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Pediatric Chiari Malformation Management: WFNS Spine Committee Recommendations
Ricardo Gepp1, June Ho Lee2, Jutty Parthiban3
1Department of Neurosurgery, Sarah Network of Hospitals, Brasília, Brazil.
Insights
Prompt diagnosis and surgical intervention for Chiari malformation in children are crucial. Early decompressive surgery with duraplasty before puberty can prevent scoliosis progression and improve outcomes.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Spine Surgery
Background:
- Chiari malformation (CM) in children poses unique management challenges distinct from adult cases.
- A global need exists for educating general neurosurgeons on pediatric CM due to a scarcity of pediatric specialists.
Purpose of the Study:
- To provide an overview of Chiari malformation in pediatric patients.
- To highlight specific clinical manifestations and surgical treatment options for pediatric CM.
Main Methods:
- A systematic literature review of PubMed articles from the last 10 years.
- Consensus reached via the Delphi method among expert spine surgeons from the World Federation of Neurosurgical Societies (WFNS) Spine Committee.
Main Results:
- Decompressive surgery with duraplasty before puberty may prevent scoliosis progression in CM1.
- Urgent craniovertebral junction decompression is recommended for Chiari type 2 without hydrocephalus decompensation.
- Cranial vault expansion may be considered for pediatric CM associated with craniosynostosis.
Conclusions:
- Pediatric age significantly influences Chiari malformation management strategies.
- Timely diagnosis and surgical intervention, particularly before puberty, are vital for optimal pediatric outcomes.
- Enhanced awareness and access to specialized neurosurgical care are critical, especially for Type 2 CM.
Study Design:
A systematic literature review and consensus using Delphi method.
Objective:
This review aims to provide an overview on Chiari malformation in pediatric patients, highlighting the specific clinical manifestations and surgical treatment options.
Summary Of Background:
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.
Material And Methods:
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.
Results:
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.
Conclusion:
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.
