Vascular bone syndrome – angio-osteodystrophy: current concepts

Congenital vascular bone syndrome (CVBS) is an abnormal enhancement or reduction of growth in long bones due to pathologic circulation during childhood. Several authors have described these clinical pictures with limb lengthening; well known are Klippel and Trenaunay and Parkes-Weber. Later, Servelle and Martorell also described cases of limb length difference, but with shortening of the pathologic limb. The mechanism of limb overgrowth is probably due to the effect of A-V shunts, while shortening occurs due to mechanical compression on bones by dysplastic vessels or flow reduction. Some molecules, like vascular endothelial growth factor and others, probably affect bone growth through a poorly understood mechanism. Diagnostically, one should try to demonstrate A-V shunts around or inside the bone or low flow vascular mass. Correction of length differences can occur spontaneously if the cause of CVBS is treated in childhood. In adults, limb length differences may be corrected by orthopaedic techniques.