ABSTRACT

Vascular bone syndrome (VBS) in venous malformations (VMs) is a limb length discrepancy, due to overgrowth or growth reduction of a limb because of an effect of local vascular anomalies. Also, molecules with both angiogenic and osteogenic activity, like vascular endothelial growth factor (VEGF), fibroblast growth factor (FGF), transforming growth factor (TGF), bone morphogenetic proteins (BMP), and others, and also some gene mutations like PIC3CA may be involved in the phenomenon. Treatment is based on vascular procedures, like surgical removal or sclerosis of VM and orthopedic procedures to stop bone growing (epiphysiodesis) or bone elongation (Ilizarov technique), or surgical bone shortening (osteotomy). In childhood, period of bone growth, vascular techniques, and orthopedic methods can be combined, preferring the first, if limb length discrepancy is not excessive (less than 4 cm) or combining both if the difference is more important.