Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery
David Benglis1, Derek Covington, Ritwik Bhatia
1Department of Neurosurgery, University of Miami/Miller School of Medicine and Miami Children's Hospital, Miami, Florida 33155, USA.
Insights
Most pediatric patients with Chiari malformation Type I (CM-I) show no clinical or radiological progression when managed without surgery. Further long-term follow-up is needed to confirm these outcomes.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Medicine
Background:
- Chiari malformation Type I (CM-I) is a structural defect in the cerebellum.
- The natural history of untreated CM-I in pediatric populations is not well-established.
- Understanding outcomes without surgical intervention is crucial for clinical management.
Purpose of the Study:
- To investigate the clinical and radiological outcomes of pediatric patients with CM-I.
- To evaluate the natural history of CM-I in patients managed non-surgically.
- To determine if CM-I progresses in asymptomatic or mildly symptomatic children.
Main Methods:
- Retrospective review of 124 pediatric CM-I cases (July 1999-July 2008).
- Patients were followed without surgical intervention, with a mean follow-up of 2.83 years.
- Data collected included imaging findings, symptoms, and neurological examination results.
Main Results:
- The mean tonsillar herniation was 8.35 mm; syrinx size remained stable in 7 patients.
- No new syrinxes or neurological deficits were observed during follow-up.
- Of 81 symptomatic patients, 5 required surgical recommendation; 43 patients were asymptomatic at diagnosis.
Conclusions:
- The majority of pediatric CM-I patients managed non-surgically exhibit stable clinical and radiological status.
- Long-term observation is necessary to fully understand the potential for delayed symptom development or progression.
- Non-operative management appears safe for many pediatric CM-I cases, pending further data.
Object:
The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention.
Methods:
The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up.
Results:
The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5-22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up.
Conclusions:
The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.
