Congenital Arteriovenous Anomalies of the Limbs

Angiodysplastic Syndrome with Capillary and Venous Malformation Associated with Soft Tissue Hypotrophy

“…Dependent on the type of vessels and the extent of the mal formation involved, increase as well as decrease in the dependent tissue is therefore possible [3,6], In some cases a multifactorial inheritance has been suggested as an addi tional etiologic factor [23], A prerequisite for the management and prognostic judgement of vascular malformations is a clinically appli cable nomenclature and classification. In the past, an array of descriptive terms and incorrectly combined eponyms such as Klippel-Trenaunay-Parkes-Weber syndrome have been created [1,5,24], Several comprehensive proposals of classifications have been made [25][26][27], but they have all been subject to controversy and were not generally ac cepted. Because there is insufficient knowledge to classify vascular malformations based on etiology or pathogenesis alone, the best basis is to categorize them according to the primary vascular defect [2], Criteria such as the type of blood vessel involved, the presence of a truncular or an extratruncular angiodysplasia and high or low blood flow velocity, supplemented by the secondary disorders such as the ectatic veins and the associated tissue and bone changes have been the basis for the Hamburg classification 1988 (table 2) [28].…”

Changing Concept on Vascular Malformation: No Longer Enigma

“…4,36) But it soon became clear that most of these self-inflicted wounds were due to their wrong approach especially to 'surgically inaccessible' lesions. Now, based on much improved assessment of its extent and severity including its relationship with surrounding tissues/systems, the decision for the treatment as well as the selection of the treatment modality can be made quite accurately.…”

“…This once condemned and abandoned disease 4) is now newly defined as congenital vascular malformation (CVM) and this new terminology began to clear much confusion on this unique vascular disorder as a universal language replacing many nosologies.…”

Major Complex Pelvic Arteriovenous Malformation in a Patient with Down Syndrome

“…However, AVM lesions are considered to be undifferentiated vascular structures resulting from arrest of embryonic development at various stages. The vessels are blocked during maturation, with either a gross differentiation for aneurysms or a blockage during a previous retiform stage for fistula [3]. Communication (macro-and micro-communications) between the arterial and venous systems produce massive venous and tissue engorgement.…”