Review
Arteriovenous malformations of the head and neck: current concepts in management

https://doi.org/10.1016/j.bjoms.2016.01.034Get rights and content

Abstract

The field of vascular anomalies and their clinical management is expanding and developments in the last few years have improved the understanding of haemangiomas and vascular malformations. In this paper, the first in the series of 3 educational reviews, we review the current trends and evidence-based management of arteriovenous malformations.

Introduction

Use of the classification by Mulliken and Glowacki has improved the care of patients with vascular malformations,1 which are rare, mainly high-flow lesions, comprising a complex network of primitive vessels with interconnecting feeding arteries and draining veins. These lesions are the most dangerous type of vascular malformation and considered the most challenging to manage. Recurrence rates of over 80% after embolisation and surgical resection have been reported,2 although recent advances and improved multidisciplinary approaches to care suggest this can be much improved upon in the future.

The traditional capillary network between the arteries and veins is usually lacking or bypassed in arteriovascular malformations. Direct shunts cause a lack of normal down-regulation in pressure and have the potential to increase and recruit new vessels. The central section of the abnormal connection and the first section of the adjoining dilated vein termed the nidus.

Section snippets

Classification

Around half of all arteriovenous malformations affect the head and neck3 and a slight female predominance (1:1.5) has been reported.4 There are several methods of clinical and radiological classification and historically, lesions have been described as focal or diffuse.5 Focal lesions have a single artery and well-defined nidus. Diffuse lesions have multiple feeding vessels, they cross and usually destroy tissue boundaries and are more difficult to treat successfully without recurrence.5 The

Natural history

Although these lesions are congenital, they commonly remain dormant during childhood and increase in size during adolescence and adulthood.2 About 80% are detected during childhood8 and all will ultimately progress.2 Spontaneous regression is almost never seen.9 The exact aetiological and pathological factors involved in their development remain uncertain, although they are thought to result from an error in vascular development during embryogenesis.2 Hormonal changes during puberty and

Clinical features

During childhood, often termed the quiescent or dormant phase, lesions can present as small vascular stains, and they become increasingly pulsatile and overgrown with soft tissue as they progress.9 Many of the clinical features at this time are attributable to the chronic haemodynamic effects of hypervascularity and venous hypertension. Typical manifestations include pain, tissue expansion and destruction, ulceration, disfigurement, and bleeding (Fig. 1). They are also warm and often pulsatile.

Imaging and diagnosis

Diagnosis is often made after clinical examination and recognition of the hallmark features of erythema, warmth, pulsatility, and thrill. Ultrasound, magnetic resonance imaging (MRI), and angiography are used to investigate the morphology and to plan treatment. Lesions have multiple vascular channels on ultrasonography, and Doppler studies show high flow and loss of normal venous damping.15, 16 Their size, shape, and association with neighbouring tissue show well on MRI and MR angiography (Fig.

Treatment

Treatment is challenging, and an interdisciplinary approach and extensive clinical experience are recommended.9 It aims to control shunting and to palliate the clinical manifestations. When available, endovascular approaches are currently considered the first line of treatment, and are often combined with resection of focal lesions or those with a well-localised nidus. Unfortunately, diffuse lesions are less easy to treat, and multiple staged procedures are often done to prevent excessive

Embolisation

Traditionally, treatment has aimed to obstruct all arterial feeders but it results in early revascularisation, and inevitably in a more complex vascular supply. When resection is impossible, this approach can make subsequent embolisation more challenging because more niduses form, and the vascular structure becomes more complex. It is now known that the lesion is better controlled when the nidus is obliterated, and efforts are now being directed at embolic materials that can safely obstruct it.

Operation

Early operation has been recommended to limit the destruction caused by the continuous growth of lesions.9 Complete resection should be the aim,18 and partial excision avoided where possible. Well-localised lesions without previous embolisation are therefore ideal,19 as they are likely to have smaller feeder vessels and well-defined borders. They also have a better chance of cure, and there is less likelihood of intraoperative haemorrhage.9

Diffuse lesions present a more challenging problem.

Re-expansion and recurrence

The risk of re-expansion and recurrence after intervention is thought to be similar to factors that result in the progressive expansion of a lesion, and it is well known that embolic and surgical treatments can stimulate recurrence through the creation of a proangiogenic environment.4, 23, 24 Embolisation leads to local hypoxia and increased levels of VEGF, MMP-2, and MMP-9.25, 26 Operation and healing also induce local hypoxia and inflammation, which are known stimulants of angiogenesis

New treatments

To date, pharmacological approaches have had limited success, and antiangiogenic drugs have been targeted as potential treatments. Marimastat is an antiangiogenic MMP inhibitor, initially developed for the treatment of metastatic breast cancer,29 but trials were abandoned because of poor outcomes. However, it has been used successfully in the management of a progressive lesion in the limb of a child.30 To our knowledge, no other clinical use has been described.

The treatment of hereditary

Conclusion

Multidisciplinary treatment is required to achieve an optimal outcome in the management of arteriovenous malformations. Preoperative intravascular embolisation with excision is recognised as the treatment of choice, and earlier intervention is now deemed desirable. Life-threatening complications should be anticipated during intervention, particularly resection. Given the difficulty in establishing surgical margins and the need for wide resection, it is important to remember to avoid a treatment

Conflict of Interest

We have no conflicts of interest.

Ethics statement/confirmation of patient permission

We confirm written consent for publication has been given by the patient described.

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