Abstract
Background and definition
In recent years thanks to the growing use of radiological assessment, Chiari I malformation became one of the major diseases for a neurosurgeon to deal with. CIM can be classified according to the extent of cerebellar tonsil tip into the foramen magnum being a protrusion over five mm considered pathological. Such a disease is a heterogeneous condition with a multifactorial pathogenetic mechanism that can subdivided into a primary and secondary form. Regardless of the form, it seems that CIM is the result of an imbalance between the volume of the braincase and its content. Acquired CIMs are secondary to conditions causing intracranial hypertension or hypotension while the pathogenesis of primary forms is still controversial.
Pathogenesis and treatment
There are several theories in the literature but the most accepted one implies an overcrowding due to a small posterior cranial fossa. While asymptomatic CIM do not need treatment, symptomatic ones prompt for surgical management. Several techniques are proposed being the dilemma centered in the need for dural opening procedures and bony decompression ones.
Conclusion
Alongside the paper, the authors will address the novelty presented in the literature on management, diagnosis and pathogenesis in order to offer a better understanding of such a heterogeneous pathology.
Similar content being viewed by others
Data availability
Not applicable.
Abbreviations
- CIM:
-
Chiari I malformation
- Cine-PCMR:
-
Cardiac gated cine phase-contrast MR
- CVJ:
-
Cranio-vertebral junction
References
Schijman E (2004) History, anatomic forms, and pathogenesis of Chiari I malformations. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 20(5):323–328
Ciaramitaro P, Garbossa D, Peretta P et al (2020) Syringomyelia and Chiari Syndrome Registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort. Ann Ist Super Sanita 56(1):48–58
Basaran R, Bozdogan C, Senol M, Gundogan D, Isik N (2021) Long-term outcomes of surgical management in subtypes of Chiari malformation. Neurol Res 43(9):760–766
Massimi L, Peretta P, Erbetta A et al (2022) Diagnosis and treatment of Chiari malformation type 1 in children: the international consensus document. Neurol Sci Off J Ital Neurol Soc Ital Soc Clin Neurophysiol 43(2):1311–1326
Massimi L, Novegno F, di Rocco C (2011) Chiari type I malformation in children. Adv Tech Stand Neurosurg 37:143–211
Han Y, Chen M, Wang H (2021) Chiari I malformation in patients with RASopathies. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 37(6):1831–1836
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(5):1050–1054; discussion 1054–1055
Bernard S, Loukas M, Rizk E, Oskouian RJ, Delashaw J, Tubbs RS (2015) The human occipital bone: review and update on its embryology and molecular development. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 31(12):2217–2223
Coll G, Abed Rabbo F, Jecko V, Sakka L, Di Rocco F, Delion M (2019) The growth of the posterior cranial fossa in FGFR2-induced faciocraniosynostosis: a review. Neurochirurgie 65(5):221–227
den Ottelander BK, de Goederen R, de Planque CA, Baart SJ, van Veelen MLC, Corel LJA, Joosten KFM, Mathijssen IMJ, Dremmen MHG (2021) Cervical spinal cord compression and sleep-disordered breathing in syndromic craniosynostosis. AJNR Am J Neuroradiol 42(1):201–205
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
Saldino RM, Steinbach HL, Epstein CJ (1972) Familial acrocephalosyndactyly (Pfeiffer syndrome). Am J Roentgenol Radium Ther Nucl Med 116(3):609–622
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
Goel A, Jadhav D, Shah A, Rai S, Dandpat S, Vutha R, Dhar A, Prasad A (2020) Chiari 1 formation redefined-clinical and radiographic observations in 388 surgically treated patients. World Neurosurg 141:e921–e934
Taylor DG, Chatrath A, Mastorakos P, Paisan G, Chen C-J, Buell TJ, Jane JA (2020) Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. J Neurosurg 134(3):825–830
Yilmaz TF, Toprak H, Sari L, Oz II, Kitis S, Kaya A, Alkan A (2022) Chiari type 1 malformation: CSF flow dynamics and morphology in the posterior fossa and craniocervical junction and correlation of these findings with syrinx formation. Neurochirurgie 68(6):595–600
Dien Esquivel MF, Gupta N, Wilson N, O’Brien CA, Gladkikh M, Barrowman N, Bijelić V, Tu A (2022) Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 38(11):2119–2128
Provenzano A, La Barbera A, Scagnet M et al (2021) Chiari 1 malformation and exome sequencing in 51 trios: the emerging role of rare missense variants in chromatin-remodeling genes. Hum Genet 140(4):625–647
Sadler B, Wilborn J, Antunes L et al (2021) Rare and de novo coding variants in chromodomain genes in Chiari I malformation. Am J Hum Genet 108(2):368
Boyles AL, Enterline DS, Hammock PH et al (2006) Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15. Am J Med Genet A 140(24):2776–2785
Merello E, Tattini L, Magi A et al (2017) Exome sequencing of two Italian pedigrees with non-isolated Chiari malformation type I reveals candidate genes for cranio-facial development. Eur J Hum Genet EJHG 25(8):952–959
Nagy L, Mobley J, Ray C (2016) Familial aggregation of Chiari malformation: presentation, pedigree, and review of the literature. Turk Neurosurg 26(2):315–320
AvŞar T, ÇaliŞ Ş, Yilmaz B, Demİrcİ OG, Holyavkİn C, KiliÇ T (2020) Genome-wide identification of Chiari malformation type I associated candidate genes and chromosomal variations. Turk J Biol Turk Biyol Derg 44(6):449–456
Urbizu A, Garrett ME, Soldano K et al (2021) Rare functional genetic variants in COL7A1, COL6A5, COL1A2 and COL5A2 frequently occur in Chiari malformation type 1. PLoS ONE 16(5):e0251289
Brockmeyer DL, Cheshier SH, Stevens J, Facelli JC, Rowe K, Heiss JD, Musolf A, Viskochil DH, Allen-Brady KL, Cannon-Albright LA (2022) A likely HOXC4 predisposition variant for Chiari malformations. J Neurosurg 1–9
Saletti V, Esposito S, Maccaro A, Giglio S, Valentini LG, Chiapparini L (2017) Chiari I malformation in a child with PTEN hamartoma tumor syndrome: association or coincidence? Eur J Med Genet 60(5):261–264
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 ChNS Off J Int Soc Pediatr Neurosurg 21(10):889–901
Rijken BFM, Lequin MH, van der Lijn F, van Veelen-Vincent M-LC, de Rooi J, Hoogendam YY, Niessen WJ, Mathijssen IMJ (2015) The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes. J Cranio-Maxillo-fac Surg Off Publ Eur Assoc Cranio-Maxillo-fac Surg 43(6):813–819
Raybaud C, Di Rocco C (2007) Brain malformation in syndromic craniosynostoses, a primary disorder of white matter: a review. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 23(12):1379–1388
Lock EF, Soldano KL, Garrett ME, Cope H, Markunas CA, Fuchs H, Grant G, Dunson DB, Gregory SG, Ashley-Koch AE (2015) Joint eQTL assessment of whole blood and dura mater tissue from individuals with Chiari type I malformation. BMC Genomics 16(1):11
Beretta E, Vetrano IG, Curone M, Chiapparini L, Furlanetto M, Bussone G, Valentini LG (2017) Chiari malformation-related headache: outcome after surgical treatment. Neurol Sci Off J Ital Neurol Soc Ital Soc Clin Neurophysiol 38(Suppl 1):95–98
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
Curone M, Valentini LG, Vetrano I, Beretta E, Furlanetto M, Chiapparini L, Erbetta A, Bussone G (2017) Chiari malformation type 1-related headache: the importance of a multidisciplinary study. Neurol Sci Off J Ital Neurol Soc Ital Soc Clin Neurophysiol 38(Suppl 1):91–93
Khalsa SSS, Siu A, DeFreitas TA, Cappuzzo JM, Myseros JS, Magge SN, Oluigbo CO, Keating RF (2017) Comparison of posterior fossa volumes and clinical outcomes after decompression of Chiari malformation type I. J Neurosurg Pediatr 19(5):511–517
Poretti A, Ashmawy R, Garzon-Muvdi T, Jallo GI, Huisman TAGM, Raybaud C (2016) Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics 47(5):293–307
Olszewski AM, Proctor MR (2018) Headache, Chiari I malformation and foramen magnum decompression. Curr Opin Pediatr 30(6):786–790
Novegno F (2019) Clinical diagnosis-part II: what is attributed to Chiari I. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1681–1693
Houston JR, Maleki J, Loth F, Klinge PM, Allen PA (2022) Influence of pain on cognitive dysfunction and emotion dysregulation in Chiari malformation type I. Adv Exp Med Biol 1378:155–178
Rogers JM, Savage G, Stoodley MA (2018) A systematic review of cognition in Chiari I malformation. Neuropsychol Rev 28(2):176–187
Noureldine MHA, Shimony N, Jallo GI, Groves ML (2019) Scoliosis in patients with Chiari malformation type I. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1853–1862
D’Arco F, Ganau M (2019) Which neuroimaging techniques are really needed in Chiari I? A short guide for radiologists and clinicians. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1801–1808
Chibbaro S, Cebula H, Aldea S et al (2017) Endonasal endoscopic odontoidectomy in ventral diseases of the craniocervical junction: results of a multicenter experience. World Neurosurg 106:382–393
Klekamp J (2015) Chiari I malformation with and without basilar invagination: a comparative study. Neurosurg Focus 38(4):E12
Salunke P, Sura S, Futane S, Aggarwal A, Khandelwal NK, Chhabra R, Mukherjee KK, Gupta SK (2012) Ventral compression in adult patients with Chiari 1 malformation sans basilar invagination: cause and management. Acta Neurochir (Wien) 154(1):147–152
Almudayni A, Alharbi M, Chowdhury A, Ince J, Alablani F, Minhas JS, Lecchini-Visintini A, Chung EML (2023) Magnetic resonance imaging of the pulsing brain: a systematic review. Magma N Y N 36(1):3–14
Bhadelia RA, Chang Y-M, Oshinski JN, Loth F (2023) Cerebrospinal fluid flow and brain motion in Chiari I malformation: past, present, and future. J Magn Reson Imaging JMRI. https://doi.org/10.1002/jmri.28717
Wymer DT, Patel KP, Burke WF, Bhatia VK (2020) Phase-contrast MRI: physics, techniques, and clinical applications. Radiogr Rev Publ Radiol Soc N Am Inc 40(1):122–140
Ohno N, Miyati T, Noda T, Alperin N, Hamaguchi T, Ohno M, Matsushita T, Mase M, Gabata T, Kobayashi S (2020) Fast phase-contrast cine MRI for assessing intracranial hemodynamics and cerebrospinal fluid dynamics. Diagn Basel Switz 10(4):241
Bezuidenhout AF, Chang Y-M, Heilman CB, Bhadelia RA (2019) Headache in Chiari malformation. Neuroimaging Clin N Am 29(2):243–253
Heiss JD, Suffredini G, Smith R, DeVroom HL, Patronas NJ, Butman JA, Thomas F, Oldfield EH (2010) Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article J Neurosurg Spine 13(6):729–742
Manara R, Concolino D, Rampazzo A, Zanetti A, Tomanin R, Faggin R, Scarpa M (2014) Chiari 1 malformation and holocord syringomyelia in hunter syndrome. JIMD Rep 12:31–35
Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJL, Maher CO (2011) Chiari malformation associated with craniosynostosis. Neurosurg Focus 31(3):E2
Grahovac G, Pundy T, Tomita T (2018) Chiari type I malformation of infants and toddlers. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 34(6):1169–1176
Amin R, Sayal P, Sayal A, Massicote C, Pham R, Al-Saleh S, Drake J, Narang I (2015) The association between sleep-disordered breathing and magnetic resonance imaging findings in a pediatric cohort with Chiari 1 malformation. Can Respir J 22(1):31–36
Leu RM (2015) Sleep-related breathing disorders and the Chiari 1 malformation. Chest 148(5):1346–1352
Losurdo A, Dittoni S, Testani E, Di Blasi C, Scarano E, Mariotti P, Paternoster G, Di Rocco C, Massimi L, Della Marca G (2013) Sleep disordered breathing in children and adolescents with Chiari malformation type I. J Clin Sleep Med JCSM Off Publ Am Acad Sleep Med 9(4):371–377
Zaffanello M, Darra F, Lo Barco T, Sala F, Gasperi E, Piacentini G (2017) Chiari 1 malformation in a child with febrile seizures, parasomnias, and sleep apnea syndrome. Case Rep Pediatr 2017:8189790
Davidson L, Phan TN, Myseros JS, Magge SN, Oluigbo C, Sanchez CE, Keating RF (2021) Long-term outcomes for children with an incidentally discovered Chiari malformation type 1: what is the clinical significance? Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 37(4):1191–1197
Killeen A, Roguski M, Chavez A, Heilman C, Hwang S (2015) Non-operative outcomes in Chiari I malformation patients. J Clin Neurosci Off J Neurosurg Soc Australas 22(1):133–138
Massimi L, Frassanito P, Bianchi F, Tamburrini G, Caldarelli M (2019) Bony decompression vs duraplasty for Chiari I malformation: does the eternal dilemma matter? Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1827–1838
Cuthbert H, Pepper J, Price R (2021) Spontaneous resolution of a Chiari malformation with syringomyelia. BMJ Case Rep 14(6):e241789
Gallo E, Rahmathulla G, Rao D, Tavanaiepour K, Tavanaiepour D (2021) Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case. J Neurosurg Case Lessons 1(26):CASE21236
Lumish HS, Wynn J, Devinsky O, Chung WK (2015) Brief report: SETD2 mutation in a child with autism, intellectual disabilities and epilepsy. J Autism Dev Disord 45(11):3764–3770
Strahle J, Smith BW, Martinez M, Bapuraj JR, Muraszko KM, Garton HJL, Maher CO (2015) The association between Chiari malformation type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr 15(6):607–611
Moncho D, Poca MA, Minoves T, Ferré A, Cañas V, Sahuquillo J (2017) Are evoked potentials clinically useful in the study of patients with Chiari malformation type 1? J Neurosurg 126(2):606–619
Frassanito P, Bianchi F, Pennisi G, Massimi L, Tamburrini G, Caldarelli M (2019) The growth of the neurocranium: literature review and implications in cranial repair. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(9):1459–1465
Naylor RM, Rotter J, Proctor MR, Bite U, Ahn ES (2023) Above-the-hairline suboccipital transverse approach for the treatment of Chiari I malformation. Oper Neurosurg Hagerstown Md 24(5):542–547
Tyagi G, Uppar AM, Pruthi N, Shukla D (2023) Minimally invasive foramen magnum decompression using tubular retractors (MIFT) for Chiari I malformations. Acta Neurochir (Wien) 165(3):767–770
Lu VM, Daniels DJ, Haile DT, Ahn ES (2020) Effects of intraoperative liposomal bupivacaine on pain control and opioid use after pediatric Chiari I malformation surgery: an initial experience. J Neurosurg Pediatr 27(1):9–15
Canheu AC, Santos MV, Furlanetti LL, Salomão JFM, de Oliveira RS (2020) The Brazilian Society for Pediatric Neurosurgery: consensus on Chiari I deformity. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 36(1):17–18
Chai Z, Xue X, Fan H, Sun L, Cai H, Ma Y, Ma C, Zhou R (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
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
Han RK, Medina MP, Giantini-Larsen AM, Chae JK, Cruz A, Garton ALA, Greenfield JP (2023) Fourth ventricular subarachnoid stent for Chiari malformation type I-associated persistent syringomyelia. Neurosurg Focus 54(3):E10
Spennato P, Vitulli F, Tafuto R, Imperato A, Mirone G, Cinalli G (2023) Fourth ventricle to spinal subarachnoid space stenting in pediatric patients with refractory syringomyelia: case series and systematic review. Neurosurg Rev 46(1):67
Sun P, Zhou M, Liu Y, Du J, Zeng G (2023) Fourth ventricle stent placement for treatment of type I Chiari malformation in children. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 39(3):671–676
Narenthiran G, Parks C, Pettorini B (2015) Management of Chiari I malformation in children: effectiveness of intra-operative ultrasound for tailoring foramen magnum decompression. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 31(8):1371–1376
Jiang E, Sha S, Yuan X, Zhu W, 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
Kitamura S, Ito K, Horiuchi T (2022) Dural sac restenosis due to regeneration of the resected C1 lamina in a patient with Chiari malformation: a case report and literature review. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 38(8):1621–1624
Tochigi S, Isoshima A, Ohashi H, Kawamura D, Karagiozov K, Hatano K, Ohashi S, Nagashima H, Murayama Y, Abe T (2023) Preoperative assessment of dominant occipital sinus in patients with Chiari malformation type I: anatomical variations and implications for preventing potentially life-threatening surgical complications. J Neurosurg 138(2):540–549
Zipfel J, da Silva FR, Lafitte LM, Kural C, Schuhmann MU (2018) U-shaped durotomy for midline posterior fossa tumor removal: technical note and evaluation of results. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 34(11):2299–2303
Hamrick F, Eli IM, Hunsaker J, Kim RB, Karsy M, Schmidt RH (2023) Dual dural patch graft with AlloDerm and DuraGen underlay for duraplasty in Chiari malformation results in significantly decreased cerebrospinal fluid leak complications. Oper Neurosurg Hagerstown Md 24(2):162–167
Hu Z, Liao G, Lu Y, Wang C, Mei J (2022) Comparison of dural graft types and graft fixation methods in Chiari malformation type i decompression surgery. World Neurosurg 164:e458–e462
Makoshi Z, Toop N, Smith LGF, Drapeau A, Pindrik J, Sribnick EA, Leonard J, Shaikhouni A (2022) Association between synthetic sealants and increased complication rates in posterior fossa decompression with duraplasty for Chiari malformations regardless of graft type. J Neurosurg Pediatr 1–10
Foreman P, Safavi-Abbasi S, Talley MC, Boeckman L, Mapstone TB (2012) Perioperative outcomes and complications associated with allogeneic duraplasty for the management of Chiari malformations type I in 48 pediatric patients. J Neurosurg Pediatr 10(2):142–149
Noguchi-Shinohara M, Hamaguchi T, Kitamoto T, Sato T, Nakamura Y, Mizusawa H, Yamada M (2007) Clinical features and diagnosis of dura mater graft associated Creutzfeldt Jakob disease. Neurology 69(4):360–367
Sen RD, Martinez V, Eaton J, Holdefer RN, Ellenbogen RG (2023) Intraoperative neuromonitoring for pediatric Chiari decompression: when is it useful? Neurosurg Focus 54(3):E9
Furtado SV, Thakar S, Hegde AS (2011) Correlation of functional outcome and natural history with clinicoradiological factors in surgically managed pediatric Chiari I malformation. Neurosurgery 68(2):319–327; discussion 328
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
Alexander H, Tsering D, Myseros JS, Magge SN, Oluigbo C, Sanchez CE, Keating RF (2019) Management of Chiari I malformations: a paradigm in evolution. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1809–1826
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
Mazzola CA, Fried AH (2003) Revision surgery for Chiari malformation decompression. Neurosurg Focus 15(3):E3
Greenberg JK, Yarbrough CK, Radmanesh A, Godzik J, Yu M, Jeffe DB, Smyth MD, Park TS, Piccirillo JF, Limbrick DD (2015) The Chiari severity index: a preoperative grading system for Chiari malformation type 1. Neurosurgery 76(3):279–285; discussion 285
Aliaga L, Hekman KE, Yassari R, Straus D, Luther G, Chen J, Sampat A, Frim D (2012) A novel scoring system for assessing Chiari malformation type I treatment outcomes. Neurosurgery 70(3):656–664; discussion 664–665
Yarbrough CK, Greenberg JK, Smyth MD, Leonard JR, Park TS, Limbrick DD (2014) External validation of the Chicago Chiari Outcome Scale. J Neurosurg Pediatr 13(6):679–684
Savchuk S, Jin MC, Choi S, Kim LH, Quon JL, Bet A, Prolo LM, Hong DS, Mahaney KB, Grant GA (2021) Incorporating patient-centered quality-of-life measures for outcome assessment after Chiari malformation type I decompression in a pediatric population: a pilot study. J Neurosurg Pediatr 1–8
LoPresti MA, Pan I-W, Gadgil N, Wagner K, Lam S (2019) Outcomes and resource utilization in surgery for Chiari I malformation in a national network of children’s hospitals. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(4):657–664
Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 20(5):341–348
Loukas M, Shayota BJ, Oelhafen K, Miller JH, Chern JJ, Tubbs RS, Oakes WJ (2011) Associated disorders of Chiari type I malformations: a review. Neurosurg Focus 31(3):E3
Di Rocco C, Frassanito P, Massimi L, Peraio S (2011) Hydrocephalus and Chiari type I malformation. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 27(10):1653–1664
Massimi L, Pennisi G, Frassanito P, Tamburrini G, Di Rocco C, Caldarelli M (2019) Chiari type I and hydrocephalus. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1701–1709
Park H, Oh S, Choi JW, Ra YS (2021) Complete posterior cranial vault distraction osteogenesis to correct Chiari malformation type I associated with craniosynostosis. J Neurosurg Pediatr 1–7
Vankipuram S, Ellenbogen J, Sinha AK (2022) Management of Chiari 1 malformation and hydrocephalus in syndromic craniosynostosis: a review. J Pediatr Neurosci 17(Suppl 1):S67–S76
Valentini LG, Saletti V, Erbetta A, Chiapparini L, Furlanetto M (2019) Chiari 1 malformation and untreated sagittal synostosis: a new subset of complex Chiari? Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1741–1753
Tamburrini G, Offi M, Massimi L, Frassanito P, Bianchi F (2021) Posterior vault “free-floating” bone flap: indications, technique, advantages, and drawbacks. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 37(10):3143–3147
Ros B, Iglesias S, Selfa A, Ruiz F, Arráez MÁ (2021) Conventional posterior cranial vault expansion: indications and results-review of the literature. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 37(10):3149–3175
Khansa I, Drapeau AI, Pearson GD (2023) Posterior cranial distraction in craniosynostosis: a systematic review of the literature. Cleft Palate-Craniofacial J Off Publ Am Cleft Palate-Craniofacial Assoc 10556656231168548
Chatterjee S, Shivhare P, Verma SG (2019) Chiari malformation and atlantoaxial instability: problems of co-existence. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1755–1761
Goel A (2018) Basilar invagination, syringomyelia and Chiari formation and their relationship with atlantoaxial instability. Neurol India 66(4):940–942
Ho WSC, Brockmeyer DL (2019) Complex Chiari malformation: using craniovertebral junction metrics to guide treatment. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 35(10):1847–1851
Alperin N, Loftus JR, Oliu CJ, Bagci AM, Lee SH, Ertl-Wagner B, Green B, Sekula R (2014) Magnetic resonance imaging measures of posterior cranial fossa morphology and cerebrospinal fluid physiology in Chiari malformation type I. Neurosurgery 75(5):515–522; discussion 522
Iskandar BJ, Quigley M, Haughton VM (2004) Foramen magnum cerebrospinal fluid flow characteristics in children with Chiari I malformation before and after craniocervical decompression. J Neurosurg 101(2 Suppl):169–178
Sansur CA, Heiss JD, DeVroom HL, Eskioglu E, Ennis R, Oldfield EH (2003) Pathophysiology of headache associated with cough in patients with Chiari I malformation. J Neurosurg 98(3):453–458
Author information
Authors and Affiliations
Contributions
Federico Bianchi wrote the majority of the paper and edited the main manuscript test. Benedetta Montedoro was in charge of classification and pathogenesis. Paolo Frassanito edited the photo. Luca Massimi and Gianpiero Tamburrini supervised the paper and were the main surgeon for the reported patients.
Corresponding author
Ethics declarations
Ethical approval
Not applicable.
Conflict of interest
None.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Bianchi, F., Montedoro, B., Frassanito, P. et al. Chiari I malformation: management evolution and technical innovation. Childs Nerv Syst 39, 2757–2769 (2023). https://doi.org/10.1007/s00381-023-06051-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-023-06051-7