Vascular Malformations and Associated Syndromes The Role of the Orthopaedic Surgeon

Vascular anomalies are among the most common congenital abnormalities in infants and children. The management of vascular anomalies requires interdisciplinary care and collaboration between orthopaedic surgeons, general surgeons, plastic surgeons, interventional radiologists, dermatologists, oncologists, and child life specialists, leading to the establishment of vascular anomaly teams at tertiary centers. Vascular anomalies are a heterogeneous group of conditions, with similar clinical appearance but substantially different manifestations. The development of an appropriate classification system has helped to reduce confusion regarding terminology, thus improving diagnosis and management. There are two groups of vascular anomalies: (1) vascular tumors and (2) vascular malformations. Differentiating between these two types, and their subtypes, is essential because of their distinct natural history and treatment1. The goal of this review is to highlight the role of the orthopaedic surgeon in the approach and treatment of extremity vascular malformations and to discuss diagnostic strategies and management at a multidisciplinary level. We also review the most common associated syndromes seen in the orthopaedic practice, including Klippel-Trenaunay syndrome, Proteus syndrome, Maffucci syndrome, Gorham-Stout disease or syndrome, Parkes Weber syndrome, and Bannayan-Riley-Ruvalcaba syndrome. In 1863, Virchow was the first to classify vascular anomalies into groups on the basis of their histopathologic appearance2. However, this nomenclature was confusing and often led to misdiagnosis with consequent inappropriate treatment. Mulliken and Glowacki proposed a classification system based on biological behavior …