Transfontanelle ultrasonography shows a large anechoic cystic structure in the posterior fossa. The cerebellar hemispheres are absent.
Non contrast enhanced CT scan of the head shows dilatation of both the lateral ventricles, third and forth ventricles with Evans index of 0.44. There is expansion of the posterior fossa with a large posterior fossa cyst communicating with the fourth ventricle. The cerebellar vermis is hypoplastic.
The malformation was first described by English surgeon John Bland-Sutton in 1887 and later in the 1900s by neurosurgeons Walter Dandy, MD, and Arthur Walker, MD.. Its current name was given by German psychiatrist Clemens Ernst Benda in 1954.
The Evans index is named after William A. Evans Jr., who first proposed this measurement in 1942