Surgical Treatment of the Giant Congenital Craniofacial Arteriovenous Malformation: A Case Report
Article Outline
Abstract
We report a case of a 35-year-old man who presented with a giant congenital craniofacial arteriovenous malformation. The angiogram showed an arteriovenous malformation in the left temporo fronto orbital region. Excision of the arteriovenous malformation was performed with fronto temporal approach. Postoperatively, the patient is completely asymptomatic.
Keywords: Arteriovenous malformation, Craniofacial
Any abnormal communication between an artery and a vein is commonly referred to as arteriovenous malformation or arteriovenous fistula. The treatment of arteriovenous malformation of the scalp and face has evolved from the primary use of surgery as an adjunct to embolisation therapy.5 Good cosmetic and functional outcomes can be obtained safely by subsequent embolisation and surgical excision.13 Because the face and scalp have a rich arterial network fed by branches of the external carotid artery, the arterial system that supplies an arteriovenous malformation is multiple and complex. Trans-arterial occlusion of multiple feeding arteries frequently causes incomplete devascularisation and collateral vessel recruitment.8
Case report
A 35-year-old man presented with a giant congenital craniofacial arteriovenous malformation (Fig. 1). The only associated symptom was a pressure sensation behind the left temporo fronto orbital lobe. The patient had a high risk of ulceration and rupture of the arteriovenous malformation in addition to substantial aesthetical and social disturbances. On palpation, a pulsative mass was observed. An angiography of the carotid artery was performed, which revealed a dilated left superficial temporal artery and arteriovenous malformation in the left temporo fronto orbital region. The draining of the blood flow into the dilated facial vein was seen (Fig. 2).

Figure 1
1- Superficial temporal artery; 2- Frontal segment of the malformation; 3- Temporal segment of the malformation; 4- Orbital segment of the malformation.

Figure 2
1- Superficial temporal artery; 2- Frontal segment of the malformation; 3- Temporal segment of the malformation; 4- Orbital segment of the malformation; 5- Facial vein.
Operative procedure
Surgery was performed with a fronto temporal skin incision. Primarily, superficial temporal artery was ligated in the proximal segment. Collateral vessels were ligated and fronto temporo orbital arteriovenous malformation was excised (Fig. 3). The supratrochlear and supraorbital nerves were protected. The facial vein was ligated in the orbital region. The deformed skin was excised and the defect closed with primary skin suture.

Figure 3
Surgical excision of the craniofacial arteriovenous malformation with fronto orbital approach.
Postoperative period
Postoperatively the patient is completely asymptomatic, with no local neurological deficit and palpebral ptosis. Cicatrisation of the surgical incisions followed without complications. After 4 months a Triplex scan was performed, which revealed a normal blood flow in the operated region.
Discussion
Any abnormal communication between an artery and a vein is commonly referred to as arteriovenous malformation or arteriovenous fistula.
The Hamburg 1988 classification and its modification in 1989 were proposed in an international consensus document from the International Society for the Study of Vascular anomalies (ISSVA). In this classification, vascular anomalies are divided into two main entities. These are vascular tumours and haemangiomas. Each of these is subdivided into slow flow and fast flow. Hence, five types of malformations have been identified. These are dominantly arterial, dominantly venous or dominantly lymphatic, with arteriovenous shunts and combined forms. These five entities are sub-classified into truncular and extratruncular forms; 70% of the pathologies are truncular. The classification into vascular tumours and vascular malformations is very categorical. The question is why large angiodysplastic syndromes almost always have angiomas and, interestingly, flat haemangiomas are almost always indicators of syndromes.6
Anatomical knowledge of abnormal communications between arteries and veins may be important for vascular surgeons and radiologists. Congenital arteriovenous malformations occur as a result of a lack of differentiation of arteries, veins and the capillaries during vascular development.1 Physical examination reveals an increase in skin temperature, visible and palpable pulsations, palpable thrill and bruit.2 Past research has defined the transmission of blood from the arterial to the venous side, thus shunting the blood back towards the heart.3 Arterioles with a size over 50
μm provide major resistance to blood flow, thereby increasing the metabolic demand. This means that there would first be vasodilatation of small arterioles, followed by that of the proximal larger arterioles.4
Majority of arteriovenous malformations are self-limiting lesions. Some of them may develop complications such as ulceration, airway obstruction, ophthalmic complications and psychosocial consequences.15
For arteriovenous vascular malformations in the scalp, various types of treatments have been used, including surgical excision, vessel ligation, embolisation, radiation, electro-coagulation, sclerosis and compression. The objective of the treatment is complete obliteration.9 Treatment can be optimised in a multidisciplinary environment, using a prescribed treatment algorithm to minimise the size of soft/hard-tissue defect and enhance cosmetics. Careful selection of therapeutic modalities based on arteriovenous malformation anatomy and aesthetic concerns can lead to safe and durable results with high patient satisfaction rates.12
The treatment of arteriovenous malformation of the scalp and face has evolved from the primary use of surgery as an adjunct to embolisation therapy.5 Good cosmetic and functional outcomes were obtained safely by subsequent embolisation and surgical excision.13 Because of the complex vascular anatomy and risk of massive haemorrhage, complete surgical removal is often difficult. The trans-arterial embolisation of the proximal feeding vessels decreased the blood supply and has been a helpful adjunct to surgery.7 After embolisation, surgical extirpation should not be delayed any longer than 72
h, because the resulting inflammatory reaction in the embolised area may make surgical approach more difficult.10 Scalp arteriovenous malformations are interesting lesions with heterogeneous anatomical features.
Because the face and scalp have a rich arterial network fed by branches of the external carotid artery, the arterial system that supplies an arteriovenous malformation is multiple and complex. Trans-arterial occlusion of multiple feeding arteries frequently causes incomplete devascularisation and collateral vessel recruitment.8 Arteriovenous malformation represents early lesions that are more amenable to surgical management.11
Surgical intervention or embolisation can be performed successfully. None of those treatment concepts, however, represents the only treatment method.14
In our case, the craniofacial malformation was vascularised by superficial temporal artery and multiple feeding arterial branches. Complete devascularisation of the arteriovenous malformation by trans-arterial occlusion was impossible and surgical excision was performed without the embolisation therapy.
Conflict of Interest
None.
Funding
None.
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PII: S1533-3167(09)00010-7
doi:10.1016/j.ejvsextra.2009.03.001
© 2009 European Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.
Refers to article:
- Surgical Treatment of the Giant Congenital Craniofacial Arteriovenous Malformation: A Case Report , 11 May 2009
