Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis.

The metamerically associated normal hindbrain and normal posterior fossa are programmed to grow together in such a way that the tonsils are located above the foramen magnum and surrounded by the cerebrospinal fluid (CSF) of the cisterna magna. This allows the pulsating CSF to move freely up and down across the craniovertebral junction (CVJ). A developmental mismatch between the rates of growth of the neural tissue and of the bony posterior fossa may result in the cerebellar tonsils being dislocated across the foramen magnum. The cause of this may be, rarely, an overgrowth of the cerebellum. More commonly, it is due to an insufficient development of the posterior fossa, possibly associated with a malformation of the craniocervical joint. When it is not due to a remediable cause, such a herniation is called a Chiari 1 deformity. This definition is anatomic (descent of the tonsils below the plane of the foramen magnum) and not clinical: many patients with the deformity are and will remain asymptomatic. Most authors consider that a descent of 5 mm or more is clinically significant but other factors, such as the diameter of the foramen magnum and the degree of tapering of the upper cervical "funnel," are likely to be as important. Morphologic markers of severity on magnetic resonance imaging are, beside the degree of descent, the peg-like deformity of the tonsils, the obstruction of the surrounding CSF spaces (at the craniocervical junction and in the whole posterior fossa), a compression of the cord, an abnormal signal of the cord, and a syringomyelia, typically cervicothoracic. The syringomyelia is assumed to be explained by the "Venturi effect" that is associated with the increased velocity of the CSF across the restricted CSF spaces. Radiologically, the etiopathogenic assessment should address the size and morphology of the posterior fossa, and the functional status of the craniocervical flexion joint. The posterior fossa is best evaluated on sagittal cuts by the posterior fossa pentagon proportionality associated with the line of Chamberlain, and on coronal cuts, by showing a possible shallowness of the posterior fossa. The functional status of the craniocervical joint is altered in case of a proatlantal hypoplasia, as this condition results in a cranial shift of the joint that brings the tip of the dens and of the flexion axis in front of the medulla, that is, in a situation of osteoneural conflict. Less commonly, similar conflicts may also occur when an abnormal craniocervical segmentation results in an instability of the joint.