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AAP Grand Rounds 9:18-19 (2003) The Chiari Malformation, Type 1, in Early ChildhoodSource: Greenlee JDW, Donovan KA, Hasan DM, et al. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics. 2002;110:12121219.
The Chiari malformation type 1 (CM1) is defined as abnormal protrusion of the tonsils of the cerebellum through the foramen magnum down into the cervical spinal canal. The quantitative threshold for diagnosis of CM1 is somewhat arbitrary, but the most widely accepted definition is descent of the tonsils >5 mm below the plane of the foramen.1 The developmental lesion responsible for CM1 is believed, in most instances, to be insufficient growth of the osseous boundaries of the posterior cranial fossa: there is simply not enough room within the posterior fossa to accommodate the neural structures that belong there.2,3 Symptoms and neurological signs may develop either on the basis of mechanical compression of the brainstem at the foramen magnum or on the basis of disturbance of cerebrospinal fluid (CSF) dynamics, leading to syringomyelia.3,4 The potential clinical manifestations of CM1 are legion.
In the current era of widely
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