Abstract
Purpose
Despite decades of experience and research, the etiology and management of Chiari I malformations (CM-I) continue to raise more questions than answers. Controversy abounds in every aspect of management, including the indications, timing, and type of surgery, as well as clinical and radiographic outcomes. This review aims to outline past experiences, consolidate current evidence, and recommend directions for the future management of the Chiari I malformation.
Methods
A review of recent literature on the management of CM-I in pediatric patients is presented, along with our experience in managing 1073 patients who were diagnosed with CM-I over the past two decades (1998–2018) at Children’s National Medical Center (CNMC) in Washington DC.
Results
The general trend reveals an increase in the diagnosis of CM-I at younger ages with a significant proportion of these being incidental findings (0.5–3.6%) in asymptomatic patients as well as a rise in the number of patients undergoing Chiari posterior fossa decompression surgery (PFD). The type of surgical intervention varies widely. At our institution, 104 (37%) Chiari surgeries were bone-only PFD with/without outer leaf durectomy, whereas 177 (63%) were PFD with duraplasty. We did not find a significant difference in outcomes between the PFD and PFDD groups (p = 0.59). An analysis of failures revealed a significant difference between patients who underwent tonsillar coagulation versus those whose tonsils were not manipulated (p = 0.02).
Conclusion
While the optimal surgical intervention continues to remain elusive, there is a shift away from intradural techniques in favor of a simple, extradural approach (including dural delamination) in pediatric patients due to high rates of clinical and radiographic success, along with a lower complication rate. The efficacy, safety, and necessity of tonsillar manipulation continue to be heavily contested, as evidence increasingly supports the efficacy and safety of less tonsillar manipulation, including our own experience.
Similar content being viewed by others
References
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(6):1033–1036
Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, Sorel M, Wu YW (2009) Chiari type I malformation in a pediatric population. Pediatr Neurol 40(6):449–454
Albert GW, Menezes AH, Hansen DR, Greenlee JD, Weinstein SL (2010) Chiari malformation type I in children younger than age 6 years: presentation and surgical outcome. J Neurosurg Pediatr 5(6):554–561
Alden TD, Ojemann JG, Park TS (2001) Surgical treatment of Chiari I malformation: indications and approaches. Neurosurg Focus 11(1):E2
Alzate JC, Kothbauer KF, Jallo GI, Epstein FJ (2001) Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg Focus 11(1):E3
Arnautovic A, Splavski B, Boop FA, Arnautovic KI (2015) Pediatric and adult chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr 15(2):161–177
Attenello FJ, McGirt MJ, Gathinji M et al (2008) Outcome of chiari-associated syringomyelia after hindbrain decompression in children: analysis of 49 consecutive cases. Neurosurgery. 62(6):1307–1313 discussion 1313
Baisden J (2012) Controversies in chiari I malformations. Surg Neurol Int 3(Suppl 3):S232–S237
Banik R, Lin D, Miller NR (2006) Prevalence of chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 247(1):71–75
Bao C, Yang F, Liu L et al (2013) Surgical treatment of chiari I malformation complicated with syringomyelia. Exp Ther Med 5(1):333–337
Bao CS, Liu L, Wang B, Xia XG, Gu YJ, Li DJ, Zhan SL, Chen GL, Yang FB (2015) Craniocervical decompression with duraplasty and cerebellar tonsillectomy as treatment for chiari malformation-I complicated with syringomyelia. Genet Mol Res 14(1):952–960
Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol 7(5):795–799
Bhangoo R, Sgouros S (2006) Scoliosis in children with chiari I-related syringomyelia. Childs Nerv Syst 22(9):1154–1157
Brockmeyer D, Gollogly S, Smith JT (2003) Scoliosis associated with chiari 1 malformations: the effect of suboccipital decompression on scoliosis curve progression: a preliminary study. Spine (Phila Pa 1976) 28(22):2505–2509
Caldarelli M, Novegno F, Vassimi L, Romani R, Tamburrini G, Di Rocco C (2007) The role of limited posterior fossa craniectomy in the surgical treatment of chiari malformation type I: experience with a pediatric series. J Neurosurg 106(3 Suppl):187–195
Castori M, Morlino S, Ghibellini G, Celletti C, Camerota F, Grammatico P (2015) Connective tissue, ehlers-danlos syndrome(s), and head and cervical pain. Am J Med Genet C: Semin Med Genet 169C(1):84–96
Chai Z, Xue X, Fan H et al (2018) Efficacy of posterior fossa decompression with duraplasty for patients with chiari malformation type I: a systematic review and meta-analysis. World Neurosurg 113:357–365.e1
Chatain GP, Tsering D, Rotter J, Ryan T, Ironside N, Veznedaroglu E, Heiss J, Sandhu F, Myseros J, Magge S, Oluigbo C, Keating R. Differences between symptom presentation for the pediatric and adult Chiari patients: is it a different disease or a reflection of time? International Society for Pediatric Neurosurgery, Denver, CO, USA. October 8-12, 2017. Oral presentation
Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, Cianciulli E, Renier D (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21(10):889–901
Cobourn K, Tsering D, Myseros J, Magge S, Oluigbo C, Keating R Does less tonsillar manipulation lead to fewer Chiari failures: review of changing practice paradigms. [PF-042]. Childs Nerv Syst 34:1997–2122
Di Rocco C, Frassanito P, Massimi L, Peraio S (2011) Hydrocephalus and chiari type I malformation. Childs Nerv Syst 27(10):1653–1664
Di Rocco C, Velardi F (2003) Acquired chiari type I malformation managed by supratentorial cranial enlargement. Childs Nerv Syst 19(12):800–807
Dlouhy BJ, Dawson JD, Menezes AH (2017) Intradural pathology and pathophysiology associated with chiari I malformation in children and adults with and without syringomyelia. J Neurosurg Pediatr 20(6):526–541
Durham SR, Fjeld-Olenec K (2008) Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of chiari malformation type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr 2(1):42–49
Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiologic reappraisal. Radiology. 183(2):347–353
Eule JM, Erickson MA, O’Brien MF, Handler M (2002) Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine (Phila Pa 1976) 27(13):1451–1455
Fagan LH, Ferguson S, Yassari R, Frim DM (2006) The chiari pseudotumor cerebri syndrome: symptom recurrence after decompressive surgery for chiari malformation type I. Pediatr Neurosurg 42(1):14–19
Fearon JA, Dimas V, Ditthakasem K (2016) Lambdoid craniosynostosis: the relationship with chiari deformations and an analysis of surgical outcomes. Plast Reconstr Surg 137(3):946–951
Feinberg M (2015) Bone-only chiari decompression failure rate is no different than that of open duraplasty. Neurosurgery 62(CN_Suppl_1):201
Feldstein NA, Choudhri TF (1999) Management of chiari I malformations with holocord syringohydromyelia. Pediatr Neurosurg 31(3):143–149
Fischer EG (1995) Posterior fossa decompression for chiari I deformity, including resection of the cerebellar tonsils. Childs Nerv Syst 11(11):625–629
Furtado SV, Reddy K, Hegde AS (2009) Posterior fossa morphometry in symptomatic pediatric and adult chiari I malformation. J Clin Neurosci 16(11):1449–1454
Furuya K, Sano K, Segawa H, Ide K, Yoneyama H (1998) Symptomatic tonsillar ectopia. J Neurol Neurosurg Psychiatry 64(2):221–226
Galarza M, Sood S, Ham S (2007) Relevance of surgical strategies for the management of pediatric chiari type I malformation. Childs Nerv Syst 23(6):691–696
Gardner WJ (1977) Syringomyelia. Surg Neurol 7(6):370
Genitori L, Peretta P, Nurisso C, Macinante L, Mussa F (2000) Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases. Childs Nerv Syst 16(10–11):707–718
Gernsback J, Tomita T. 2019 Management of chiari I malformation in children: personal opinions. Childs Nerv Syst
Gil Z, Rao S, Constantini S (2000) Expansion of chiari I-associated syringomyelia after posterior-fossa decompression. Childs Nerv Syst 16(9):555–558
Godzik J, Kelly MP, Radmanesh A, Kim D, Holekamp TF, Smyth MD, Lenke LG, Shimony JS, Park TS, Leonard J, Limbrick DD (2014) Relationship of syrinx size and tonsillar descent to spinal deformity in chiari malformation type I with associated syringomyelia. J Neurosurg Pediatr 13(4):368–374
Goel A (2015) Is atlantoaxial instability the cause of chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine 22(2):116–127
Goldstein HE, Anderson RC (2015) Craniovertebral junction instability in the setting of chiari I malformation. Neurosurg Clin N Am 26(4):561–569
Greenlee JD, Donovan KA, Hasan DM, Menezes AH (2002) Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics. 110(6):1212–1219
Guan J, Riva-Cambrin J, Brockmeyer DL (2016) Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients. Neurosurg Focus 41(5):E2
Gupta SN, Belay B (2008) Intracranial incidental findings on brain MR images in a pediatric neurology practice: a retrospective study. J Neurol Sci 264(1–2):34–37
Guyotat J, Bret P, Jouanneau E, Ricci AC, Lapras C (1998) 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 140(8):745–754
Haines SJ, Berger M (1991) Current treatment of chiari malformations types I and II: a survey of the pediatric section of the american association of neurological surgeons. Neurosurgery. 28(3):353–357
Hanieh A, Sutherland A, Foster B, Cundy P (2000) Syringomyelia in children with primary scoliosis. Childs Nerv Syst 16(4):200–202
Haroun RI, Guarnieri M, Meadow JJ, Kraut M, Carson BS (2000) Current opinions for the treatment of syringomyelia and chiari malformations: survey of the pediatric section of the american association of neurological surgeons. Pediatr Neurosurg 33(6):311–317
Henderson FCS, Austin C, Benzel E et al (2017) Neurological and spinal manifestations of the ehlers-danlos syndromes. Am J Med Genet C: Semin Med Genet 175(1):195–211
Hida K, Iwasaki Y, Koyanagi I, Abe H (1999) Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol 51(4):383–390 discussion 390-1
Hwang SW, Samdani AF, Jea A, Raval A, Gaughan JP, Betz RR, Cahill PJ (2012) Outcomes of chiari I-associated scoliosis after intervention: a meta-analysis of the pediatric literature. Childs Nerv Syst 28(8):1213–1219
Isu T, Iwasaki Y, Akino M, Abe H (1990) Hydrosyringomyelia associated with a chiari I malformation in children and adolescents. Neurosurgery. 26(4):591–596 discussion 596-7
Isu T, Sasaki H, Takamura H, Kobayashi N (1993) Foramen magnum decompression with removal of the outer layer of the dura as treatment for syringomyelia occurring with chiari I malformation. Neurosurgery. 33(5):845–849 discussion 849-50
Jiang E, Sha S, Yuan X, Zhu WG, Jiang J, Ni H, Liu Z, Qiu Y, Zhu Z (2018) Comparison of clinical and radiographic outcomes for posterior fossa decompression with and without duraplasty for treatment of pediatric chiari I malformation: a prospective study. World Neurosurg 110:e465–e472
Kahn EN, Muraszko KM, Maher CO (2015) Prevalence of chiari I malformation and syringomyelia. Neurosurg Clin N Am 26(4):501–507
Kennedy BC, Kelly KM, Phan MQ, Bruce SS, McDowell MM, Anderson RCE, Feldstein NA (2015) Outcomes after suboccipital decompression without dural opening in children with chiari malformation type I. J Neurosurg Pediatr 16(2):150–158
Khalsa SSS, Siu A, DeFreitas TA et al (2017) Comparison of posterior fossa volumes and clinical outcomes after decompression of chiari malformation type I. J Neurosurg Pediatr 19(5):511–517
Krieger MD, Falkinstein Y, Bowen IE, Tolo VT, McComb JG (2011) Scoliosis and chiari malformation type I in children. J Neurosurg Pediatr 7(1):25–29
Krieger MD, McComb JG, Levy ML (1999) Toward a simpler surgical management of chiari I malformation in a pediatric population. Pediatr Neurosurg 30(3):113–121
Lazareff JA, Galarza M, Gravori T, Spinks TJ (2002) Tonsillectomy without craniectomy for the management of infantile chiari I malformation. J Neurosurg 97(5):1018–1022
Lee A, Yarbrough CK, Greenberg JK, Barber J, Limbrick DD, Smyth MD (2014) Comparison of posterior fossa decompression with or without duraplasty in children with type I chiari malformation. Childs Nerv Syst 30(8):1419–1424
Limonadi FM, Selden NR (2004) Dura-splitting decompression of the craniocervical junction: reduced operative time, hospital stay, and cost with equivalent early outcome. J Neurosurg 101(2 Suppl):184–188
Lin W, Duan G, Xie J, Shao J, Wang Z, Jiao B (2018) Comparison of results between posterior fossa decompression with and without duraplasty for the surgical treatment of chiari malformation type I: a systematic review and meta-analysis. World Neurosurg 110:460–474.e5
Liu H, Yang C, Yang J, Xu Y (2017) Pediatric chiari malformation type I: long-term outcomes following small-bone-window posterior fossa decompression with autologous-fascia duraplasty. Exp Ther Med 14(6):5652–5658
Lu VM, Phan K, Crowley SP, Daniels DJ (2017) The addition of duraplasty to posterior fossa decompression in the surgical treatment of pediatric chiari malformation type I: a systematic review and meta-analysis of surgical and performance outcomes. J Neurosurg Pediatr 20(5):439–449
Massimi L, Caldarelli M, Paternoster G, Novegno F, Tamburrini G, Di Rocco C (2008) Mini-invasive surgery for chiari type I malformation. Neuroradiol J 21(1):65–70
Massimi L, Frassanito P, Chieffo D, Tamburrini G, Caldarelli M (2019) Bony decompression for chiari malformation type I: long-term follow-up. Acta Neurochir Suppl 125:119–124
Massimi L, Peppucci E, Peraio S, Di Rocco C (2011) History of chiari type I malformation. Neurol Sci 32(Suppl 3):S263–S265
Massimi L, Pravata E, Tamburrini G et al (2011) Endoscopic third ventriculostomy for the management of chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery. 68(4):950–956
Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic chiari type I malformations identified on magnetic resonance imaging. J Neurosurg 92(6):920–926
Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain herniation syndrome (chiari I): data base analysis. Pediatr Neurosurg 23(5):260–269
Mikulis DJ, Diaz O, Egglin TK, Sanchez R (1992) Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology. 183(3):725–728
Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA (2007) Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine 7(6):601–609
Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005–1017
Naftel RP, Tubbs RS, Menendez JY, Wellons JC 3rd, Pollack IF, Oakes WJ (2013) Worsening or development of syringomyelia following chiari I decompression: case report. J Neurosurg Pediatr 12(4):351–356
Nagib MG (1994) An approach to symptomatic children (ages 4-14 years) with chiari type I malformation. Pediatr Neurosurg 21(1):31–35
Navarro R, Olavarria G, Seshadri R, Gonzales-Portillo G, McLone DG, Tomita T (2004) Surgical results of posterior fossa decompression for patients with chiari I malformation. Childs Nerv Syst 20(5):349–356
Nohria V, Oakes WJ (1990) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16(4–5):222–227
Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (2017) Pathogenesis of Chiari I pathophysiology of Syringomyelia: implications for therapy: A summary of 3 decades of clinical research. Neurosurgery 64(CN_suppl_1):66–77
Passias PG, Pyne A, Horn SR, Poorman GW, Janjua MB, Vasquez-Montes D, Bortz CA, Segreto FA, Frangella NJ, Siow MY, Sure A, Zhou PL, Moon JY, Diebo BG, Vira SN (2018) Developments in the treatment of chiari type 1 malformations over the past decade. J Spine Surg 4(1):45–54
Piper RJ, Pike M, Harrington R, Magdum SA (2019) Chiari malformations: principles of diagnosis and management. BMJ. 365:l1159
Pomeraniec IJ, Ksendzovsky A, Awad AJ, Fezeu F, Jane JA Jr (2016) Natural and surgical history of chiari malformation type I in the pediatric population. J Neurosurg Pediatr 17(3):343–352
Poretti A, Ashmawy R, Garzon-Muvdi T, Jallo GI, Huisman TA, Raybaud C (2016) Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 47(5):293–307
Raza-Knight S, Mankad K, Prabhakar P, Thompson D (2017) Headache outcomes in children undergoing foramen magnum decompression for chiari I malformation. Arch Dis Child 102(3):238–243
Rekate HL (2008) Natural history of the chiari type I anomaly. J Neurosurg Pediatr 2(3):177–178 discussion 178
Riordan CP, Scott RM (2018) Fourth ventricle stent placement for treatment of recurrent syringomyelia in patients with type I chiari malformations. J Neurosurg Pediatr 23(2):164–170
Rocque BG, George TM, Kestle J, Iskandar BJ (2011) Treatment practices for chiari malformation type I with syringomyelia: results of a survey of the American society of pediatric neurosurgeons. J Neurosurg Pediatr 8(5):430–437
Sacco D, Scott RM (2003) Reoperation for chiari malformations. Pediatr Neurosurg 39(4):171–178
Schijman E, Steinbok P (2004) International survey on the management of chiari I malformation and syringomyelia. Childs Nerv Syst 20(5):341–348
Schwedt TJ, Guo Y, Rothner AD (2006) “Benign” imaging abnormalities in children and adolescents with headache. Headache. 46(3):387–398
Sinclair N, Assaad N, Johnston I (2002) Pseudotumour cerebri occurring in association with the chiari malformation. J Clin Neurosci 9(1):99–101
Singhal A, Cheong A, Steinbok P (2018) International survey on the management of chiari 1 malformation and syringomyelia: evolving worldwide opinions. Childs Nerv Syst 34(6):1177–1182
Smith BW, Strahle J, Bapuraj JR, Muraszko KM, Garton HJ, Maher CO (2013) Distribution of cerebellar tonsil position: implications for understanding chiari malformation. J Neurosurg 119(3):812–819
Soleman J, Bartoli A, Korn A, Constantini S, Roth J. (2018) Treatment failure of syringomyelia associated with chiari I malformation following foramen magnum decompression: how should we proceed? Neurosurg Rev
Soleman J, Roth J, Bartoli A, Rosenthal D, Korn A, Constantini S (2017) Syringo-subarachnoid shunt for the treatment of persistent syringomyelia following decompression for chiari type I malformation: surgical results. World Neurosurg 108:836–843
Strahle J Pediatrics-radiologic and clinical predictors of scoliosis in patients with chiari malformation type I and spinal cord syrinx from the park-reeves syringomyelia research consortium. J Neurosurg (Accepted, Ahead of Print)
Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJ, Maher CO (2011) Chiari malformation associated with craniosynostosis. Neurosurg Focus 31(3):E2
Strahle J, Muraszko KM, Garton HJ et al (2015) Syrinx location and size according to etiology: identification of chiari-associated syrinx. J Neurosurg Pediatr 16(1):21–29
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Chiari malformation type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr 8(2):205–213
Tamburrini G, Caldarelli M, Massimi L, Gasparini G, Pelo S, Di Rocco C (2012) Complex craniosynostoses: a review of the prominent clinical features and the related management strategies. Childs Nerv Syst 28(9):1511–1523
Trigylidas T, Baronia B, Vassilyadi M, Ventureyra EC (2008) Posterior fossa dimension and volume estimates in pediatric patients with chiari I malformations. Childs Nerv Syst 24(3):329–336
Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC, Rozzelle CJ, Blount JP, Oakes WJ (2011) Institutional experience with 500 cases of surgically treated pediatric chiari malformation type I. J Neurosurg Pediatr 7(3):248–256
Tubbs RS, Cohen-Gadol AA (2010) Hans chiari (1851-1916). J Neurol 257(7):1218–1220
Tubbs RS, Hill M, Loukas M, Shoja MM, Oakes WJ (2008) Volumetric analysis of the posterior cranial fossa in a family with four generations of the chiari malformation type I. J Neurosurg Pediatr 1(1):21–24
Tubbs RS, Lyerly MJ, Loukas M, Shoja MM, Oakes WJ (2007) The pediatric chiari I malformation: a review. Childs Nerv Syst 23(11):1239–1250
Tubbs RS, McGirt MJ, Oakes WJ (2003) Surgical experience in 130 pediatric patients with chiari I malformations. J Neurosurg 99(2):291–296
Tubbs RS, Smyth MD, Wellons JC 3rd, Oakes WJ (2004) Arachnoid veils and the chiari I malformation. J Neurosurg 100(5 Suppl Pediatrics):465–467
Valentini L, Visintini S, Saletti V, Chiapparini L, Estienne M, Solero CL (2011) Treatment for chiari 1 malformation (CIM): analysis of a pediatric surgical series. Neurol Sci 32(Suppl 3):S321–S324
Vega A, Quintana F, Berciano J (1990) Basichondrocranium anomalies in adult chiari type I malformation: a morphometric study. J Neurol Sci 99(2–3):137–145
Wetjen NM, Heiss JD, Oldfield EH (2008) Time course of syringomyelia resolution following decompression of chiari malformation type I. J Neurosurg Pediatr 1(2):118–123
Wilkinson DA, Johnson K, Garton HJ, Muraszko KM, Maher CO (2017) Trends in surgical treatment of chiari malformation type I in the United States. J Neurosurg Pediatr 19(2):208–216
Williams B (1980) On the pathogenesis of syringomyelia: a review. J R Soc Med 73(11):798–806
Won DJ, Nambiar U, Muszynski CA, Epstein FJ (1997) Coagulation of herniated cerebellar tonsils for cerebrospinal fluid pathway restoration. Pediatr Neurosurg 27(5):272–275
Wu T, Zhu Z, Jiang J, Zheng X, Sun X, Qian B, Zhu F, Qiu Y (2012) Syrinx resolution after posterior fossa decompression in patients with scoliosis secondary to chiari malformation type I. Eur Spine J 21(6):1143–1150
Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM (1999) Pediatric chiari I malformations: do clinical and radiologic features correlate? Neurology. 53(6):1271–1276
Xie D, Qiu Y, Sha S, Liu Z, Jiang L, Yan H, Chen L, Shi B, Zhu Z (2015) Syrinx resolution is correlated with the upward shifting of cerebellar tonsil following posterior fossa decompression in pediatric patients with chiari malformation type I. Eur Spine J 24(1):155–161
Xu H, Chu L, He R, Ge C, Lei T (2017) Posterior fossa decompression with and without duraplasty for the treatment of chiari malformation type I-a systematic review and meta-analysis. Neurosurg Rev 40(2):213–221
Yan H, Han X, Jin M, Liu Z, Xie D, Sha S, Qiu Y, Zhu Z (2016) Morphometric features of posterior cranial fossa are different between chiari I malformation with and without syringomyelia. Eur Spine J 25(7):2202–2209
Zhao JL, Li MH, Wang CL, Meng W (2016) A systematic review of chiari I malformation: techniques and outcomes. World Neurosurg 88:7–14
Bartoli A, Soleman J, Berger A, Wisoff JH, Hidalgo ET, Mangano FT, Keating RF, Thomale UW, Boop F, Roth J, Constantini S, Treatment options for hydrocephalus following foramen magnum decompression for Chiari I malformation: A multicenter study., Neurosurgery 2019, PMID 31232427.
Acknowledgements
The authors would like to acknowledge the support of the American Syringomyelia and Chiari Alliance Project for grant support in the above clinical research at CNMC.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Alexander, H., Tsering, D., Myseros, J.S. et al. Management of Chiari I malformations: a paradigm in evolution. Childs Nerv Syst 35, 1809–1826 (2019). https://doi.org/10.1007/s00381-019-04265-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-019-04265-2