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184 S. I. Wahl and P. S. Lakritz
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A
FIGURE 17-8. Subclavian steal syndrome. A: Arch aortogram reveals an occlusion of the proxi-
mal left subclavian artery (arrow). B: Late-phase of arteriogram shows retrograde flow down the left vertebral artery into the left subclavian artery (arrow).
axillofemoral bypass graft. The offending lesion of the proximal subclavian artery or brachiocephalic artery is often amenable to percutaneous transluminal angio­plasty or stenting.
25
Potential complications of subclavian and axillary artery angioplasty include distal emboliza­tion and stroke.
The most common cause of subclavian steal is athero­sclerotic disease. Other causes include trauma, arteritis, vascular obstruction by tumor, radiation therapy, and con­genital malformation associated withcongenital heart dis­ease.
Thoracic outlet obstruction
The term thoracic outlet syndrome refers to a group of dis­orders resulting in vascular or neurologic compromise at the thoracic outlet, where the neurovascular bundle exits the chest. Only 1% of the cases of thoracic outlet syn­drome involve the subclavian artery; a slightly greater percent are venous related, and 97% have a neurologic
26,27
basis.
The thoracic outlet is anatomically defined as a region bounded by the first rib inferiorly, the clavicle superiorly, the scalenus muscles posteriorly, and the costoclavicular ligament anteriorly. compromise is most often compression of the subclavian artery between a cervicle rib and the clavicle. Other sites of compression may be between the scalenus anticus and the scalenus medius tendons or at the costoclavicular space due to a prominent subclavius muscle. The axillary
28
The anatomic basis for vascular
B
artery may course between the coracoid process and the pectoralis tendon.
1,29
Arterial thoracic outlet syndrome
Angiography plays an important role in the diagnosis and treatment of arterial thoracic outlet syndrome and is in­dicated in patients with suspected arterial thoracic outlet syndrome when ischemia or embolism is suspected. An­giographic findings include stenoses or occlusions of the subclavian, axillary, or brachiocephlic arteries or their branches; extrinsic compression of vessels between the clavicle and first rib; poststenotic dilatation, increased collateral circulation; mural thrombus or embolization; and retrograde filling of the subclavian artery via the vertebral artery. in the plane of view and is unseen; its presence is indi­cated only by visualization of the poststenotic dilatation.
It can be difficult to separate an arterial cause of symp­toms of the upper extremity and hand from a neurologic etiology. It also can be difficult to determine the signifi­cance of a subclavian artery narrowing seen on an angio­gram with the extremity stressed as some degree of nar­rowing can be induced in an asymptomatic patient with the arm hyperextended (Fig. 17-9).
In addition to symptoms produced by direct vascular compression, subclavian artery compression can lead to poststenotic aneurysm formation. The aneurysm walls can be lined with thrombus that can potentially embolize distally. Arteriography is performed with the upper extremity in adduction or in a neutral position and then
30
Frequently, the arterial stenosis is not
Thoracic Outlet and Upper Extremities
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A B
FIGURE 17-9. Thoracic outlet syndrome. A: A right subclavian arteriogram in adduction is essen-
tially normal. B: Repeat arteriogram with the right arm abducted in a position that produced the patient’s symptoms shows an obvious narrowing of the sublcavian artery and poststenotic dilata­tion (arrow).
185
in a position that elicits the described symptoms. Despite first rib resection and scalenectomy, 15 to 20% of patients will develop recurrence of symptoms.
31
Today, consider­able confusion and disagreement remain over the ana­tomic basis of thoracic outlet obstruction, the implica­tions of its angiographic findings, and the indications and results of surgical correction.
26,27
Venous thoracic outlet syndrome
Venous outlet syndrome is synonomous with axillosubclavian vein obstruction, and the problems related to venous tho-
racic syndrome remain more confusing than those of ar­terial or neurologic obstruction. Deep venous thrombosis of the upper extremities is exceedingly uncommon com­pared with that occurring in the lower extremities.
The etiology of upper-extremity venous thrombosis is variable and includes trauma, SVC syndrome, intratho­racic tumors, foreign bodies, polycythemia, thrombocy­tosis, cor pulmonale, congestive heart disease, and coagu­lopathies.
34,35
Primary or effort thrombosis of the axillosubclavian veins, also known as Paget-Schoeder’s syndrome, is a distinct clinical entity leading to disabling arm swelling that can be distinguished from other causes of axillosubclavian vein thrombosis. This condition is seen primarily in young men 40years of age or younger who are otherwise healthy. Axillosubclavian vein occlusion typically occurs following repetitive trauma. Frequently, there is a histor y of vig­orous or unusual exercise preceding the onset of symp-
36
toms.
The two major radiographic patterns identified in pri-
32,33
mary thrombosis of the axillosubclavian veins are local­ized obstruction of the axillosubclavian veins at the level of the junction of the first rib and clavicle (Fig. 17-10) and a long segment of occlusion of the axillary vein.
36
Although the diagnosis of axillosubclavian venous thrombosis is much easier to make than neurologic tho­racic outlet syndrome, the decisions regarding treatment are more complex. Catheter-directed intravenous throm­bolysis may be effective in patients who have acute symp­toms of axillosubclavian venous thrombosis. With less acute or chronic occlusions, the incidence of complete lysis is of little or no benefit because rethrombosis is virtually inevitable. Some investigators advocate balloon angioplasty for underlying residual venous stenoses after thrombolysis.
37
Protection against rethrombosis after complete lysis with long-term warfarin therapy has not proven effective in most patients, even over the short term, when awaiting surgical decompression of the thoracic outlet to prevent rethrombosis after initial lysis. Most agree that surgical decompression to remove external anomalies and outlet compression should be performed immediately follow­ing thrombolysis.
26
Superior vena cava syndrome
The SVC syndrome consists of a constellation of findings that are the result ofSVC or bilateral brachiocephalic vein obstruction. The classic presentation of SVC syndrome is the development of head, neck, and upper body edema and cyanosis. Patients frequently complain of chest dis­comfort, headache, and dizziness. More severe symptoms
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A
FIGURE 17-10. Paget-Schroeder syndrome. Young male weightlifter with acute onset of disabling right arm swelling. A: Right upper-extremity venogram demonstrates occlusion of the axillary and subclavian veins at the junction of the first rib and clavicle with an extensive network of venous collaterals. B: Follow-up venography 24 hours after direct urokinase infusion shows near complete thrombolysis with only minimal irregularity of the brachiocephalic vein. The patient underwent surgical decompression directly after lysis.
B
can include changes in mentation, and laryngeal edema with upper airway obstruction. Symptoms may be sub­acute and slowly progressive, or they may have an acute
38
onset.
The SVC is a relatively thin-walled vessel lying within a nondistensible space in the mediastinum, making it sus­ceptible to extrinsic compression by primary tumors or lymphadenopathy.
The SVC syndrome most commonly is secondary to an underlying malignancy of the mediastinum and has been shown to be responsible for 85 to 97% of all cases.
39,40
Furthermore, SVC syndrome may occur in up to 10% of all patients with a right-sided intrathoracic mass lesion. Prior to the onset of antibiotic therapy, infectious etiolo­gies of the chest and mediastinum were commonly the source of SVC obstruction. Nevertheless, with the in­crease in the number of immunosuppressed patients sec­ondary to human immunodeficiency virus (HIV) infec­tion with an associated increased incidence of tuberculosis, histoplasmosis, and syphilis, infectious proc­esses remain in the differential diagnosis of SVC syn­drome.
39
Rare causes of SVC obstruction result from goi­ters, benign idiopathic mediastinal fibrosis, and aortic valve replacement.
42–44
Additionally, indwelling central catheters and transvenous pacemaker leads may result in iatrogenic SVC obstruction and should be considered in the differential diagnosis.
45,46
Radiologic evaluation of suspected SVC syndrome in-
cludes conventional chest x-ray, computed tomography
(CT), magnetic resonance imaging (MRI), conventional contrast venography, and radionuclide imaging. CT is the modality of choice because it is readily available, relatively noninvasive, and is useful in accurately local­izing the site and often the etiology of the obstruction as well as potential collateral veins. The surrounding lung and mediastinal structures can be evaluated with
39
CT.
Radionuclide imaging using technetium-99m (Tc99m)­labeled sulfur colloid is a valuable screening tool for suspected SVC obstruction. Serial radionuclide examina­tions can be obtained to evaluate for possible recanali-
41
zation ofthe central veins orthe restoration of flowfollow­ing therapy.
47
Patients with SVC syndrome often gain significant symptomatic improvement from conservative treatment measures, including head elevation and supplemental oxygen. Underlying malignant neoplasms of the chest, predominately small-cell lung carcinoma and lym­phoma, may be managed initially with chemotherapeu­tic agents.
48,49
Additionally, radiotherapy is an effective treatment modality for most underlying causes of SVC syndrome and leads to symptomatic improvement in most patients.
50
Thrombolytic therapy may be required for patients who have demonstrable intraluminal throm­bus within the SVC.
51,52
Surgical bypass for SVC obstruc­tion with either autologous vein or synthetic material may be a useful way to palliate symptoms in selected patients.
53
Catheter- directed thrombolysis in conjunc-
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tion with endovascular stent placement has been shown to be a safe and effective treatment for SVC syndrome.
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J.A. Bello and B. BerkowitzCarotid, Vertebral,andSpinal Arteri ography
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18
■■■
Carotid, Vertebral, and Spinal Arteriography
JACQUELINE A. BELLO AND BRUCE BERKOWITZ
Although usually they are managed by neuroradiologists, especially in academic institutions, the head, neck, and spine are of interest to the vascular and interventional radiologist, who also may perform diagnostic and inter­ventional procedures in these anatomic areas. This chap­ter provides a brief, overview of this vascular anatomy and pathology of these regions.
■ Anatomy
Arteries of the neck
Within the superior mediastinum, the aortic arch nor­mally gives rise to three major arteries that supply the upper extremities, head, and neck. The first branch is the brachiocephalic trunk, also called the innominate artery. This vessel courses superiorly for a short distance before bifurcating into the right subclavian and right common carotid arteries (Fig. 18-1). The right sub­clavian artery (RSCA) courses superiorly, gives off the right vertebral artery, and then turns laterally to supply the right upper extremity. The right common carotid artery (RCCA) courses superiorly to bifurcate into the right internal and external carotid arteries The second vessel off the aortic arch is the left common carotid ar­tery, which travels cephalad and bifurcates into the left internal and external carotid arteries. The left subclavian artery is normally the final arch vessel. It courses supe­riorly before turning sharply laterally to supply the left upper extremity. The common carotid arteries are paired vessels located anteriorly within the neck. Each common carotid artery courses superiorly, along with the
vagus nerve and internal jugular vein, without branch­ing, and then it bifurcates into the internal carotid artery (ICA) and external carotid artery (ECA) (Figs. 18-2 and 18-3). The bifurcation of the common carotid artery is generally at the level of C3–4, but it may occur anywhere from C1–T2.
The ICA supplies the anterior circulation of the brain as well as a portion of the skull base and is divided into four segments: cervical, petrous, cavernous, and supracli­noid. Only the cervical segment is extracranial. The most proximal portion of the ICA is posterolateral in relation to the ECA and then courses medially to enter the skull base through the carotid canal. The cervical ICA has no named branches. The remaining three segments of the ICA are named as to their location; the petrous and cavernous portions lie within the petrous bone and cavernous sinus, respectively, and the supraclinoid seg­ment runs above the level of the anterior clinoid. The major arteries of the anterior circulation of the brain are branches of the supraclinoid ICA (Fig. 18-4).
The ECA is the smaller of the two branches of the common carotid artery. At its origin, the ECA is antero­medial to the ICA. The branches of the ECA are the superior thyroid, ascending pharyngeal, lingual, facial, occipital, and posterior auricular arteries. The ECA then bifurcates into the internal maxillary and superficial tem­poral arteries. Certain branches of the ECA may serve as important collaterals to the intracranial circulation in the setting of ICA stenosis or occlusion. These include the distal branches of the internal maxillary artery and the facial artery, both of which may fill the supraclinoid ICA by anastomosing with the ophthalmic artery. Less com­monly, the ascending pharyngeal artery and the middle
1
189
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FIGURE 18-1. The aortic arch and its major branches (antero­posterior view).
meningeal branch of the internal maxillar y artery may serve as collaterals.
The vertebral arteries arise as the first and largest branches of the subclavian arteries and supply the poste­rior circulation of the brain, spinal cord, and muscles of the neck. The vertebral arteries travel superiorly to enter
FIGURE 18-2. The external carotid artery and its branches.
the foramina transversaria of the C6 vertebra and then run within bony canals, exiting at the C2 foramena. The artery then turns sharply laterally to course around the lateral mass of C1 before returning medially to ascend through the foramen magnum to join and form the basi­lar artery. The occipital artery and ascending pharyngeal artery may serve as collateral supply to the vertebral ar­teries and posterior circulation.
Veins of the neck
The interal jugular veins (IJV), formed by the confluence of the sigmoid sinuses and the inferior petrosal sinuses, drain most of the blood from the brain (Fig. 18-5). The IJVs are paired structures that course inferiorly, lateral to the carotid arteries and deep to the sternocleidomastoid muscle (SCM) in the neck, to join the ipsilateral sub­clavian vein. The external jugular veins (EJV) drain por­tions of the face and neck; they course superficial to the SCM to join the subclavian veins. The vertebral veins arise in the occipital region from multiple small veins and
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A
FIGURE 18-3. Film subtraction angiography ofthe left common carotid arterybifurcation in anteroposterior (A) and lateral (B) views
demonstrate the normal branching pattern of the common carotid artery (1) into the internal (2) and external (3) carotid arteries.
travel inferiorly adjacent to the vertebral arteries within the foraminae transversaria to empty into the innominate veins.
■ Pathologic Processes of the Arteries
of the Neck
Atherosclerosis
Atherosclerosis is by far the most common disease proc­ess to involve the arteries of the neck. Caused by the formation of fibrofatty intimal plaques, its various mani­festations include intimal calcification, vessel tortuosity, and ectasia. This process may progress to stenosis, occlu­sion, and dolichoectasia and aneurysm formation (Fig. 18-6). The most common sites of atherosclerotic stenoses within the neck are, in order of frequency, the common carotid bifurcation, origins of the ICA and ECA, the ori­gins of the vertebral arteries, and the proximal subclavian arteries.
2
Angiography has an important role in this dis-
ease: Several large-scale prospective studies found that carotid endarterectomy is effective in the long-term pre­vention of stroke in certain patients with moderate to severe stenosis.
3,4
Fibromuscular dysplasia
Fibromuscular dysplasia (FMD) is an idiopathic process that leads to stenoses of medium to small arteries as a result of segmental overgrowth of fibrous and muscular elements of the arterial media and, to a lesser extent, the adventitia (Fig. 18-7). Originally described in renal arter­ies, FMD most commonly affects the cervical ICA, but it may involve any of the major arteries of the neck. is often bilateral and is associated with spontaneous arte­rial dissection as well as intracranial aneurysms. It is more common in women and tends to occur in patients younger than the expected age distribution for athero­sclerosis. Radiographically, FMD usually appears as alter­nating segments of stenosis and dilatation, producing a so-called “string of beads” appearance.
2
FMD
B
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FIGURE 18-4. Internal carotid artery distribution (lateral view).
Takayasu arteritis
Takayasu arteritis, also known as pulseless disease, is char­acterized by long-segment, smooth stenoses of the proxi­mal arch vessels, although any of the branches of the aorta may be involved (Fig. 18-8). The left subclavian is the artery that is most often involved, although typically several vessels are involved.
5
The process often leads to complete occlusions of vessels. The disease typically af­fects females aged 15 to 45 years. The pathogenesis of this disease is unknown.
Giant cell arteritis
Giant cell arteritis is an idiopathic process manifested by focal granulomatous inflammation of medium and small arteries. This disease usually affects smaller vessels, such as the superficial temporal artery, but it may involve the larger vessels of the neck. The most common radio-
FIGURE 18-5. The draining veins of the cranium and neck.
graphic pattern in giant cell arteritis is multiple discrete stenoses.
1
Subclavian steal
In some cases, collateral pathways are already present but do not manifest until normal hemodynamics are altered, as in the case of subclavian steal syndrome. Subclavian steal syndrome occurs when the subclavian artery, most commonly the left, becomes occluded proximal to the ori­gin of the vertebral artery. In this setting, the distal sub­clavian artery “steals”blood from the posterior circulation of the brain by means of retrograde flow through the ipsi­lateral vertebral artery (Fig. 18-9). The decreased blood flow to the posterior circulation of the brain may result in ischemia, from which patients often become symptomatic when exercising the affected upper extremity.
Trauma
Traumatic injury to the neck is a common indication for performing angiography, especially in the setting of pene­trating trauma to regions where physical examination is
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FIGURE 18-6. Digital subtraction angiography of left common carotid artery in anteroposterior oblique projection demon­strates stenosis involving the carotid bulb with a focal collection of contrast projecting posteriorly. The appearance is typical of an ulcerated atherosclerotic plaque.
difficult. By convention, the neck is divided into three zones:
• Zone 1 extends from the thoracic inlet to the cricoid cartilage
• Zone 2 extends from the cricoid to the angle of the mandible
• Zone 3 extends from the mandibular angle to the skull base.
Traditionally, penetrating trauma to zones 1 and 3 calls for an angiogram in most surgical algorithms, although angiographic studies for these “proximity” injuries have
6
notoriously low yields.
Zone 2 injuries are not usually examined by angiography because damage to the artery is assessed relatively easily clinically because of the super­ficial position of the artery in this zone. Damage to the cervical vessels may be caused by penetrating or blunt trauma and may manifest as spasm, thrombosis, partial or complete transection, dissection, arteriovenous fistula (AVF), or pseudoaneurysm (Fig. 18-10). As a general rule, wounds cephalad to the angle of the mandible are more likely to result in damage to the vessels, and stab wounds are associated with vascular injury less frequently than gunshot wounds.
6
These criteria pertain mainly to the carotid circulation,
FIGURE 18-7. A 40-year-old man presented with a history of recent stroke. Digital subtraction angiography of left common carotid artery (anteroposterior view) demonstrates a long seg­ment of alternating areas of stenosis and dilatation in the left internal carotid artery distal to the normal bifurcation. This is typical of fibromuscular dysplasia and is commonly referred to as a “string of beads” appearance. (Case courtesy of Dr. De­borah Shatzkes.)
because the vertebral artery is relatively well protected within its bony canal. Clinical indicators of vascular injury include expanding hematoma, absent pulses, bruit, and altered neurological status.
Neoplasms and other disease processes
Although multiplanar imaging modalities have mostly re­placed angiography in the evaluation of lesions that do not directly involve the normal vessels of the neck, the ability to facilitate therapy by embolization secures the role of angiography in a select set of disease processes, both neoplastic and nonneoplastic. In the neoplastic cate­gory, paraganglioma, juvenile angiofibroma, and menin­gioma are the tumors most commonly considered for em­bolization and, hence, for angiography.