Ectopia lentis (EL) is a condition that can either herald underlying systemic 
conditions, or be isolated. The recent expansion in the genetics of these 
conditions has furthered the understanding of the underlying molecular 
aetiology. It is becoming apparent that novel genes, and in particular the 
ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) family, 
are important in ocular development. The common link in these genes seems to be 
EL. The clinical management of EL is challenging. In particular, the options for 
addressing surgically induced aphakia in the context of an ectopic capsule are 
varied. Little evidence exists to direct management of these issues. This review 
summarises the molecular pathogenesis of EL and conditions associated with it, 
using the genetic aetiology as a framework. Furthermore, it summarises some of 
the issues involved in its clinical management.