Conclusion: A correct and universally accepted terminology based on the biologic behavior of orbital vascular anomalies is essential to avoid misleading diagnoses, to choose an adequate therapy regimen, and to compare different therapeutic approaches. The management of these diseases is multi-modal and an interdisciplinary challenge.
Background: There is persisting terminological and clinical confusion about vascular anomalies involving the orbit. Review of the literature reveals a diverse or misleading terminology and a lack of consensus for the treatment of vascular malformations or vascular tumors of the orbit.
Methods: This study comprised a detailed analysis of cases presenting with orbital vascular anomalies followed by an extensive review of the literature.
Results: Thirty-six patients were analyzed, among them 19 patients with peri- and/or intraorbital hemangiomas, 3 with intraorbital and 2 with periorbital venous malformations, 2 with orbital involvement of complex vascular malformations, and 10 with lymphatic malformations involving the orbit.