Copyright © 2017 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. An Algorithm for Managing Intraosseous Vascular Anomalies of the Craniofacial Skeleton Kathryn V. Isaac, MD, FRCSC,  Tara Lynn Teshima, MD, MSc, y Richard I. Aviv, MBChB, z§ Mahmood Fazl, MD, FRCSC, jj Leodante da Costa, MD, FRCSC, jj Todd Mainprize, MD, FRCSC, jj and Oleh Antonyshyn, MD, FRCSC ô Background: Intraosseous vascular anomalies (IOVA) are rare in the craniofacial skeleton and present a diagnostic and therapeutic challenge. This study aims to describe the clinical management based on a large case series. Methods: A retrospective chart review was performed and 9 IOVA were identified over a 15-year period. Data on demographics, diagnostic features, clinical management, and outcomes were reviewed. Results: Five frontal bone IOVA and 4 orbital IOVA were identified. The postoperative follow-up ranged from 4 months to 4 years. All 9 lesions were diagnosed with computed tomography (CT) imaging. Magnetic resonance imaging (MRI) was used to delineate soft tissue involvement in 2 patients presenting with oculo-orbital dystopia and ophthalmoplegia. En bloc excision was performed in all patients. Preoperative interventional embolization was critical in the successful resection of an orbital IOVA following 2 previously failed attempts that were aborted secondary to hemorrhage. Intraoperative 3-dimensional stereotactic navigation was used for the accurate en bloc excision of a frontal IOVA to prevent injury to the frontal sinus. Reconstruction of esthetic and functional deformities was successfully accomplished. Conclusion: The diagnosis of IOVA relies primarily on clinical assessment and CT imaging. Further interpretation of the involvement of periorbital, facial, and intracranial soft tissue is best defined by MRI. Multidisciplinary care with interventional radiology and neurosurgery must be considered for ensuring the safe and adequate en bloc excision of craniofacial IOVA. Key Words: Bone tumor, craniofacial tumor, intraosseous hemangioma, intraosseous vascular anomaly (J Craniofac Surg 2017;00: 00–00) I ntraosseous vascular anomalies (IOVA) are benign, slow-grow- ing vascular lesions that occur within bone and exhibit either vascular dysmorphogenesis (malformation) or vascular prolifera- tion (tumor). 1 Previously, IOVA were incorrectly referred to as ‘‘intraosseous hemangiomas’’ to describe both vascular malforma- tions and tumors located within a bone structure. Since the devel- opment of guidelines set out by the International Society for the Study of Vascular Anomalies, the term ‘‘hemangioma’’ should be reserved for the true endothelial tumor present since infancy. 1–5 Although the true incidence of IOVA is difficult to determine given the historical use of variable terminology, IOVA are considered rare and constitute approximately 1% of bone neoplasms. 3,6 The most common locations of IOVA are the vertebral column and calvarium, with infrequent involvement of the facial skeleton. 7–10 Intraosseous vascular anomalies of the craniofacial region typi- cally present as a slow-growing firm bony mass. Signs and symp- toms include contour deformities, facial asymmetry, headaches, and/or visual disturbances. 3,5,10–12 The diagnosis is challenging because of its rarity, nonspecific clinical presentation, and a lack of exclusive findings on diagnostic imaging. 6,13–25 Radiologic and pathologic investigations are typically performed even though the accuracy of different imaging modalities and the need for biopsies are unclear. 6,15–25 The management of craniofacial IOVA remains controversial with poorly defined indications for excision, preop- erative embolization, and involvement of a multidisciplinary team. This study describes an algorithm for the clinical management of craniofacial IOVA based on our cumulative experience with 9 patients with craniofacial IOVA. MATERIALS AND METHODS A retrospective review of our database was done using computer- generated search for patients diagnosed with IOVA of the skull and craniofacial skeleton. Of the 30 patients identified and reviewed, 9 IOVA were confirmed over a 15-year period. Data on demo- graphics, clinical course, surgical management, and outcome were obtained from medical charts. Review of the pathology specimens and radiologic investigations was conducted. RESULTS In our study population, IOVA were located in the frontal bone (n ¼ 5), the supraorbital rim (n ¼ 2), and the orbito-zygomatic complex (n ¼ 2) (Table 1). All patients presented with nontender contour deformities. Location-specific symptoms included pain in 1 patient with a frontal IOVA, diplopia and/or ophthalmoplegia in 3 patients with orbital IOVA. All patients with orbital IOVA demonstrated vertical oculo-orbital dystopia and in 1 patient asso- ciated with proptosis. Time from the initial presentation to time of excision ranged from 6 months to 3 years. Biopsies had been performed at referring institutions prior to our assessment in 2 patients with orbito-zygomatic masses. One biopsy revealed a ‘‘cavernous hemangioma’’ and the second biopsy was nondiagnostic. No biopsies were performed at our institution in the From the  Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Toronto, Toronto; y Division of Plastic and Reconstructive Surgery, Markham Stouffville Hospital, Markham; z Department of Medical Imaging, University of Toronto, Toronto; § Division of Neuroradiology, Sunnybrook Health Sciences Centre; jj Division of Neurosurgery, Sunnybrook Health Sciences Centre; and ô Division of Plastic and Reconstructive Surgery, Sunnybrook Health Sciences Centre, Toronto, Canada. Received March 7, 2017. Accepted for publication June 9, 2017. Address correspondence and reprint requests to Kathryn V. Isaac, MD, FRCSC, Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Toronto, M1 502 Sunnybrook Health Sciences Centre, Toronto, Ontario M4N 3M5, Canada; E-mail: kathryn.isaac@mail.utoronto.ca The authors report no conflicts of interest. Copyright # 2017 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000004014 ORIGINAL ARTICLE The Journal of Craniofacial Surgery  Volume 00, Number 00, Month 2017 1