The Chiari I malformation, once thought to be a disorder of clinical importance only in the teen years and beyond, is now recognized as being significant in the pediatric population as well. We have reviewed 7 cases of Chiari I malformation in children less than 12 years of age. Hydrosyringomyelia was often, but not invariably, an accompanying feature, and this in turn was associated with scoliosis and motor weakness. All patients had posterior fossa and cervical decompression with dural grafting procedures, as well as various types of syringosubarachnoid shunts placed. Intraoperative ultrasound was found to be a valuable surgical adjunct. Symptoms and signs were improved or stabilized in all patients. We feel that this disorder is now more commonly reported in young children because of the increased availability of noninvasive neuroimaging techniques, especially magnetic resonance imaging. Questions are raised as to the actual incidence of undiscovered cases of this disorder in children with commonly seen conditions such as scoliosis.

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