Pitx2, is of interest, as it is a direct transcriptional target of canonical Wnt signaling and because heterozygous loss of function PITX2 mutations in humans results in Riegers‐Axenfeld syndrome which can include VBW anomalies ranging from exomphalos to excessive umbilical skin.228, 267, 320. This evidence concerns the gene PITX2 and Axenfeld-Rieger syndrome.