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Pediatric Chiari I malformations

Do clinical and radiologic features correlate?

From the Departments of Neurology (Drs. Wu, Barkovich, and Ferriero), Pediatrics (Drs. Wu, Barkovich, and Ferriero), Radiology (Drs. Chin and Barkovich), and Neurosurgery (Dr. Barkovich), University of California at San Francisco, CA; and the Department of Radiology (Dr. Chan), Kaiser Permanente, Oakland, CA.

Address correspondence and reprint requests to Dr. Yvonne W. Wu, Department of Neurology, University of California at San Francisco, Box 0114, San Francisco, CA 94143-0114.

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.

Key words: Brain malformation—Herniation—Headache—Syringomyelia.