Neurointerventional Management of High-Flow Vascular Malformations of the Head and Neck
Section snippets
Incidence and clinical presentation
The pathogenesis of vascular malformations is complex and incompletely understood. Postulated mechanisms have been summarized in several recent reviews.2, 3 Rather than representing neoplasms, these lesions result from errors in vascular development.
HFVMs of the head and neck are quite rare and their true incidence is unknown. There may be a slightly higher prevalence in girls, with at least one series reporting a female-to-male ratio of 1.5:1.5 The lesions are likely present at birth, but many
Diagnosis
The diagnosis of a high-flow vascular malformation can be reliably made based on clinical history and physical exam. On exam, the high-flow lesions are usually reddish, warm, firm, and pulsatile (Fig. 1). Local skin ischemia, ulceration, and/or hemorrhage are frequently seen with more advanced lesions (Fig. 2). As described elsewhere in this issue, these are not typical features for low-flow lesions,1 and as such, the diagnosis is usually not dependent upon imaging results.
Cross-sectional
Therapeutic goal and endovascular management
Although lethal complications such as massive hemorrhage do occur, HFVMs of the head and neck are usually not life-threatening. Given the inherently trans-spatial nature of these lesions, the therapeutic goal for most patients with complex lesions is symptomatic control, preservation of vital functions (eg, vision, hearing, or mastication), or aesthetic restoration, rather than a complete “cure.” Treatment options may depend on the site, size, and complexity of the lesion, as well as the
Endovascular embolization with Onyx
The use of ethylene-vinyl alcohol copolymer (Onyx, EV3 Neurovascular, Irvine, CA) as an embolic agent for AVMs was first reported in the early 1990s in Japan.14, 15 It has since become commercially available as a nonadhesive liquid embolic agent under the trade name of Onyx, and is approved by the Food and Drug Administration for use in embolizing brain AVMs.
The availability of Onyx has brought about a sea-change in the embolization of central nervous system AVMs.16, 17, 18 In contact with
High-flow vascular malformations at specific sites in the head and neck
As mentioned previously, HFVMs of the head and neck favor several primary sites, occurring most commonly in the cheek (31%), followed by the ear (16%) and nose (11%).5 Anatomic and functional challenges posed by particular locations in the head and neck impart a level of complexity to the management of these AVMs that is often lacking at other sites in the body. Here we consider several head and neck sites where HFVMs present unique treatment challenges.
Syndromes associated with arteriovenous malformations of the head and neck
Although rare, HFVMs of the head and neck are associated with several nonhereditary and hereditary conditions. The following are selected examples.
Summary
Head and neck high-flow vascular malformations are uncommon lesions whose management presents a clinical challenge. Although in some rare cases a complete cure is possible, in the vast majority the primary objective is symptom control, cosmesis improvement, and preservation of vital functions. Striving for “complete cure” in most cases results in potentially devastating clinical and cosmetic outcome. Collateral supply via intracranial vessels is not uncommon, and scrupulous efforts to avoid
Acknowledgments
The authors express their profound indebtedness to the physicians and staff of the Vascular Anomalies Center at Children's Hospital Boston, headed by Drs. John B. Mulliken and Stephen J. Fishman, and to Dr. Patricia E. Burrows, who was involved with earlier treatment of several of the patients discussed here. Additionally, the authors thank the staff of the Interventional Radiology Division at Children's Hospital Boston and the Neurointerventional Service at Brigham and Women's Hospital.
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