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Natural history of Chiari type I malformation in children
Luca Massimi1, Massimo Caldarelli, Paolo Frassanito
1Department of Neurosciences, Pediatric Neurosurgery, A. Gemelli Hospital, Rome, Italy. lmassimi@email.it
Insights
Asymptomatic children with Chiari type I malformation (CIM) often have a favorable natural outcome. Conservative management is suggested for these cases, as surgery is rarely needed.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Developmental Pediatrics
Background:
- Chiari type I malformation (CIM) diagnosis in asymptomatic children presents a management dilemma due to limited natural history data.
- Deciding on surgical intervention for pediatric CIM is challenging and debated.
Purpose of the Study:
- To evaluate the natural evolution of incidentally diagnosed asymptomatic Chiari type I malformation in children.
- To inform clinical decision-making regarding the management of pediatric CIM.
Main Methods:
- Retrospective analysis of 16 asymptomatic children with incidentally diagnosed CIM.
- Mean age at diagnosis: 6.7 years; mean follow-up duration: 5.8 years.
- Assessment of clinical symptoms and radiological changes over the follow-up period.
Main Results:
- No patients required suboccipital decompression surgery.
- 13 children remained asymptomatic with stable or improved radiological findings.
- 3 children developed symptoms; 1 managed conservatively, 2 underwent endoscopic third ventriculostomy for hydrocephalus.
Conclusions:
- Asymptomatic Chiari type I malformation in children generally shows a favorable natural course.
- Conservative management appears appropriate for asymptomatic pediatric CIM.
- Multicenter studies are needed to confirm these findings and guide treatment protocols.
Abstract:
The decision on whether or not to operate children with Chiari type I malformation (CIM) is difficult and controversial, because of the lack of information about the natural evolution of such a disease. Herein, we report on the evolution of 16 asymptomatic children with incidentally diagnosed CIM (mean age: 6.7 years; mean follow-up: 5.8 years). No patients required suboccipital decompression. Thirteen children remained asymptomatic, with stable or improved radiological picture (worsening in 2 cases). Three cases showed appearance of symptoms: one did not require any treatment; the remaining two underwent endoscopic third ventriculostomy because of hydrocephalus, which is a possible consequence of CIM. This analysis shows a favorable natural outcome of CIM in children, thus suggesting a conservative management in asymptomatic cases. However, multicentric studies are required to validate this data.
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