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Chiari Malformation Type 1 and Syringomyelia: Why Do Patients Claim for International Guidelines? Commentary on the 2021 Chiari and Syringomyelia Consensus Document

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The Funnel: From the Skull Base to the Sacrum

Part of the book series: Acta Neurochirurgica Supplement ((NEUROCHIRURGICA,volume 135))

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Abstract

The diagnosis of Chiari malformation type 1 (CM1) and Syringomyelia (Syr) has become increasingly common during the past few years. Contemporarily, the body of literature on these topics is growing, although randomized controlled studies on significant case series to drive guidelines are missing in the pediatric and adult populations. As a result of the different opinions about surgical indications and techniques raised by CM1-Syr, an increasing number of well-informed but disoriented patients is emerging. To bridge this gap, an International Consensus Conference on CM1-Syr held in Milan in November 2019 aimed to find a consensus among international experts, to suggest some recommendations that, in the near future, could lead to guidelines. Here, we comment on the most relevant recommendations about the definition, diagnosis, surgical management, failures and re-intervention, and outcome. We also focus on some “wrong” indications or techniques that, although widely disapproved by the experts, and negatively experienced by many patients, are still largely in use.

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References

  1. Ciaramitaro P, Massimi L, Bertuccio A, et al. Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document. Neurol Sci. 2022;43(2):1327–42.

    Article  PubMed  Google Scholar 

  2. Massimi L, Peretta P, Erbetta A, et al. Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document. Neurol Sci. 2022;43(2):1311–26.

    Article  PubMed  Google Scholar 

  3. Ciaramitaro P, Garbossa D, Peretta P, et al. Syringomyelia and Chiari Syndrome Registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort. Ann Ist Super Sanita. 2020;56(1):48–58.

    PubMed  Google Scholar 

  4. Saletti V, Farinotti M, Peretta P, Massimi L, Ciaramitaro P, Motta S, Solari A, Valentini LG. The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature. Neurol Sci. 2021;42(12):4965–95.

    Article  PubMed  Google Scholar 

  5. Adler M, Ziglio E. Gazing into the oracle: the Delphi method and its application to social policy and public health. London: Jessica Kingsley Publishers; 1996.

    Google Scholar 

  6. Bolognese PA, Brodbelt A, Bloom AB, Kula RW. Chiari I malformation: opinions on diagnostic trends and controversies from a panel of 63 international experts. World Neurosurg. 2019;130:e9–16.

    Article  PubMed  Google Scholar 

  7. Lin W, Duan G, Xie J, Shao J, Wang Z, Jiao B. 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. 2018;110:460–474.e5.

    Article  PubMed  Google Scholar 

  8. Massimi L, Peraio S, Peppucci E, Tamburrini G, Di Rocco C. Section of the filum terminale: is it worthwhile in Chiari type I malformation? Neurol Sci. 2011;32(Suppl 3):S349–51.

    Article  PubMed  Google Scholar 

  9. Meehan WP III, Jordaan M, Prabhu SP, Carew L, Mannix RC, Proctor MR. Risk of athletes with Chiari malformations suffering catastrophic injuries during sports participation is low. Clin J Sport Med. 2015;25(2):133–7.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Spencer R, Leach P. Asymptomatic Chiari Type I malformation: should patients be advised against participation in contact sports? Br J Neurosurg. 2017;31(4):415–21.

    Article  PubMed  Google Scholar 

  11. Strahle J, Geh N, Selzer BJ, Bower R, Himedan M, Strahle M, Wetjen NM, Muraszko KM, Garton HJL, Maher CO. Sports participation with Chiari I malformation. J Neurosurg Pediatr. 2016;17(4):403–9.

    Article  PubMed  Google Scholar 

  12. Klekamp J. How should syringomyelia be defined and diagnosed? World Neurosurg. 2018;111:e729–45.

    Article  PubMed  Google Scholar 

  13. Shen J, Shen J, Huang K, Wu Y, Pan J, Zhan R. Syringobulbia in patients with Chiari malformation type I: a systematic review. Biomed Res Int. 2019;2019:4829102.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Mehta A, Chilakamarri P, Zubair A, Kuruvilla D. Chiari headache. Curr Pain Headache Rep. 2018;22(7):49.

    Article  PubMed  Google Scholar 

  15. Victorio MC, Khoury CK. Headache and Chiari I malformation in children and adolescents. Semin Pediatr Neurol. 2016;23(1):35–9.

    Article  PubMed  Google Scholar 

  16. Granata T, Valentini LG. Epilepsy in type 1 Chiari malformation. Neurol Sci. 2011;32(Suppl 3):S303–6.

    Article  PubMed  Google Scholar 

  17. Riva D, Usilla A, Saletti V, Esposito S, Bulgheroni S. Can Chiari malformation negatively affect higher mental functioning in developmental age? Neurol Sci. 2011;32(Suppl 3):S307–9.

    Article  PubMed  Google Scholar 

  18. Jayarao M, Sohl K, Tanaka T. Chiari malformation I and autism spectrum disorder: an underrecognized coexistence. J Neurosurg Pediatr. 2015;15(1):96–100.

    Article  PubMed  Google Scholar 

  19. Rogers JM, Savage G, Stoodley MA. A systematic review of cognition in Chiari I malformation. Neuropsychol Rev. 2018;28(2):176–87.

    Article  PubMed  Google Scholar 

  20. Tamburrini G, Caldarelli M, Massimi L, Gasparini G, Pelo S, Di Rocco C. Complex craniosynostoses: a review of the prominent clinical features and the related management strategies. Childs Nerv Syst. 2012;28(9):1511–23.

    Article  CAS  PubMed  Google Scholar 

  21. Massimi L, Pennisi G, Frassanito P, Tamburrini G, Di Rocco C, Caldarelli M. Chiari type I and hydrocephalus. Childs Nerv Syst. 2019;35(10):1701–9.

    Article  PubMed  Google Scholar 

  22. Massimi L, Pravatà E, Tamburrini G, Gaudino S, Pettorini B, Novegno F, Colosimo C, Di RC. Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery. 2011;68(4):950–6.

    Article  PubMed  Google Scholar 

  23. Valentini LG, Selvaggio G, Visintini S, Erbetta A, Scaioli V, Solero CL. Tethered cord: natural history, surgical outcome and risk for Chiari malformation 1 (CM1). Neurol Sci. 2011;32(3):353–6.

    Article  PubMed Central  Google Scholar 

  24. Canheu AC, Santos MV, Furlanetti LL, Salomão JFM, de Oliveira RS. The Brazilian Society for Pediatric Neurosurgery: consensus on Chiari I deformity. Childs Nerv Syst. 2020;36(1):17–8.

    Article  PubMed  Google Scholar 

  25. Milano JB, Barcelos ACES, Onishi FJ, et al. The effect of filum terminale sectioning for Chiari 1 malformation treatment: systematic review. Neurol Sci. 2020;41(2):249–56.

    Article  PubMed  Google Scholar 

  26. Chai Z, Xue X, Fan H, Sun L, Cai H, Ma Y, Ma C, Zhou R. Efficacy of posterior fossa decompression with duraplasty for patients with Chiari malformation type I: a systematic review and meta-analysis. World Neurosurg. 2018;113:357–365.e1.

    Article  PubMed  Google Scholar 

  27. Durham SR, Fjeld-Olenec K. 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. 2008;2(1):42–9.

    Article  PubMed  Google Scholar 

  28. Förander P, Sjåvik K, Solheim O, Riphagen I, Gulati S, Salvesen Ø, Jakola AS. The case for duraplasty in adults undergoing posterior fossa decompression for Chiari I malformation: a systematic review and meta-analysis of observational studies. Clin Neurol Neurosurg. 2014;125:58–64.

    Article  PubMed  Google Scholar 

  29. Hankinson T, Tubbs RS, Wellons JC. Duraplasty or not? An evidence-based review of the pediatric Chiari I malformation. Childs Nerv Syst. 2011;27(1):35–40.

    Article  PubMed  Google Scholar 

  30. Lu VM, Phan K, Crowley SP, Daniels DJ. 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. 2017;20(5):439–49.

    Article  PubMed  Google Scholar 

  31. Xu H, Chu LY, He R, Ge C, Lei T. Posterior fossa decompression with and without duraplasty for the treatment of Chiari malformation type I—a systematic review and meta-analysis. Neurosurg Rev. 2017;40(2):213–21.

    Article  PubMed  Google Scholar 

  32. Zhao JL, Li MH, Wang CL, Meng W. A systematic review of Chiari I malformation: techniques and outcomes. World Neurosurg. 2016;88:7–14.

    Article  PubMed  Google Scholar 

  33. Jiang E, Sha S, Yuan X, Zhu W, Jiang J, Ni H, Liu Z, Qiu Y, Zhu Z. 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. 2018;110:e465–72.

    Article  PubMed  Google Scholar 

  34. Goel A. Basilar invagination, syringomyelia and Chiari formation and their relationship with atlantoaxial instability. Neurol India. 2018;66(4):940–2.

    Article  PubMed  Google Scholar 

  35. Goel A, Kaswa A, Shah A. Atlantoaxial fixation for treatment of Chiari formation and syringomyelia with no craniovertebral bone anomaly: report of an experience with 57 cases. Acta Neurochir Suppl. 2019;125:101–10.

    Article  PubMed  Google Scholar 

  36. Deora H, Behari S, Sardhara J, Singh S, Srivastava AK. Is cervical stabilization for all cases of Chiari-I malformation an overkill? Evidence speaks louder than words! Neurospine. 2019;16(2):195–206.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Salunke P, Karthigeyan M, Malik P, Panchal C. Changing perception but unaltered reality: how effective is C1-C2 fixation for Chiari malformations without instability? World Neurosurg. 2020;136:e234–44.

    Article  PubMed  Google Scholar 

  38. Wagner A, Grassner L, Kögl N, Hartmann S, Thomé C, Wostrack M, Meyer B. Chiari malformation type I and basilar invagination originating from atlantoaxial instability: a literature review and critical analysis. Acta Neurochir. 2020;162(7):1553–63.

    Article  PubMed  Google Scholar 

  39. Ho WSC, Brockmeyer DL. Complex Chiari malformation: using craniovertebral junction metrics to guide treatment. Childs Nerv Syst. 2019;35(10):1847–51.

    Article  PubMed  Google Scholar 

  40. Holly LT, Batzdorf U. Management of cerebellar ptosis following craniovertebral decompression for Chiari I malformation. J Neurosurg. 2001;94(1):21–6.

    Article  CAS  PubMed  Google Scholar 

  41. Riew KD, Raich AL, Dettori JR, Heller JG. Neck pain following cervical laminoplasty: does preservation of the C2 muscle attachments and/or C7 matter? Evid Based Spine Care J. 2013;4(1):42–53.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Tatter C, Fletcher-Sandersjöö A, Persson O, Burström G, Grane P, Edström E, Elmi-Terander A. Incidence and predictors of kyphotic deformity following resection of cervical intradural tumors in adults: a population-based cohort study. Acta Neurochir. 2020;162(11):2905–13.

    Article  PubMed  Google Scholar 

  43. Guan J, Riva-Cambrin J, Brockmeyer DL. Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients. Neurosurg Focus. 2016;41(5):E2.

    Article  PubMed  Google Scholar 

  44. Heidary M, Respondek M, Klekamp J. Histological and intraoperative evaluations of the arachnoid in patients with Chiari I malformation. Acta Neurochir. 2021;163(1):219–25.

    Article  PubMed  Google Scholar 

  45. Dlouhy BJ, Dawson JD, Menezes AH. Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia. J Neurosurg Pediatr. 2017;20(6):526–41.

    Article  PubMed  Google Scholar 

  46. Kurzbuch AR, Jayamohan J, Magdum S. Decompressive surgery for Chiari I malformation in children without dural repair: a still effective and safe procedure? Childs Nerv Syst. 2019;35(10):1839–46.

    Article  PubMed  Google Scholar 

  47. Greenberg JK, Milner E, Yarbrough CK, Lipsey K, Piccirillo JF, Smyth MD, Park TS, Limbrick DD. Outcome methods used in clinical studies of Chiari malformation type I: a systematic review. J Neurosurg. 2015;122(2):262–72.

    Article  PubMed  Google Scholar 

  48. Chotai S, Chan EW, Ladner TR, Hale AT, Gannon SR, Shannon CN, Bonfield CM, Naftel RP, Wellons JC. Timing of syrinx reduction and stabilization after posterior fossa decompression for pediatric Chiari malformation type I. J Neurosurg Pediatr. 2020;26(2):193–9.

    Article  PubMed  Google Scholar 

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Acknowledgment

The authors want to thank The Jury of the Chiari & Syringomyelia Consensus Conference, Milan, November 11-13, 2019: Barbanera Andrea, Bolognese Paolo, Brodbelt Andrew, Ciaramitaro Palma, Caldarelli Massimo, Canheu Alexandre, Celada Carlo, Chieffo Daniela, Cinalli Giuselle, Cocito Dario, Curone Marcella, Devigili Grazia, Di Rocco Federico, Erbetta Alessandra, Farinotti Mariangela, Ferraris Marilena, Garbossa Diego, Gilanton Mado, Giordano Flavio, Furlanetto Marika, Jallo George, Karadjova Marieta, Klekamp Jörg, James Syril, Morar Sylvia, Parker Fabrice, Lanteri Paola, Messing Junger Martina, Parazzini Cecilia, Paternoster Giovanna, Piatelli Gianluca, Perrini Paolo, Poca Mariantonia, Prabahkar Prab, Righini Andrea, Ricci Federica, Sahuquillo Juan, Sala Francesco, Stoodley Marcus, Talamonti Giuseppe, Thompson Dominic, Triulzi Fabio, Valentini Maria Consuelo, Zucchelli Mino, and the Patients’ Association for their participation and support to the 2019 Consensus Conference (in alphabetical order): AICRA for Craniosynostosis (Italy), AISMAC (Italy), APAISER (France), ASAP (USA), Bobby Jones Chiari & Syringomyelia Foundation’s (USA), Deutsche Syringomyelie und Chiari Malformation (Germany), FEMACPA (Spain), National Syringomyelia Association (Bulgaria), and SACA (Ireland).

Disclosure Statement

The authors have no conflicts of interest to declare.

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Authors and Affiliations

  1. Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS – Università Cattolica del Sacro Cuore, Rome, Italy

    Luca Massimi

  2. Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Ignazio Gaspare Vetrano & Laura Grazia Valentini

  3. Pediatric Neurosurgery, AOU Città della Salute e della Scienza di Torino, Turin, Italy

    Paola Peretta

  4. Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Luisa Chiapparini

  5. Developmental Neurology Unit, Mariani Foundation Center for Complex Disabilities, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Veronica Saletti

  6. CRESSC, Clinical Neurophysiology, Department of Neuroscience, AOU Citta’ della Salute e della Scienza di Torino, Turin, Italy

    Palma Ciaramitaro

  7. Institute of Neurosurgery, Catholic University of Rome, Rome, Italy

    Massimiliano Visocchi

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  1. Luca Massimi
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Correspondence to Laura Grazia Valentini .

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    Massimiliano Visocchi

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Massimi, L. et al. (2023). Chiari Malformation Type 1 and Syringomyelia: Why Do Patients Claim for International Guidelines? Commentary on the 2021 Chiari and Syringomyelia Consensus Document. In: Visocchi, M. (eds) The Funnel: From the Skull Base to the Sacrum. Acta Neurochirurgica Supplement, vol 135. Springer, Cham. https://doi.org/10.1007/978-3-031-36084-8_23

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  • DOI: https://doi.org/10.1007/978-3-031-36084-8_23

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