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Natural history of Chiari I malformation in children: a retrospective analysis
Matthew Carey1, William Fuell1, Thomas Harkey1
1Division of Neurosurgery, Arkansas Children's Hospital, 1 Children's Way, Slot 838, Little Rock, AR, 72202, USA.
Insights
The natural history of pediatric Chiari I malformation (CM1) is generally benign when managed non-operatively. Most patients do not progress, and if they do, it typically occurs within two years without predictable factors.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Medical Research
Background:
- Chiari I malformation (CM1) is increasingly studied in children, but research on its natural history with conservative management is limited.
- Existing studies primarily focus on surgical interventions for CM1, including indications, techniques, and outcomes.
Purpose of the Study:
- To determine the surgical intervention rates for pediatric CM1 patients after initial consultation and delayed presentation.
- To investigate the natural history of CM1 in children managed non-operatively.
- To identify patient factors that may predict symptom development or worsening in pediatric CM1.
Main Methods:
- Retrospective analysis of 465 pediatric CM1 patients, with 226 meeting inclusion criteria for initial consultation between July 2011 and June 2016.
- Examination of surgical intervention rates, types of surgery, patient demographics (age, gender), and clinical presentation (headache, syrinx).
- Detailed review of patients experiencing new or worsening symptoms that prompted delayed surgical intervention.
Main Results:
- 15% of pediatric CM1 patients underwent surgery, predominantly Chiari decompression, with over half occurring within six months of consultation.
- Among patients with delayed surgery, only 5 (4 with new symptoms/syrinx, 1 with symptom progression) showed progression; other delays were for non-clinical reasons.
- No identifiable patient factors predicted symptom progression or development in those managed conservatively.
Conclusions:
- The natural history of asymptomatic pediatric Chiari I malformation is favorable with non-operative management.
- Progression of symptoms or syrinx in non-operatively managed pediatric CM1 is uncommon and likely to occur within two years of initial consultation.
- This study did not identify specific predictors for symptom development or worsening in pediatric CM1 patients managed conservatively.
Purpose:
While there are increasing numbers of studies published regarding Chiari I malformation (CM1) in children, most of these focus on surgical indications, technique, and outcomes. Few studies examine the natural history of CM1 once the decision is made to treat a patient conservatively. In this study, we seek to determine the percentage of pediatric patients who undergo surgery for CM1, both after initial consultation and in a delayed fashion, the natural history of CM1 after a decision to pursue non-operative management, and attempt to identify patient factors that may predict development of new or worsening CM1 symptoms.
Methods:
From our database of 465 pediatric patients with CM1, we identified those who were seen for initial consultation from July 1, 2011, to June 30, 2016. We examined rates of surgical intervention, types of surgical intervention, age, gender, and presence or absence of headache and syrinx, and looked carefully at the patients who had new or worsening symptoms prompting delayed surgical intervention.
Results:
We identified 226 patients meeting inclusion criteria. Overall, 15% of patients had surgery, the majority being Chiari decompression. Just over half of these patients had surgery within 6 months of initial consultation. Of those with delayed surgery, only 4 patients had new symptoms/syrinx and 1 patient had symptom progression. The other patients had various reasons for surgical delay not related to symptom development or progression. There were no obvious commonalities among these 5 patients that could predict progression prospectively. All patients who had surgery did so within 2 years of initial consultation.
Conclusion:
Overall, the natural history of asymptomatic CM1 is benign. Patients treated non-operatively are unlikely to progress. If they do progress, this is likely to occur within 2 years of initial consultation. There were no factors identified in this study that predicted new or worsening symptoms over time.
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