Pediatric Chiari Malformation Management: WFNS Spine Committee Recommendations.

Abstract

A systematic literature review and consensus using Delphi method. This review aims to provide an overview on Chiari malformation in pediatric patients, highlighting the specific clinical manifestations and surgical treatment options. Chiari malformation in children presents a real difficulty to the general neurosurgeon because children are not smaller adults. In the absence of pediatric neurosurgeons, as in many countries of the world, a need for education of general neurosurgeons on the management of Chiari malformation in children was identified. The authors carried out an extensive review of the literature in PubMed database of the last 10 years addressing the topic of Chiari malformation in children. A total of 64 studies were selected for analysis and five statements were drawn to be voted by a panel of expert spine surgeons in two consensus meetings organized by the World Federation of Neurosurgical Societies (WFNS) Spine Committee. A consensus was reached using the Delphi method. In children with CM1, a decompressive surgery with duraplasty before puberty may avoid scoliosis progression. In Chiari type 2, the recommendation is to perform urgently extensive decompression of the craniovertebral junction and cervical canal if there is no decompensation of hydrocephalus. Cranial vault expansion may be recommended in pediatric Chiari malformation associated with craniosynostosis. Children with Chiari type I can play sports because of the low risk of worsening. Pediatric age is a modifier for the management of Chiari malformation (CM). Prompt diagnosis and appropriate decompressive surgery with duraplasty before puberty are essential to mitigate the impact of the condition on the child's well-being. Increased awareness among health care professionals, timely access to specialized expertise in neurosurgical interventions are crucial, especially for type 2 CM patients that require urgent decompression of CVJ and cervical spine.