Chapter 2 Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis

Publication date: 2018 Source:Handbook of Clinical Neurology, Volume 155 Author(s): Charles Raybaud, George I. Jallo 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 to...
Source: Handbook of Clinical Neurology - Category: Neurology Source Type: research