Synopsis

In this brief presentation, we review how both embryological and environmental factors can create a diverse spectrum of malformations involving the posterior fossa of the brain. We first review entities where the neuroimaging phenotyping is strong and well evolved, matching these to the clinical phenotype as well as genetic bases (where known); We then look at some novel entities with distinct phenotypes, as well as new mechanistic insights and philosophies into existing conditions. Associated supratentorial malformations are discussed and described where relevant. In addition, we review the role of diffusion tensor imaging (DTI) in entities where the technique adds value.