in the management of spina bifida. Pediatr Neurosci 18:254–256
McLone DG, Nakahara S, Knepper PA (1991) Chiari II malformation: pathogenesis and dynamics. Concepts Pediatr Neurosurg 11:1–17
Milhorat TH, Johnson WD, Miller JL, Bergland RM, Hollenberg-Sher J (1992) Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. Neurosurgery 31:231–245
Mori K, Uchida Y, Nishimura T, Eghwrudjakpor P (1988) Brainstem auditory evoked potentials in Chiari-II malformation. Childs Nerv Syst 4:154–157
Narayan P, Mapstone TB, Tubbs RS, Grabb PA, Frye T (2001) Clinical significance of cervicomedullary deformity in Chiari II malformation. Pediatr Neurosurg 35:140–144
Oakes WJ, Worley G, Spock A (1988) Surgical intervention in twenty-nine patients with symptomatic type II Chiari malformations: clinical presentation and outcome. Concepts Pediatr Neurosurg 8:76–85
Oakes WJ, Gaskill SJ (1992) Symptomatic Chiari malformation and syringohydromyelia in childhood. In: Park TS (ed) Spinal dysraphism. Blackwell, Boston, pp 104–105
Park TS, Hoffman HJ, Hendrick EB, Humphreys RP (1983) Experience with surgical decompression of the Arnold-Chiari malformation with myelomeningocele. Neurosurgery 13:147–152
Park TS, Cail WS, Maggio WM, Mitchell DC (1985) Progressive spasticity and scoliosis in children with myelomeningocele: radiologic investigation and surgical treatment. J Neurosurg 62:367–375
Pollack IF, Pang D, Kocoshis S, Putnam P (1992) Neurogenic dysphagia resulting from Chiari malformations. Neurosurgery 30:709–719
Pollack IF, Pang D, Albright AL, Krieger D (1992) Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77:881–888
Pollack IF, Kinnunen D, Albright AL (1996) The effect of early craniocervical decompression on functional outcome in neonates and young infants with myelodysplasia and symptomatic Chiari II malformations: results from a prospective series. Neurosurgery 38:703–710
Salomao JF, Bellas AR, Leibinger RD, Barbosa AP, Brandao MA (1998) Symptomatic Chiari type II malformation. Arq Neuropsiquiatr 56:98–106
Teo C, Parker EC, Aureli S, Boop FA (1997) The Chiari II malformation: a surgical series. Pediatr Neurosurg 27:223–229
Tomita T, McLone DG (1983) Acute respiratory arrest. A complication of malformation of the shunt in children with myelomeningocele and Arnold-Chiari malformation. Am J Dis Child 137:142–144
Vandertop WP, Asai A, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, Becker LE (1992) Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg 77:541–544
Venes JL (1974) Multiple cranial nerve palsies in an infant with the Arnold-Chiari malformation. Dev Med Child Neurol 16:817–819
Venes JL, Black KL, Latack JT (1986) Preoperative evaluation and surgical management of the Arnold-Chiari malformation. J Neurosurg 64:363–370
Vernet O, Farmer JP, Montes JL (1996) Comparison of syringopleural and syringosubarachnoid shunting in the treatment of syringomyelia in children. J Neurosurg 84:624–628
Williams B (1981) Simultaneous cerebral and spinal fluid pressure recordings. I. Technique, physiology and normal results. Acta Neurochir (Wien) 58:167–185
Williams B (1981) Simultaneous cerebral and spinal fluid pressure recordings. II. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir (Wien) 59:123–142
Yamada H, Tanaka Y, Nakamura S (1985) Laryngeal stridor associated with the Chiari II malformation. Childs Nerv Syst 1:312–318
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Tubbs, R.S., Oakes, W.J. Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20, 375–381 (2004). https://doi.org/10.1007/s00381-004-0969-4
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-004-0969-4