Child's Nervous System

, Volume 35, Issue 10, pp 1889–1894 | Cite as

Management opinions from different centers (Rio de Janeiro)

  • José Francisco M. SalomãoEmail author
  • Tatiana Protzenko Cervante
  • Antonio Rosa Bellas
Special Annual Issue



The surgical treatment of Chiari type 1 (CM1) malformation is controversial and depends largely on the preference of the surgeon. The evolution of neuroimaging resulted in an increased number of asymptomatic patients incidentally diagnosed.


To study retrospectively a population of 24 symptomatic patients with CM1 operated between 1999 and 2017 in which intraoperative ultrasonography (IOUS)–assisted posterior fossa-C1 decompression was used to decide whether the dura mater should be opened (CVD+) or not (CVD).


Most of the patients complained of headache or neck pain, 15 had hydrosyringomyelia and 14 had some spinal cord involvement. Patients were categorized in improved, unchanged, or worse according the preoperative signs and symptoms. Overall, 19 patients improved, 3 deteriorated, and 2 remained unchanged. Among these, 4 out 5 had syringohydromyelia.


IOUS-assisted posterior fossa-C1 decompression is our preferred option to treat CM1. Children submitted to intradural procedures, initially or subsequently, had increased postoperative complications. CSF fistula or pseudomeningocele was the major cause of complication. The final result seems to correlate with the preoperative neurological status.


Chiari 1 malformation Hydrosyryngomyelia Craniovertebral decompression 


Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest.


  1. 1.
    Krieger MD, Mccomb JG, Ml L (1999) Toward a simpler surgical management of Chiari I malformation in a pediatric population. Pediatr Neurosurg 30:113–121. CrossRefPubMedGoogle Scholar
  2. 2.
    Galarza M, Martínez-Lage JF, Ham S, Sood S (2010) Cerebral anomalies and Chiari type 1 malformation. Pediatr Neurosurg 46:442–449. CrossRefPubMedGoogle Scholar
  3. 3.
    Aitken LA, Linda CE, Sidney S, Gupta N, Barkovich AJ, Sorel M et al (2009) Chiari type I malformation in a pediatric population. Pediatr Neurol 40:449–454. CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Goel A (2015) Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine 22:116–127. CrossRefGoogle Scholar
  5. 5.
    Poretti A, Ashmawy AR, Garzon-Muvdi AT, Jallo GI, Huisman TA, Raybaud C (2016) Chiari type 1 deformity in children : pathogenetic , clinical , neuroimaging , and management aspects. Neuropediatrics 47:293–307. CrossRefPubMedGoogle Scholar
  6. 6.
    Azahraa Haddad F, Qaisi I, Joudeh N, Dajani H, Jumah F, Elmashala A, Adeeb N, Chern JJ, Tubbs RS (2018) The newer classifications of the chiari malformations with clarifications: an anatomical review. Clin Anat 31:314–322. CrossRefPubMedGoogle Scholar
  7. 7.
    International Headache Society (2018) The international classification of headache disorders, 3rd edition. Cephalalgia 38(1):1–211. CrossRefGoogle Scholar
  8. 8.
    Pindrik J, Johnston JM (2015) Clinical presentation of Chiari I malformation and syringomyelia in children. Neurosurg Clin N Am 26:509–514. CrossRefPubMedGoogle Scholar
  9. 9.
    Mehta A, Chilakamarri P, Zubair A, Kuruvilla D (2018) Chiari headache. Curr Pain Headache Rep 22(49):49. CrossRefPubMedGoogle Scholar
  10. 10.
    Zaffanello M, Sala F, Sacchetto L, Gasperi E, Piacentini G, Sala F (2017) Evaluation of the central sleep apnea in asymptomatic children with Chiari 1 malformation : an open question. Childs Nerv Syst 33:829–832. CrossRefPubMedGoogle Scholar
  11. 11.
    Losurdo A, Testani E, Scarano E, Massimi L, Della MG (2013) What causes sleep-disordered breathing in Chiari I malformation ? Comment on: “MRI findings and sleep apnea in children with Chiari I malformation”. Pediatr Neurol 49:11–13. CrossRefGoogle Scholar
  12. 12.
    Milhorat TH (2003) Tailored operative technique for Chiari type I. Neurosurgery 53:899–906CrossRefGoogle Scholar
  13. 13.
    Godzik J, Dardas A, Kelly MP, Holekamp TF, Lenke LG, Smyth MD, Park TS, Leonard JR, Limbrick DD (2016) Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort study. Eur Spine J 25:619–626. CrossRefPubMedGoogle Scholar
  14. 14.
    Eule JM, Erickson MA, Brien MFO, 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 27:1451–1455CrossRefPubMedGoogle Scholar
  15. 15.
    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 20:349–356. CrossRefPubMedGoogle Scholar
  16. 16.
    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 30:1419–1424. CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Brockmeyer DL, Spader HS (2015) Complex Chiari malformations in children. Diagnosis and Management Neurosurg Clin N Am 26:555–560. CrossRefPubMedGoogle Scholar
  18. 18.
    Entezami P, Gooch MR, Poggi J, Perloff E, Dupin M, Adamo MA (2019) Current management of pediatric chiari type 1 malformations. Clin Neurol Neurosurg 176:122–126. CrossRefPubMedGoogle Scholar
  19. 19.
    Macinante GL (2000) Chiari type I anomalies in children and adolescents : minimally invasive management in a series of 53 cases. Childs Nerv Syst 16:707–718. CrossRefPubMedGoogle Scholar
  20. 20.
    Massimi L, Caldarelli M (2011) Natural history of Chiari type I malformation in children. Neurol Sci 32:275–277. CrossRefGoogle Scholar
  21. 21.
    Schijman E (2004) Steinbok P. International survey on the management of Chiari I malformation and syringomyelia 2004:341–348. CrossRefGoogle Scholar
  22. 22.
    Nishizawa S, Yokoyama T, Yokota N, Tokuyama T, Ohta S (2001) Incidentally identified syringomyelia associated with Chiari I malformations: is early interventional surgery necessary? Neurosurgery 49:637–640; discussion 640-1PubMedGoogle Scholar
  23. 23.
    Sala F, Squintani G, Tramontano V, Coppola A, Gerosa M (2011) Intraoperative neurophysiological monitoring during surgery for Chiari malformations. Neurol Sci 32(Suppl 3):317–319. CrossRefGoogle Scholar
  24. 24.
    Kawasaki Y, Uchida S, Onishi K, Toyokuni M, Okanari K, Fujiki M (2017) Intraoperative neurophysiologic monitoring for prediction of postoperative neurological improvement in a child with Chiari type I malformation. J Craniofac Surg 28:1837–1841. CrossRefPubMedGoogle Scholar
  25. 25.
    Barzilai O, Roth J, Korn A, Constantini S (2016) The value of multimodality intraoperative neurophysiological monitoring in treating pediatric Chiari malformation type I. Acta Neurochir 158:335–340. CrossRefPubMedGoogle Scholar
  26. 26.
    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. CrossRefPubMedGoogle Scholar
  27. 27.
    Gallo P, Sokol D, Kaliaperumal C, Kandasamy J (2017) Comparison of three different cranio-cervical decompression procedures in children with Chiari malformation type I: does the surgical technique matter? Pediatr Neurosurg 52:289–297. CrossRefPubMedGoogle Scholar
  28. 28.
    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 31:1371–1376. CrossRefPubMedGoogle Scholar
  29. 29.
    Pisapia JM, Merkow MB, Brewington D, Henn RE, Sutton LN, Storm PB et al (2017) External validity of the Chiari severity index and outcomes among pediatric Chiari I patients treated with intra- or extra-dural decompression. Childs Nerv Syst 33:313–320. CrossRefPubMedGoogle Scholar
  30. 30.
    Klekamp J (2015) Chiari I malformation with and without basilar invagination: a comparative study. Neurosurg Focus 38(4):E12. CrossRefGoogle Scholar
  31. 31.
    Menezes AH, Vogel TW (2008) Specific entities affecting the craniocervical region: syndromes affecting the craniocervical junction. Childs Nerv Syst 24:1155–1163. CrossRefPubMedGoogle Scholar
  32. 32.
    Valentini L, Visintini S, Saletti V, Solero CL (2011) Treatment for Chiari 1 malformation ( CIM ): analysis of a pediatric surgical series. Neurol Sci 32(Suppl 3):321–324. CrossRefGoogle Scholar
  33. 33.
    Yarbrough CK, Greenberg JK, Park TS (2015) Clinical outcome measures in Chiari I malformation. Neurosurg Clin N Am 26:533–541. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Pediatric Neurosurgery Division, Fernandes Figueira InstituteOswaldo Cruz Foundation (IFF-Fiocruz)Rio de JaneiroBrazil

Personalised recommendations