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Surgical management of Chiari II malformation: a systematic review of literature

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Abstract

Purpose

Chiari II malformation (CM-II) is a congenital malformation of the posterior fossa associated with myelomeningocele. Of the symptomatic patients, 10–33% require surgical treatment. To this date, there is not a consensus about the best surgical technique, and whether to do duroplasty.

Methods

A literature search of the PubMed database and crossed references was performed, per PRISMA guidelines. Data regarding demographic features, extent of cervicomedullary deformity, clinical presentation, surgical techniques, and clinical outcomes were extracted. Pearson’s chi-squared test was applied. The p-values under 0.05 were considered statistically significant.

Results

Twenty studies (N = 330) were analyzed. C3 and C4 levels represented 56.4% of the lowest tonsil displacement. The most reported symptom was dysphagia/swallowing dysfunction (53.8%). Suboccipital craniectomy (SOC) and cervical spine expansion (CSE) with duroplasty were the most reported technique. Dural augmentation was performed in 57.4% of the patients. After surgery, 59.6% observed an improvement in symptoms and quality of life, 12.5% were unchanged, and 27.8% had a worsened clinical status. The mortality rate was 2.5% during the first month after surgery, and 17.4% at the last follow-up evaluation. Patients who underwent CSE presented a better clinical outcome (p = 0.002). The SOC procedure could not be correlated with symptom improvement (p = 0.06).

Conclusion

CM-II is associated with high morbidity and mortality. An early onset symptomatic CM-II demands intervention, which provided an improvement of outcome in most patients included in this review. The best surgical technique and the exact effect of the surgical management of CM-II on mortality are not yet clear.

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Data availability

No datasets were generated or analyzed during the current study.

References

  1. Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-gadol AA (2015) The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28(2):184–194. https://doi.org/10.1002/ca.22442

    Article  PubMed  Google Scholar 

  2. Azahraa Haddad F, Qaisi I, Joudeh N, Dajani H, Jumah F, Elmashala A et al (2018) The newer classifications of the Chiari malformations with clarifications: an anatomical review. Clin Anat 31(3):314–322. https://doi.org/10.1002/ca.23051

    Article  PubMed  Google Scholar 

  3. Talamonti G, Marcati E, Mastino L, Meccariello G, Picano M, D’Aliberti G (2020) Surgical management of Chiari malformation type II. Childs Nerv Syst 36(8):1621–1634. https://doi.org/10.1007/s00381-020-04675-7

    Article  PubMed  Google Scholar 

  4. Kim I, Hopson B, Aban I, Rizk EB, Dias MS, Bowman R et al (2018) Decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry. J Neurosurg Pediatr 22(6):652–658. https://doi.org/10.3171/2018.5.PEDS18160

    Article  PubMed  PubMed Central  Google Scholar 

  5. Akbari SHA, Limbrick DD, Kim DH et al (2013) Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. https://doi.org/10.1007/s00381-013-2040-9

    Article  PubMed  Google Scholar 

  6. McDowell MM, Blatt JE, Deibert CP, Zwagerman NT, Tempel ZJ, Greene S (2018) Predictors of mortality in children with myelomeningocele and symptomatic Chiari type II malformation. J Neurosurg Pediatr. https://doi.org/10.3171/2018.1.PEDS17496

    Article  PubMed  Google Scholar 

  7. Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, Shamseer L, Tetzlaff JM, Akl EA, Brennan SE, Chou R, Glanville J, Grimshaw JM, Hróbjartsson A, Lalu MM, Li T, Loder EW, Mayo-Wilson E, McDonald S, McGuinness LA, Stewart LA, Thomas J, Tricco AC, Welch VA, Whiting P, Moher D (2021) The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ 372:n71

    Article  PubMed  PubMed Central  Google Scholar 

  8. Ogiwara H, Morota N (2013) Surgical decompression without dural opening for symptomatic Chiari type II malformation in young infants. Childs Nerv Syst. https://doi.org/10.1007/s00381-013-2116-6

    Article  PubMed  Google Scholar 

  9. Vandertop WP, Asai A, Hoffman HJ et al (1992) Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg. https://doi.org/10.3171/jns.1992.77.4.0541

    Article  PubMed  Google Scholar 

  10. Venes JL, Black KL, Latack JT (1986) Preoperative evaluation and surgical management of the Arnold-Chiari II malformation. J Neurosurg. https://doi.org/10.3171/jns.1986.64.3.0363

    Article  PubMed  Google Scholar 

  11. Jang M, Biggs P, North L, Foy A, Chun R (2018) Management and outcomes of pediatric vocal cord paresis in Chiari malformation. Int J Pediatr Otorhinolaryngol. https://doi.org/10.1016/j.ijporl.2018.09.014

    Article  PubMed  Google Scholar 

  12. Jenkinson MD, Campbell S, Hayhurst C et al (2011) Cognitive and functional outcome in spina bifida–Chiari II malformation. Childs Nerv Syst. https://doi.org/10.1007/s00381-010-1368-7

    Article  PubMed  Google Scholar 

  13. Yundt KD, Park TS, Tantuwaya VS, Kaufman BA (1996) Posterior fossa decompression without duraplasty in infants and young children for treatment of Chiari malformation and achondroplasia. Pediatr Neurosurg. https://doi.org/10.1159/000121129

    Article  PubMed  Google Scholar 

  14. Kellogg R, Lee P, Deibert CP et al (2018) Twenty years’ experience with myelomeningocele management at a single institution: lessons learned. J Neurosurg Pediatr. https://doi.org/10.3171/2018.5.PEDS17584

    Article  PubMed  Google Scholar 

  15. Hudgins RJ, Boydston WR (1995) Bone regrowth and recurrence of symptoms following decompression in the infant with Chiari II malformation. Pediatr Neurosurg. https://doi.org/10.1159/000120979

    Article  PubMed  Google Scholar 

  16. Ocal E, Irwin B, Cochrane D, Singhal A, Steinbok P (2012) Stridor at birth predicts poor outcome in neonates with myelomeningocele. Childs Nerv Syst. https://doi.org/10.1007/s00381-011-1585-8

    Article  PubMed  Google Scholar 

  17. Teo C, Parker EC, Aureli S, Boop FA (1997) The Chiari II malformation: a surgical series. Pediatr Neurosurg. https://doi.org/10.1159/000121258

    Article  PubMed  Google Scholar 

  18. 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. Neurosurg. https://doi.org/10.1227/00006123-199604000-00015

    Article  Google Scholar 

  19. Aronson 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. https://doi.org/10.2106/00004623-199173060-00012

    Article  PubMed  Google Scholar 

  20. Charney EB, Rorke LB, Sutton LN, Schut L (1987) Management of Chiari II complications in infants with myelomeningocele. J Pediatr. https://doi.org/10.1016/S0022-3476(87)80455-9

    Article  PubMed  Google Scholar 

  21. Yamada H, Tanaka Y, Nakamura S (1985) Laryngeal stridor associated with the chiari II malformation. Childs Nerv Syst. https://doi.org/10.1007/BF00270814

    Article  PubMed  Google Scholar 

  22. 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. https://doi.org/10.3171/jns.1992.77.6.0881

    Article  PubMed  Google Scholar 

  23. Jones RF, Ayer JG, Stening W (1996) Hydromyelia and Chiari malformation in children and adolescents. J Clin Neurosci. https://doi.org/10.1016/S0967-5868(96)90081-8

    Article  PubMed  Google Scholar 

  24. Narayan P, Mapstone TB, Tubbs RS, Grabb PA, Frye T (2001) Clinical significance of cervicomedullary deformity in Chiari II malformation. Pediatr Neurosurg. https://doi.org/10.1159/000050408

    Article  PubMed  Google Scholar 

  25. Stevenson K (2004) Chiari Type II malformation: past, present, and future. Neurosurg Focus 16(2):E5. https://doi.org/10.3171/foc.2004.16.2.6

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Medicine, Pontifical Catholic University of Goiás, Goiânia, Brazil

    Rafael C. C. Vencio & Renata C. F. Bonfim

  2. Department of Neurological Surgery, Children’s Hospital, Goiânia, Brazil

    Nayara M. Pereira, Cilmária L. Franco, Paulo Ronaldo J. Ribeiro & Bárbara A. Morais

  3. Department of Neurological Surgery, Clinics Hospital, School of Medicine, Federal University of Goiás, Goiânia, Brazil

    Adriano M. Lino-Filho & Paulo Ronaldo J. Ribeiro

Authors
  1. Rafael C. C. Vencio
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  3. Renata C. F. Bonfim
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  4. Nayara M. Pereira
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  5. Cilmária L. Franco
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Contributions

This study was idealized and designed by B.A.M. B.A.M, R.C.C.V, and R.C.F.B wrote the main manuscript. All the data was collected by R.C.C.V and R.C.F.B. The statistical analysis was performed by B.A.M. All authors contributed to write and review the manuscript.

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Correspondence to Bárbara A. Morais.

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Vencio, R.C.C., Lino-Filho, A.M., Bonfim, R.C.F. et al. Surgical management of Chiari II malformation: a systematic review of literature. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06368-x

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