Management opinions from different centers (Rio de Janeiro)
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.
KeywordsChiari 1 malformation Hydrosyryngomyelia Craniovertebral decompression
Compliance with ethical standards
Conflict of interest
The authors declare no conflict of interest.
- 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. https://doi.org/10.1016/j.pediatrneurol.2009.01.003 CrossRefPubMedPubMedCentralGoogle Scholar
- 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. https://doi.org/10.1016/j.pediatrneurol.2013.04.016 CrossRefGoogle Scholar
- 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. https://doi.org/10.1007/s00586-015-4011-1 CrossRefPubMedGoogle Scholar
- 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. https://doi.org/10.1007/s00381-014-2424-5 CrossRefPubMedPubMedCentralGoogle Scholar
- 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. https://doi.org/10.1097/SCS.0000000000003926 CrossRefPubMedGoogle Scholar
- 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. https://doi.org/10.1007/s00381-016-3300-2 CrossRefPubMedGoogle Scholar