Vascular bone syndrome – angio-osteodystrophy: current concepts

Abstract

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.

Diagnosis and management of extensive vascular malformations of the lower limb

“…7 Bone involvement is so frequent in patients with extensive vascular malformations that some authors include it under the term congenital vascular bone syndrome (CVBS; ie, a pathologic enhancement or reduction in long bone growth related to congenital abnormal circulation). 8 temperature, and augmented oxygenation in the bone caused by arteriovenous fistulae may stimulate the osteoblasts to produce bone overgrowth. 9 However, some authors concluded that the cause of CVBS is venostasis rather than hyperemia after obtaining elongation of long bones in animals by ligation of deep veins, 10 while bone hypotrophy has been related to a mechanical compression on bones by dilated vascular malformations.…”

Cervical dural arteriovenous malformation and large epidural venous varices in a rare adult presentation of congenital vascular bone syndrome

“…Each of the original authors presented cases with inexact characteristics and often anecdotal exceptions. Klippel and Trenaunay acknowledged "incomplete" cases without one of the three signs [6]. Subsequent researcher have "published cases of Klippel-Trenaunay syndrome with a-v fistulas, cases of Parkes Weber syndrome without fistulas,… [and] cases of Klippel-Trenaunay syndrome with shortening of the limb" [7].…”

Extracranial arteriovenous malformations in children: features of clinical course, diagnosis and treatment

“…Окрім оцінки пер-винного вогнища АВМ, необхідно обстежити органи і системи, які можуть вторинно уражатися, зокрема опорно-руховий апарат. Рання діагностика судинно-кісткового синдрому, який супроводжується різницею в довжині довгих кісток, є необхідною умовою своєчас-ного й патогенетично обґрунтованого лікування [20]. Базові неінвазивні та мінімально інвазивні ме-тодики, які слід виконати в першу чергу при підозрі на АВМ, включають: УЗД у режимі КДС і Доппле-ра; дуплексну ультрасонографію (артеріальна і ве-нозна); МРТ; КТ-ангіографію з контрастуванням, 3D-реконструкцією.…”