Pediatric Chiari I malformations

BACKGROUND: Although Chiari I malformation is increasingly recognized in children, little is known about its clinical presentation in this age group. OBJECTIVE: To evaluate the relationship between clinical and MRI features of pediatric Chiari I malformations. METHODS: We performed a chart review and MRI analysis of 49 children with Chiari I malformation. The degree of tonsillar ectopia was compared with age at onset, presence of syringomyelia, and a neurologic severity score as follows: asymptomatic = 0, symptomatic with normal neurologic examination = 1, and symptomatic with abnormal examination = 2. RESULTS: Age at onset of symptoms ranged from 10 months to 14 years. Fifty-seven percent of patients were asymptomatic. Headache and neck pain were the most frequent complaints. Syringomyelia was detected in 14% of patients and skull base abnormalities in 50%. The magnitude of tonsillar ectopia (5 to 23 mm) correlated with severity score (p = 0.04) but not with other clinical measures. CONCLUSIONS: The clinical symptoms of Chiari I malformations in children are nearly identical to those seen in adults. Children with greater amounts of tonsillar ectopia on MRI are more likely to be symptomatic.