Angiographic evaluation of the head and neck

• Central hemangioma of bone

• Aneurysm

• Carotid body tumors

• Carotid-cavernous fistula

• Dissecting carotid aneurysm

• Arteriovenous malformations

• References

• Copyright

Facial trauma, craniomaxillofacial surgery, and certain idiopathic events can cause a variety of vascular pathologies. Those of special interest to craniomaxillofacial surgeons include the following: superficial vascular malformations (hemangiomas), intraosseous vascular tumors (central hemangioma of bone), aneurysms (false or true), arteriovenous fistulas (including carotid-cavernous fistulae (CCFs)), fibrovascular tumors (including juvenile nasopharyngeal angiofibromas), and carotid body tumors. These vascular injuries can lead to airway compromise, significant hemorrhage, irreversible neurologic damage, or death. Appropriate management begins with early diagnosis. Because vascular injuries can go unrecognized in the acute setting when a diagnosis is based solely on clinical history and physical examination, interventional radiology has become the standard for definitive diagnosis.

Angiography, the diagnostic mainstay of interventional radiology, provides images of normal and abnormal anatomical structures. It is also used to diagnose a vascular injury whose presence is suggested by noninvasive radiographic and clinical examinations. Thus, it is a useful adjunct before any surgical identification and repair, ligation, or ablation of indicated vasculature. When combined with endovascular embolization, angiography can successfully manage vascular injury, spontaneous vascular pathology, and congenital vascular anomalies. Complications of angiography combined with embolization are varied but infrequent. Ischemic tissue necrosis after artery occlusion can involve large areas of the maxillofacial region. More serious unpredictable sequelae range from permanent neurologic morbidity to death.

A working knowledge of angiography will facilitate communication between craniomaxillofacial surgeons and interventional radiologists or interventional neuroradiologists. The images and associated descriptions in this article are presented as examples of vascular abnormalities that can be diagnosed with angiography.

Hemangioma

Frequency/incidence

Hemangiomas of soft tissue are benign vascular tumors. They are the most common tumor of infancy and childhood, constituting 7% of all benign soft tissue tumors [1], [2], [3]. Hemangiomas are found in 1.1% to 2.6% of neonates, and they eventually develop in 10% to 12% of children, more often in girls than in boys (5:1). Approximately one fourth to one third of these tumors occur in the head and neck region [1].

Signs and symptoms

Hemangiomas may begin as a flat area of red pigmentation that rapidly progresses to become a red to purple raised mass. A thrill or bruit may be detectable in arteriovenous hemangiomas because of the abnormal connection between arterial and venous circulation [1], [2]. Approximately 20% of hemangiomas grow large enough to require intervention [2]. Indications for medical or surgical treatment are compression of vital structures, hemorrhage as the result of trauma, and cosmetic concerns [2].

Etiology/pathophysiology

Hemangiomas are the result of a benign proliferation of vascular channels lined with endothelium [1], [2].

Image of choice for diagnosis

Computed tomography (CT), ultrasonography, magnetic resonance imaging (MRI), and Doppler studies are useful noninvasive diagnostic techniques [2], [3]. Angiography is performed before embolization or to map the extent of a lesion if surgery is planned [2], [3].

Image hallmark

Angiography will show a hypervascular tumor with an intense capillary blush and early venous filling [2] (Fig. 1).

Fig. 1. Angiogram of a peripheral hemangioma involving the skin and subcutaneous layer of the forehead of a 6-month-old infant. (From Burrows P. Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin 1998;16:462; with permission.)

Management

Reported therapies include compression therapy for localized lesions, intralesional or systemic corticosteroids, argon laser therapy, cryotherapy, sclerosing agents, and embolization with or without surgical identification and removal [2].

Central hemangioma of bone

Frequency/incidence

Central hemangiomas of bone are rare intrabony lesions; some are caused by trauma, others are hamartomas, and still others are true neoplasms [1]. Hemangiomas of bone are more common in female patients and occur most frequently in patients in the first and second decades of life [1], [3]. Common sites include the skull, the vertebrae, and the jaws; lesions appear twice as often in the mandible as in the maxilla [1].

Signs and symptoms

Clinical findings associated with central hemangiomas include the following: spontaneous bleeding from the gingival sulcus, mobility of teeth, gingival discoloration, facial swelling or asymmetry, and a detectable thrill or bruit [3]. Indications for medical or surgical therapy are compression of vital structures, hemorrhage as the result of trauma, and cosmetic concerns [2].

Etiology/pathophysiology

Hemangiomas are the result of a benign proliferation of vascular channels lined with epithelium [1], [2].

Image of choice for diagnosis

CT, ultrasonography, MRI, and Doppler studies are useful noninvasive diagnostic techniques [2], [3]. Angiography combined with embolization can effectively reduce intraoperative hemorrhage [3].

Image hallmark

Angiography will show a hypervascular tumor with an intense capillary blush and early venous filling [2] (Fig. 2).

Fig. 2. (Top) CT showing a hemangioma of the right maxilla. (Bottom) Angiographic image of the same maxillary lesion.

Management

Reported therapies include systemic corticosteroids, laser therapy, sclerosing agents, radiation therapy, and embolization with or without surgical identification and removal [2].

Aneurysm

Frequency/incidence

Intracranial and extracranial aneurysms rarely affect the head and neck [4]. They have been reported to occur after facial trauma, head trauma, orthognathic surgery, and other head and neck surgery. Seventy percent of aneurysms result from blunt trauma, whereas 25% result from penetrating trauma [5].

Signs and symptoms

Aneurysms typically remain asymptomatic until they rupture, usually within 1 month of their development; rupture causes a thromboembolic event or hemorrhage [6]. Aneurysms are often unrecognized in the acute clinical setting because the onset of symptoms can be delayed for a few hours to a few weeks after the traumatic incident [7]. Symptoms can range from neck stiffness and pain to focal or severe neurologic defects resulting from showering emboli [8], [9]. Dissection or expansion of traumatic lesions in the neck can cause airway compromise, soft tissue deformity, asymmetry, or cranial nerve deficits, and can even mimic a tonsilar abscess [10]. Massive posterior epistaxis may occur as the result of a post-traumatic pseudoaneurysm of the intracavernous portion of the internal carotid artery (ICA) and can be life threatening [11], [12].

Etiology/pathophysiology

Aneurysms are classified as either true or false (pseudoaneurysm). True aneurysms result from the partial tearing or rupture of only the muscularis and intimal layers of an artery; this rupture allows the adventitia to prevent blood extravasation [6]. Complete transection of one side of an arterial wall leads to the formation of a pseudoaneurysm or pulsating hematoma that is contained by contiguous tissues. The organizing clot forms a psuedofibrous capsule with a liquefied central matrix. Without an arterial coat, the pressure in the hematoma increases until the pressure in the periarterial zone equals the mean arterial pressure [4]. The endothelial lining frequently produces a pseudointima continuous with the arterial lumen [4]. Hematoma liquefaction leads to the formation of a pulsating mass that can either rupture or continue to enlarge [4]. The instability of the vessel wall in both true and false aneurysms poses the constant threat of hemorrhage or infarction as the result of a thromboembolic event [6]. Left untreated, these lesions can lead to stenosis, dissection, arteriovenous fistulas, or hemorrhage [9], [13].

Image of choice for diagnosis

Angiography is the preferred imaging method for diagnosis, surgical mapping, or definitive treatment through endovascular approaches.

Image hallmark

An aneurysm appears as a projection or out-pouching from the parent vessel and may extend with dissection through soft tissue [6] (Fig. 3).

Fig. 3. (A) Anterior-posterior (AP) image of an aneurysm of the right internal carotid artery-posterior wall at the junction with the posterior communicating artery (not shown). (B) Lateral view. This patient presented with transient ischemic attacks and weakness of the left arm and face. An A-P (C) and lateral (D) view of an aneurysm of the basilar artery with the dome pointing down and to the right.

Management

Medical treatment with anticoagulant therapy may allow time for spontaneous healing of a pseudoaneurysm. However, medical therapy alone rarely accomplishes complete resolution, and there is a continuous threat of thrombotic emboli or spontaneous and rapid dissection leading to airway compromise [1]. The traditional surgical approach includes excision of the aneurysmal pouch and either direct closure or placement of a patch graft (synthetic or autologous). More recently, the placement of stents and coils through a vascular approach has proved successful. In addition, detachable balloons placed proximal and distal to the pseudoaneurysm have been shown to be effective [11], [13], [14]. Early treatment is imperative if detrimental sequelae are to be prevented [14].

Carotid body tumors

Frequency/incidence

Carotid body tumors are very rare, and their exact incidence is unknown.

Signs and symptoms

The presenting problem is usually a pulsatile and expansile mass at the angle of the mandible. Cranial nerve dysfunction can involve the vagus, hypoglossal, and cervical sympathetic nerves. More commonly, patients have vague complaints such as headache, neck and ear pain, hoarseness, syncope, tinnitus, and dysphagia [15].

Etiology/pathophysiology

Tumors arising at the bifurcation of the internal and external carotid arteries are called carotid body tumors. Because of their rarity and interesting histologic appearance, they are also known by several other names: glomus tumors, paragangliomas, nonchromaffin paragangliomas, and chemodectomas. These tumors are derived from neural crest tissue; their malignant potential is low but definite [15], [16].

Image of choice for diagnosis

Duplex scanning with color flow images can help to determine the dimensions of the tumor and can display the vascular flow. CT scans are useful for evaluating neck masses in general, and MRI techniques can readily differentiate the carotid body tumor from other soft tissues in the neck. Angiography is the definitive diagnostic test for these lesions and is helpful in surgical planning.

Image hallmark

Carotid body tumors are extremely vascular and may appear as an angiomatous tumor [15]. Findings include early tumor blush and splaying of the internal and external carotid arteries at the carotid bifurcation [16] (Fig. 4).

Fig. 4. Angiogram of a carotid body tumor showing the high vascularity of the tumor: (A) lateral and (B) A-P views. This tumor receives vascular supply from three branches of the external carotid artery.

Management

When the differential diagnosis includes carotid body tumor, needle aspiration or biopsy is contraindicated because of the vascularity of the tumor. In the presence of severe hypertension, screening for elevated serum catecholamine concentration may be warranted, as may other laboratory studies to rule out the presence of other functioning paraganglionic tumors (eg, pheochromocytomas). Surgical removal of these masses is indicated. Selective preoperative embolization of the tumor's blood supply is helpful in decreasing intraoperative blood loss.

Carotid-cavernous fistula

Frequency/incidence

Carotid-cavernous fistulas (CCFs) are vascular connections between the carotid artery and the cavernous sinus and are associated most frequently with trauma to the base of the skull [15].

Signs and symptoms

Pulsating exophthalmus, edema of the preseptal orbital tissues, enlargement and restriction of motion of the extraocular muscles, and eventual ocular ischemia are caused by the reverse flow through the venous system. Patients with these symptoms should undergo frequent ophthalmologic examinations with checks of visual acuity and ocular pressure. Changes in visual acuity necessitate immediate treatment [17].

Etiology/pathophysiology

CCFs are classified as indirect or direct. Indirect CCFs occur by spontaneous dural arteriovenous communications supplied by the ICA or the external carotid artery. Multiple lesions can exist within the cavernous sinus wall. The exact causes of indirect CCFs are unknown. Direct (traumatic) CCFs most often demonstrate a single communication between the ICA and the cavernous sinus. Causes other than blunt or penetrating trauma include direct surgical trauma, ruptured aneurysm, collagen deficiency syndromes, fibromuscular dysplasia, or arterial dissection [17], [18].

Image of choice for diagnosis

Selective cerebral angiography is the diagnostic standard. CT scanning and MRI can be helpful in determining whether brain damage exists or in identifying fractures.

Image hallmark

In the case of a traumatic transection of the ICA, the cavernous sinus will opacify, and there may be decreased filling of intracranial vasculature from ipsilateral angiography (Fig. 5). Vascular injuries and associated pseudoaneurysm may be seen. Venous outflow from the cavernous sinus will be seen through the ophthalmic venous system. This flow may continue to the facial vein and the external jugular vein. Some patients may exhibit outflow through posterior drainage of the cavernous sinus and the superior and inferior petrosal sinuses. Superior drainage may be displayed as opacification of the sphenoparietal sinus, which places the patients at risk of intracerebral hemorrhage. Most often, a combined pattern will be seen [17].

Fig. 5. Angiogram of a cavernous-carotid fistula following a gunshot wound to the head. The fistula is demonstrated by the blush in the cavernous sinus and during angiography of the right internal carotid artery.

Management

Patients with severe proptosis, increasing intraoccular pressure, and severely declining visual acuity may require lateral canthotomy as a temporary step for preventing loss of vision. Endovascular approaches with either balloon embolization or coil embolization are the preferred means of treatment. Proximal suture ligation of the ICA has been shown to be ineffective and may induce or increase steal syndrome. Thus, this intervention should be avoided [19].

Dissecting carotid aneurysm

Frequency/incidence

Aneurysms of the intracranial and extracranial portions of the carotid artery are rare and may be difficult to diagnose. Aneurysms of the extracranial carotid artery compose fewer than 5% of all extracranial arterial aneurysms [20].

Signs and symptoms

The signs and symptoms can vary greatly depending on the location of these aneurysms. Cervical aneurysms can bulge into the lateral parapharyngeal wall, mimicking a neck mass [21], [22]. The mass may or may not pulsate, depending on whether a thrombus has formed within the lumen [20]. Symptoms may include hemorrhage, either otorrhagia or epistaxis. Nerves immediately adjacent to the expanding mass may be compressed, and Horner syndrome has been described in several cases of carotid aneurysm. Patients with extracranial aneurysm may exhibit paralysis of cranial nerves IX, X, XI, and XII. Patients with intracranial aneurysm may exhibit headaches, nausea, vomiting, drowsiness, transient ischemic attacks, and loss of vision and hearing. Associated signs and symptoms such as fever and upper airway obstruction can be related to the underlying cause of the aneurysm (see Etiology/pathophysiology).

Etiology/pathophysiology

Dissecting carotid aneurysms may be congenital or caused by trauma or infection [23], [24]. They have been reported to occur after blunt trauma to the head and after a tonsillectomy. Although trauma appears to be the most common cause of these aneurysms, they may develop for several reasons, which vary according to the age of the patient. These aneurysms have occurred in patients younger than 5 years and in patients older than 40 years. Dissecting carotid aneurysms may be caused by a deep space infection of bacterial or mycotic origin. When they occur as a complication of neck space infection, the diagnosis may be difficult. Patients with fibromuscular dysplasia, Marfan syndrome, or Ehlers-Danlos syndrome, and those who have undergone radiation therapy, appear to be at greater risk of carotid aneurysm. The lesion may be a true or false aneurysm. True aneurysms are usually congenital, whereas false aneurysms are usually the result of trauma or infection.

Image of choice for diagnosis

CT scanning, MRI, and magnetic resonance angiography have been used to image carotid aneurysms; however, arteriography is the method of choice.

Image hallmark

Arteriography reveals a large outpouching of contrast beyond the walls of the ICA (Fig. 6).

Fig. 6. Dissecting carotid aneurism following blunt head trauma. Note the saclike projection extending superiorly from the internal carotid artery.

Management

The choice of treatment depends on the location of the lesion and on the patient's condition. Surgical intervention is used most often for lesions in the neck; this treatment involves resection of the lesion with repair of the carotid artery. Balloons and coils are used most often to treat intracranial lesions. The use of balloons allows an assessment of the status of the contralateral cerebral blood flow.

Arteriovenous malformations

Frequency/incidence

Arteriovenous malformations (AVMs) are rare lesions that may be congenital or may result from trauma. Congenital lesions grow as the patient ages and may increase in size dramatically during puberty or pregnancy [25].

Signs and symptoms

The clinical presentation of AVMs depends on the characteristics of the lesion. Patients with lesions in the head and neck may exhibit increased intracranial pressure, mass effect, or steal syndrome. Symptoms may include disfigurement, pain, bruit, or hemorrhage. When they occur in the region of the maxilla and mandible, AVMs may cause facial swelling, loose teeth, tinnitus, and headaches.

Etiology/pathophysiology

The clinical behavior and prognosis associated with AVMs are determined by the presence and degree of shunting. Congenital lesions are presumed to have been present at birth and to have grown with age. Traumatic lesions usually result from penetrating injuries and can originate from the internal or external carotid arteries, the vertebral arteries, or a transected vessel.

Image of choice for diagnosis

A Doppler ultrasound probe may be used to examine the lesion and to determine the need for further tests. Angiography may be either diagnostic or therapeutic. When the lesions are very large and involve both bone and soft tissue, MRI may be used to determine the extent of soft tissue involvement [26].

Image hallmark

The appearance of AVMs varies greatly depending on their location, size, and degree of shunting and collateral circulation (Fig. 7).