Home > Encyclopedia of Genetic Disorders > Dandy-Walker malformation

Definition

Dandy-Walker malformation is a congenital (present at birth) condition involving several abnormalities in the development of the brain. The malformation appears to result from destructive processes, such as inflammation or trauma, which block the circulation of cerebrospinal fluid (CSF) inside the head after the brain has been formed in the embryo.

Description

Dandy-Walker malformation was first described in 1914 by Drs. Dandy and Blackfan. The disorder typically includes the following abnormalities in brain structure:

• Absence or incomplete formation of the vermis, the middle portion of the cerebellum, which is the part of the human brain that lies behind the two cerebral hemispheres.
• Enlargement of the fourth ventricle, one of the human brain's four...