Approximately 67% of human conceptuses die prenatally. Of these, a sig
nificant number involve a disorder of the lymphatic system. A small nu
mber of live-born children also exhibit congenital lymphatic malformat
ions, including an estimated 60% of patients with Turner syndrome. The
se observations have prompted a search for the genetic and dysmorpholo
gic basis and the different patterns of congenital lymphedema and asso
ciated anomalies. In this article, we attempt to summarize available p
ertinent information on congenital disorders of the lymphatic system a
nd to propose a conceptual overview of lymphatic development.