A 34-year-old patient presented with a right orbito-facial mass since childhood, consistent with a congenital arteriovenous (AV) malformation. Prior to presentation, she had multiple incomplete surgical resections and embolizations with N-butyl acetyl acrylate and Onyx-18. The patient reported gradual, progressive vision loss shortly after Onyx-18 embolization. Five months after embolization, she presented with decreased vision, disfigurement and mechanical ptosis relating to a large subcutaneous mass affecting the medial right upper eyelid and forehead. Significant exam findings included a visual acuity of 20/400 (20/60 prior to embolization), an afferent pupillary defect, and optic disc pallor. MRI and angiography revealed a persistent AV malformation with feeders from the ophthalmic artery and an absent choroidal flush to the right eye. Pathology from surgical resection showed a significant foreign body giant cell reaction to the embolization material adjacent to the vessels. We suggest that an incomplete embolization with Onyx-18 may have caused vascular steal syndrome from the ophthalmic artery.

1.
Lin CJ, Blanc R, Clarencon F, Piotin M, Spelle L, Williams M, Moret J: Transvenous embolization of an intraorbital arteriovenous fistula using Onyx. J Clin Neurosci 2010;17:783-785.
2.
Trivelatto F, Nakiri GS, Manisor M, Riva R, Al-Khawaldeh M, Kessler I, Mounayer C: Preoperative onyx embolization of meningiomas fed by the ophthalmic artery: a case series. AJNR Am J Neuroradiol 2011;32:1762-1766.
3.
Warrier S, Prabhakaran VC, Valenzuela A, Sullivan TJ, Davis G, Selva D: Orbital arteriovenous malformations. Arch Ophthalmol 2008;126:1669-1675.
4.
Rootman J, Heran MK, Graeb DA: Vascular malformations of the orbit: classification and the role of imaging in diagnosis and treatment strategies*. Ophthal Plast Reconstr Surg 2014;30:91-104.
5.
Krol A, MacArthur CJ: Congenital hemangiomas: rapidly involuting and noninvoluting congenital hemangiomas. Arch Facial Plast Surg 2005;7:307-311.
6.
Clarencon F, Blanc R, Lin CJ, Mounayer C, Galatoire O, Morax S, Moret J, Piotin M: Combined endovascular and surgical approach for the treatment of palpebral arteriovenous malformations: experience of a single center. AJNR Am J Neuroradiol 2012;33:148-153.
7.
Kohout MP, Hansen M, Pribaz JJ, Mulliken JB: Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg 1998;102:643-654.
8.
Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE: Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg 2001;107:1647-1654.
9.
Trombly R, Sandberg DI, Wolfe SA, Ragheb J: High-flow orbital arteriovenous malformation in a child: current management and options. J Craniofac Surg 2006;17:779-782.
10.
Andracchi S, Kupersmith MJ, Nelson PK, Slakter JS, Setton A, Berenstein A: Visual loss from arterial steal in patients with maxillofacial arteriovenous malformation. Ophthalmology 2000;107:730-736.
11.
de Keizer R: Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit 2003;22:121-142.
12.
Lv X, Li Y, Yang X, Jiang C, Wu Z: Endovascular management of direct carotid-cavernous sinus fistulas. Neuroradiol J 2012;25:130-134.
14.
Natarajan SK, Born D, Ghodke B, Britz GW, Sekhar LN: Histopathological changes in brain arteriovenous malformations after embolization using Onyx or N-butyl cyanoacrylate. Laboratory investigation. J Neurosurg 2009;111:105-113.
15.
Byrne JV, Hope JK, Hubbard N, Morris JH: The nature of thrombosis induced by platinum and tungsten coils in saccular aneurysms. AJNR Am J Neuroradiol 1997;18:29-33.
16.
Killer M, Arthur AS, Barr JD, Richling B, Cruise GM: Histomorphology of thrombus organization, neointima formation, and foreign body response in retrieved human aneurysms treated with hydrocoil devices. J Biomed Mater Res B Appl Biomater 2010;94:486-492.
17.
Turner T, Trobe JD, Deveikis JP: Sequential branch retinal artery occlusions following embolization of an intracranial meningioma. Arch Ophthalmol 2002;120:857-860.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.