Arnold-Chiari Malformation (Postmortem study of 6 cases)

Abstract

A total of 6 autopsy cases of Arnold-Chiari malformation was examined at the Department of Pathology of Seoul National University. These cases showed characteristic features of Chiari type II malformation, i.e., displacement of a portion of the inferior vermis, whole medulla oblongata and the fourth ventricle into the cervical canal and associated cerebellar dysplasia. All of the cases had hydrocephalus and accompaning meningomyelocele at various levels. All cases showed polygyria but not micropolygyria, and in three cases there were craniolacunia. Three out of 6 cases showed severe malformations of the extraneural system. These malformations included bilateral renal agenesis, exstrophia splanchnica, cardiovascular and skeletal abnormalities, etc. On the basis of our observations we think that multifactorial causes are involved in the pathogenesis of the Arnold-Chiari malformation.