It is important to note, however, that mutations in genes encoding ADAM metallopeptidase with thrombospondin type 1 motif 3 (ADAMTS3) and the atypical cadherin FAT4 have also been associated with Hennekam’s syndrome, thus highlighting the complex pathophysiology underlying the development of primary lymphatic disorders [56,57]. This evidence concerns the gene ADAMTS3 and Hennekam syndrome.