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Updated: May 29, 2026
Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Treatment for Chiari 1 malformation (CIM): analysis of a pediatric surgical series
Laura Valentini1, Sergio Visintini, Veronica Saletti
1Department of Neurosurgery, Fondazione Istituto Neurologico C. Besta, Via Celoria 11, 20133, Milan, Italy. lvalentini@istituto-besta.it
Insights
Chiari malformation (CM) in children often presents with unique symptoms like syncope and paraparesis. Surgical outcomes, particularly for associated syringomyelia, show significant improvement in pediatric patients.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Craniofacial Surgery
Background:
- Chiari malformation (CM) presents distinct neurological symptoms in children, including syncope and acute paraparesis.
- Previous studies indicate varied outcomes for CM treatment in pediatric populations.
- Associated conditions like nonsyndromic craniosynostosis and syringomyelia are frequently observed in pediatric CM cases.
Purpose of the Study:
- To evaluate the surgical outcomes and clinical presentation of Chiari malformation in a pediatric cohort.
- To compare the efficacy of surgical interventions for CM in children versus adults.
- To assess the impact of surgical treatment on associated conditions such as syringomyelia and tethered cord.
Main Methods:
- Retrospective analysis of 99 pediatric patients operated for Chiari malformation (CM) at FINCB.
- Evaluation of surgical morbidity, mortality, and preoperative symptom improvement.
- Assessment of outcomes for associated conditions including syringomyelia, craniosynostosis, and tethered cord.
Main Results:
- No major surgical morbidity or mortality was observed in the pediatric cohort.
- Preoperative symptoms showed greater improvement in children compared to adults, potentially due to shorter symptom duration and greater plasticity.
- Tonsillar resection led to unexpected psychiatric symptom improvement in some CM cases; syringomyelia reduced in over 80% of children, with significant disappearance in many.
- Associated tethered cord (5%) required combined surgical treatment (detethering and addressing tonsillar descent).
Conclusions:
- Surgical intervention for Chiari malformation in children is safe, with significant symptom resolution, especially for associated syringomyelia.
- Pediatric patients generally experience better surgical outcomes than adults, likely due to physiological factors.
- Comprehensive surgical planning is crucial for cases involving complex associated conditions like tethered cord.
Abstract:
Children may present a peculiar picture of CIM, as syncopes and acute paraparesis. In a series of 99 children operated for CIM at FINCB there were no major surgical morbidity nor mortality. The preoperative symptoms improved more in this pediatric series than in the adult cases treated at the same institution in the same period; a reason could be the shorter duration of symptoms and another the children plasticity. An untreated nonsyndromic craniosynostosis was present in 10 cases. In our hands, the results of the limited extradural decompression were poor. In some CIM associated with psychiatric symptoms an unexpected improvement was observed after tonsilar resection. The associated Syringomyelia reduced in more than 80% of children and disappeared in a significant number. The rare associated tethered cord (5%) needed a double treatment, detethering by itself being insufficient to treat also tonsillar descent. The clinical symptoms are often more serious in children than in the adults, but the results of surgery, especially on the syrinx, are better.