Disclosure
Background: Thoracic outlet syndromes are due to the compression of the neurovascular structures passing through the thoracic outlet. The syndromes can be classified into 3 subgroups based on the neurologic or vascular structure involved. The specific clinical presentations, demographics, treatments, and outcomes vary among the subgroups. Subgroup 1, or the neurologic type, is the most common, and it is responsible for approximately 95% of cases of thoracic outlet syndrome. This type is secondary to compression of the brachial plexus caused by various soft tissue and bony abnormalities where the nerves pass between the anterior and middle scalene muscles. For a discussion of the neurology of this syndrome, see Thoracic Outlet Syndrome in the Neurology section of the journal. Subgroup 2, the venous type, is the more common of the vascular causes and is seen in approximately 3-4% of patients with thoracic outlet syndrome. Venous thrombosis may be categorized into primary and secondary kinds, based on the etiology. Primary venous thoracic outlet syndrome, or primary venous thrombosis, is also called Paget-Schrötter syndrome. The disease is named after the 2 individuals who first described this entity: Paget, who described it in 1875, and von Schrötter, in 1884. Other terms for this condition include effort thrombosis, spontaneous thrombosis, and traumatic thrombosis. Subgroup 3, or the arterial type of vascular causes, is the least common form of thoracic outlet syndrome and is seen in approximately 1-2% of patients. This type is associated with the most serious complications, including limb ischemia, which may result in the loss of the affected upper extremity. Rarely, compression of a combination of structures may be responsible for the symptoms. This article is limited to the vascular causes of thoracic outlet syndrome. Pathophysiology: Compression of the vascular structures passing through the thoracic outlet may occur at several anatomic sites and includes the following: (1) compression of arteries or veins medial to the scalene triangle in the costoclavicular space or beneath the pectoralis minor tendon in the axilla, (2) arterial compression within the scalene triangle itself, and (3) venous compression between the anterior scalene muscle and the clavicle. The relatively small size of these spaces, the hypertrophy of muscles around these spaces, congenital abnormalities, and pathologic masses (eg, tumors or callous formation) may all cause compression of adjacent vascular structures. Arterial causes The essential mechanism of subclavian artery thoracic outlet syndrome is chronic compression that results in intimal injury with fibrosis; thickening of the wall; and eventually, luminal narrowing. Poststenotic dilation develops as a result of hemodynamic turbulence distal to the site of narrowing. Distal thromboembolism is a severe complication that may result either from mural thrombus originating within the area of poststenotic dilation or from an intimal lesion at the site of compression with resultant formation of platelet aggregates. These platelet aggregates may microembolize distal to the small vessels of the hands and fingers, resulting in ischemia with eventual tissue necrosis. Mural thrombi typically result in the occlusion of more proximal arteries with larger collateral supplies; therefore, these thrombi are less likely than the others to produce severe ischemic changes. Rarely, occlusion of the subclavian artery may occur. The most common cause of subclavian artery compression is a cervical rib, which is seen in 50% of cases. A cervical rib can posteriorly compress the subclavian artery at the scalene triangle against the anterior scalene muscle and first rib. Other etiologies include congenital first-rib anomalies, first-rib exostoses, and malunited fractures of the clavicle. Rare causes include congenital fibromuscular bands and anterior scalene muscle anomalies. Venous causes Primary venous thrombosis is most likely to be related to a multifactorial etiology, including extrinsic compression or trauma with a congenitally narrow thoracic inlet. Chronic extrinsic compression may be caused by anatomic anomalies, such as a cervical rib, the first rib, hypertrophied subclavius or anterior scalene muscles, or a malunited clavicle fracture with abundant callous formation. Compression may be exaggerated when the upper extremity is in certain positions, such as in the rigid military style of sitting with the back straight and the shoulders placed posteriorly and inferiorly. With chronic irritation of the vessel walls, these anomalies may predispose an individual to stasis, intimal damage, and hypercoagulability, which form a constellation of pathophysiologic events called the Virchow triad. At least 2 of these 3 factors are typically found in patients with primary venous thrombosis. The eventual result is the formation of an intraluminal thrombus, which causes the lumen to become narrowed and possibly entirely occluded. Most authors classify the anatomic causes of axillosubclavian vein thrombosis as primary. However, the etiology is investigated in all patients, and as the body of knowledge of causes of venous thrombosis improves, the label of primary venous thrombosis is slowly falling out of favor. Secondary venous thrombosis has a number of causes, including the following:
The most common cause of secondary venous thoracic outlet syndrome is central venous catheter placement. Other causes Intraluminal foreign bodies often result in intimal injury; the incidence increases with the size of the object. As in primary venous thrombosis, this predisposes the individual to the formation of a thrombus. Radiation therapy is known to cause arterial occlusion, and several studies have been performed to investigate the occurrence of venous thrombosis after radiation therapy. Wilson reported findings in 2 patients with breast cancer who were treated with tamoxifen and radiation therapy, with ipsilateral arm swelling 3 or 4 years after therapy. Venography revealed subclavian vein thrombosis in both patients. Schreiber and Kapp reviewed findings in 225 patients who underwent combined chemotherapy and mantle radiation therapy for mediastinal lymphoma. They identified 4 patients with posttreatment subclavian vein thrombosis, of whom 3 received chemotherapy in the same arm as the venous thrombosis. Their observation suggests that the chemotherapeutic agent is a potential factor. Malignant tumors have also been associated with venous thrombosis, and at least 2 mechanisms (direct venous compression and transitory migratory thrombophlebitis) have been postulated; both may exist simultaneously. Frequency:
Mortality/Morbidity:
Race: No racial predilection exists. Sex: Thoracic outlet syndrome is traditionally more common in women than in men, although recent authors report a higher frequency in women, with a female-to-male ratio as high as 3:1. Age: Thoracic outlet syndrome is most common in those aged 10-50 years. Anatomy: The right subclavian artery arises from the innominate artery, which is the first major branch of the aortic arch. The left subclavian artery arises directly from the aortic arch as the final major branch. After leaving the thoracic cavity posterior to the sternoclavicular joint and arching over the pleural cupola, the subclavian artery passes through the scalene triangle, which is formed by the first rib inferiorly, the anterior scalene muscle anterolaterally, and the medial scalene muscle posteromedially. The artery then continues under the clavicle and subclavius muscle and enters the axilla, where it is renamed the axillary artery. After passing inferior to the pectoralis minor muscle tendon, the artery is called the brachial artery, which continues distally along the medial aspect of the humerus. After occlusion of the subclavian artery, the blood supply to the peripheral arm is maintained by the collateral vessels present among the suprascapular, circumflex scapular, subscapular, and posterior circumflex humeral arteries as well as between the transverse cervical and posterior circumflex humeral arteries. Veins Superficial veins along the ulnar aspect of the arm drain into the median antebrachial and median antecubital veins, which in turn drain into the basilic vein. The basilic vein becomes the axillary vein after joining with the brachial vein. The radial aspect of the arm is drained by the cephalic vein, which passes along the deltopectoral groove lateral to the clavicle, and joins the azygous vein. At the outer border of the first rib, the axillary vein becomes the subclavian vein, which passes through the costoclavicular space. The first rib and anterior scalene muscles are positioned posteriorly, and the clavicle and subclavius muscle are positioned anteriorly. This path is unlike that of the subclavian artery, which is posterior to the anterior scalene muscle. Clinical Details: The most common initial clinical sign of arterial thoracic outlet syndrome is ischemia of the affected arm resulting from distal embolization. The site of ischemia depends on the size of the emboli. Microembolization of the arteries of the fingers and digital arch may result in ischemic signs and symptoms ranging from Raynaud phenomenon to gross digital ischemia. Arterial thoracic outlet syndrome infrequently appears prior to the onset of acute upper extremity ischemia. Occasionally, neurologic symptoms resulting from a bony abnormality, such as a cervical rib, may occur before the onset of signs and symptoms secondary to arterial ischemia. A subclavian artery aneurysm may be detected as a palpable mass during routine physical examination, or a cervical rib may result in anterior and superior displacement, which results in prominence of the subclavian artery. All patients with upper extremity ischemic symptoms require a thorough evaluation for possible embolic sources, including a complete vascular examination. During the examination, the contralateral arm should be checked because evidence of bilateral upper extremity ischemia supports a central thromboembolic source, such as the heart. In particular, arterial thoracic outlet syndrome should be considered in a young patient with upper extremity ischemic symptoms. The patient should be asked about a history of trauma to the upper body with a healed fracture of the clavicle or upper ribs. Previous chest radiographs or other radiologic studies of the upper body should be reviewed to search for a cervical rib or other bony abnormality. Venous thoracic outlet syndrome With primary venous thoracic outlet syndrome, male patients are typically affected more often than female patients, with a ratio of 3:2 or 4:1 depending on the author. The right upper extremity is affected more often than the left upper extremity. Symptoms commonly begin within the first 24 hours in primary venous thrombosis, although, in some patients, symptoms may be insidious at the onset of thrombosis. Patients are usually aged 20-50 years and otherwise healthy. Secondary venous thrombosis, unlike the primary variant, typically occurs in older patients and has a more uniform sex distribution. The most common symptoms of subclavian or axillary venous thrombosis include swelling, discoloration, collateral vein dilatation, and aching. Secondary venous thrombosis tends to develop gradually, with a relative delay in the clinical presentation and, therefore, in treatment. Initial symptoms may range from minor discomfort, aching, or weakness, to severe pain. Over time, the hand and forearm become cold to the touch, with diminished finger movements. Untreated, this condition eventually results in swelling and bluish discoloration of the entire affected arm. Signs of secondary venous thrombosis during physical examination are typically more prominent in the distal structure; with the fingers and dorsal aspect of the hand having the most severe findings. Pitting edema, bluish discoloration, and coolness to the touch may be present. Distension of the venous system of the arm is also common, with the basilic and cephalic veins distension occurring first, followed by generalized distension of the remaining veins and venules. On examination, the distended veins feel tense and do not collapse with abduction of the arm to above the level of the right atrium. In approximately one half of patients, the axillary vein is palpable as a cordlike mass in the lateral aspect of the axilla. In addition, supraclavicular tenderness may indicate extension of thrombus into the subclavian vein, which is a common finding. Further extension into the internal jugular vein or superior vena cava may result in swelling of the face and neck, which is similar to the findings of superior vena cava syndrome. During the following weeks, as further collateral pathways form between the axillary and cephalic veins to the mediastinal and intercostal veins, collateral veins may become visible over the upper part of the chest and the shoulder. These veins may allow adequate drainage of the affected extremity and, thus, improvement or resolution of the symptoms. Preferred Examination: Various examination techniques can be used to distinguish among the etiologies of the thoracic outlet syndromes.
Findings of the Allen maneuver, the hyperabduction maneuver, are considered positive when the radial pulse disappears during extreme abduction of the arm. However, this finding is also present in individuals who do not have thoracic outlet syndrome and in individuals with asymptomatic cervical ribs; therefore, this finding is not diagnostic.
A positive Adson finding occurs when the radial pulse is reduced or disappears or when the patient's blood pressure changes when the patient (1) is in a sitting position, (2) holding a deep inspiration, (3) fully extending the neck, and (4) turning the head toward the ipsilateral and contralateral sides. Some investigators believe that the cause of these findings is compression by the anterior scalene muscle. A supraclavicular bruit may be audible with this maneuver and is believed to result from an associated subclavian stenosis.
The costoclavicular maneuver is performed when the patient assumes an exaggerated military posture and positions his or her shoulders back and downward; this positioning induces compression between the clavicle and the first rib.
Ultrasonography is readily available and relatively inexpensive, and it can be performed in both arterial and venous thoracic outlet syndrome. Magnetic resonance (MR) angiography and computed tomographic (CT) angiography of the thoracic inlet, especially with recently devised techniques and protocols, are promising noninvasive modalities that may soon provide image quality comparable to that of angiography and venography. Angiography and venography remain the criterion standards for the radiologic diagnosis of these conditions, and they have the added benefit of enabling potential endovascular treatment. Limitations of Techniques: Despite considerable investigation to identify a clinical maneuver for the accurate diagnosis of vascular thoracic outlet syndrome, no clinical test has a consistently high degree of accuracy. The same positive findings are occasionally found in individuals without vascular thoracic outlet syndrome. Therefore, consider a positive result at clinical examination in context with the clinical history and the results of other diagnostic tests. The final diagnosis often depends on invasive procedures such as arteriography. MR angiography and CT angiography techniques are evolving, and, in the near future, they may be able to replace many of today's invasive diagnostic angiographic examinations.
Aorta, Coarctation
Lung, abscess
Findings: Plain images, such as chest radiographs and upper thoracic and cervical spine studies, can effectively depict congenital or acquired bony anomalies (eg, cervical ribs, healed fractures). Radiography is important if prior images are not available. Such findings may help focus subsequent, more complex and more invasive radiologic studies of a particular region. In addition, unsuspected findings such as a Pancoast tumor of the lung may be identified on these initial studies. False Positives/Negatives: Although plain radiography is relatively inexpensive as a radiologic screening test, it is highly limited in its ability to depict the fine anatomic details that contribute to symptoms of thoracic outlet syndrome. Plain radiography has a lower sensitivity for these findings than other modalities such as CT. |
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Findings: CT is most helpful when plain radiographs show abnormal findings involving the thoracic outlet. CT, performed both before and after intravenous administration of contrast agent, as well as CT angiography, is useful in identifying lesions (eg, cervical disks, neoplasms, bony spurs) that encroach on the structures of the thoracic outlet. Recently, investigators have reported the use of various sophisticated protocols for spiral CT angiography. After a prospective analysis, Remy-Jardin et al (J Comput Assist Tomogr, 2000) concluded that reconstructed volume-rendering images have the highest sensitivity (95%) and specificity (100%) compared with those of cross-sectional imaging and image reconstruction with multiplanar and 3D-shaded surface display techniques. In another article, Remy-Jardin et al (AJR Am J Roentgenol, 2000) describe changes in the functional anatomy of the thoracic outlet in 79 patients with symptomatic thoracic outlet syndrome who underwent CT angiography. Degree of Confidence: Most clinicians consider arteriography or venography to be the definitive tests for identifying vascular lesions and their associated complications. Both CT and MR angiography continue to evolve and are relatively new and promising techniques for the vascular evaluation of the thoracic inlet.
Findings: Multiple MR angiographic sequences and protocols can be used to obtain images of the arterial and venous anatomy of the body, including 2-dimensional (2D) or 3-dimensional (3D) time-of-flight, pulse-echo, and phase-contrast imaging. Compared with conventional angiography, MRI combined with MR angiography has the added advantage of enabling visualization of adjacent soft tissue abnormalities, and it is particularly useful in evaluating fibromuscular causes of narrowing. Recently, the specific application of MR angiography to the thoracic outlet has been reported. Dymarkowski et al have reported the use of 3D time-resolved contrast-enhanced MR angiography and T1-weighted spin-echo imaging for the evaluation of vascular causes of the thoracic outlet syndrome. Five patients with symptoms and clinical examination findings suggestive of arterial or venous thoracic outlet syndrome were studied during ipsilateral arm adduction and hyperabduction. In all patients, 3D MR angiography during hyperabduction revealed the location of the vascular compression, while the images obtained during adduction showed normal vessel patency. The stenosis was precisely located with the maximum intensity projection images. In 3 patients, results of conventional angiography performed within a 2-day interval confirmed the location. The T1-weighted images showed the anatomic etiology of the vascular compression, which was confirmed during surgery. The findings of this small study demonstrate the potential of MR angiography in the diagnosis of vascular causes of thoracic outlet syndrome. Demondion et al (2000) also describe the normal MR anatomy of the thoracic outlet and its alteration with positional maneuvers by correlating the gross anatomic images with the corresponding MR images. Degree of Confidence: As discussed above, MRI is a promising noninvasive technique for the diagnosis of vascular causes of thoracic outlet syndrome, allowing the evaluation of both the vascular and soft tissue anatomy of the thoracic inlet. Potential drawbacks of MR imaging include factors that may limit the image quality (see False Positives/Negatives below), and the relatively time-consuming process of obtaining images. Dymarkowski et al noted that the need for a time interval between the acquisition of the 2 series of images was a potential drawback to their approach. Additional larger trials are needed to compare the accuracy of MRI with that of conventional angiography and to confirm the clinical utility of MR angiography. False Positives/Negatives: Several factors may limit the image quality with MRI and contribute to artifacts that reduce its sensitivity. These factors include abrupt changes in the path of a vessel, turbulent flow, changes in the direction of flow relative to the plane of imaging, and patient motion. As MR angiography continues to evolve, newer techniques may be able to overcome many of these pitfalls. The need to study the patient in different positions (eg, in abduction, in adduction) is important for the physician performing MRI because a simple image obtained in the anatomic position may obscure unprovoked vascular compression.
Findings: The arterial system and the venous system of both upper extremities are usually studied during the duplex ultrasonography. The patient lies supine with his or her head turned to the side opposite to that being examined. The arm is initially examined in a neutral position and then at varying degrees of abduction, such as 90°, 135°, and 180°. The subclavian artery and the entire venous circulation, including the internal jugular vein, subclavian vein, axillary vein, and innominate vein (in which the portion just above the superior vena cava may not be visualized), are examined. The criterion for hemodynamically significant venous compression include the obliteration of flow through the subclavian vein or the loss of normal cardiac pulsatility or respiratory phasicity. The criterion for hemodynamically significant arterial narrowing include (1) a 2-fold or greater increase in the peak systolic velocity compared with that measured with the arm in the neutral position or (2) the obliteration of flow. Duplex ultrasonography has been useful in the follow-up care of patients after surgical or radiologic intervention, and a baseline postprocedural examination is routinely performed. Degree of Confidence: See False Positives/Negatives below. False Positives/Negatives: Although ultrasonography is a useful noninvasive test, the false-positive rate with the arterial criteria is 20%, and the rate with the venous criteria is 10%. Another limitation of duplex ultrasonography is the incorrect identification of a large collateral vein as the subclavian vein in subclavian vein thrombosis. Careful delineation of the entire course of the vessel and its relationship to the artery, which normally is posterior to the subclavian vein, is necessary to avoid this mistake. At the present time, another study (eg, conventional angiography) is usually performed to confirm abnormalities identified with ultrasonography.
Findings: Arteriography is the most specific diagnostic examination and is indicated in a patient with ischemic upper extremity symptoms. The entire arterial circulation of the upper extremity is examined from the aortic arch to the distal arteries of the fingers. Access through the common femoral artery is preferred to evaluate both upper extremities, if necessary, and to evaluate various stress positions. In addition, in the presence of subclavian artery occlusion, delayed imaging after injection may be useful to demonstrate antegrade collateral circulation, with distal reconstitution of the artery beyond the point of obstruction (see Image 1). Examination in a minimum of 3 positions may be required to demonstrate findings that may not be present in 1 position but are evident in another. In many patients, smooth extrinsic arterial narrowing is seen only on images obtained during stress. The examination is begun with the patient in the completely adducted neutral position. Although the findings are often limited to minimal narrowing at most (or no findings are seen at all), the neutral position is best for evaluating intrinsic arterial diseases, arterial thrombosis, and poststenotic dilatation or aneurysms. The Lang maneuver then is performed, with the arm abducted to 90° and with the patient lifting a 2-lb weight 2 cm above a tabletop. Images are obtained during deep inspiration and with the patient's head turned sharply to the opposite side. The Lang maneuver, also called the modified Allen maneuver, elicits sites of compression in the costoclavicular space, the scalene triangle, and the costocoracoid space inferior to the pectoralis minor tendon, a site of compression of the axillary artery. However, evidence of minor compression with this exaggerated stress position has also been shown in persons without thoracic outlet syndrome. The Adson maneuver consists of depression of the patient's shoulder with his or her head turned to the symptomatic side. Other positions for imaging include hyperabduction of the arm and the costoclavicular maneuver; the clinical examinations with both of these positions are equivalent (see Preferred Examination; Images 2-3). If technically feasible, an evaluation of the patient under conditions that produce the symptoms should be considered. These conditions may include various sitting positions with the arm abducted. Frequently, findings are readily apparent, with severe narrowing of the subclavian artery accompanied by poststenotic dilatation or a subclavian artery aneurysm. Such findings are strong indicators of a hemodynamically significant lesion. Other findings that may indicate extrinsic compression of the subclavian artery include the following: (1) a ridgelike defect of the inferior margin, which indicates compression against the first rib; (2) a similar defect combined with an impression along its superior margin, which indicates narrowing of both the scalene triangle and the costoclavicular space; and (3) a tapered cutoff of the artery as it emerges from the scalene tunnel, which indicates compression within the scalene triangle and the scalene tunnel. Another finding that is not accepted as a strong indicator of a hemodynamically significant lesion includes an oblique compression defect or a twist of the subclavian artery as it passes through the scalene tunnel. Venography Ascending brachial venography is the preferred and most definitive examination for diagnosing venous thrombosis. Collateral veins are more evident with venography than with ultrasonography, and therapeutic techniques, such as thrombolysis, can be readily performed if necessary. Venography is indicated in patients with definite symptoms of venous obstruction or when a duplex ultrasonographic finding suggests venous abnormalities. During the procedure, the patient's arm is placed in slight abduction to prevent artificial venous occlusion. Because the cephalic vein may lead to collateral vessels that bypass the site of thrombosis in the subclavian or axillary veins, the basilic vein is injected to ensure complete opacification around the venous thrombosis. One potential drawback of venography is that the proximal extent of the thrombus may be difficult to accurately identify. Two major findings of upper extremity venous thrombosis are described. The first finding is a short area of obstruction at the subclavian-axillary vein junction between the first rib and the clavicle at the thoracic inlet. A variable number of adjacent collateral veins are visualized. This finding is often seen with acute obstruction. The second finding is a long area of obstruction extending distally from the axillary vein and possibly into the brachial vein. Approximately 75% of cases also involve the subclavian vein, though innominate vein involvement is rare (see Image 4). Chronic and intermittent venous obstruction may be seen as an area of scarring or narrowing at the subclavian-axillary vein junction, with or without a thrombus. Degree of Confidence: At the present time, most clinicians consider catheter-based arteriography or venography to be the criterion standard for evaluation of the vascular anatomy of the thoracic outlet. MR angiography and CT angiography, however, are 2 promising and relatively new techniques that may be able to replace many diagnostic conventional angiographic examinations. Further clinical trials are required to compare the accuracy of these new techniques with that of conventional angiography before they can be considered acceptable alternatives in clinical decision making.
Intervention: In a patient presenting with acute symptoms of arterial thoracic outlet syndrome with associated severe ischemia of the extremity, emergency surgery is the preferred treatment to restore circulation. However, if extensive distal thrombosis or embolization is present, transcatheter thrombolysis of the occluded runoff or emboli should be considered prior to surgical repair of the causative central lesion. Goals of surgery include the following: (1) restoration perfusion to the distal extremity (eg, with thromboembolectomy), (2) repair of the anatomic anomaly causing the narrowing of the thoracic outlet (This commonly involves excision of the first rib and other abnormal bone and soft tissue.), and (3) reconstruction of the arterial segment in the thoracic outlet, which is often the source of the emboli. Surgical results for arterial complications are most favorable with prompt diagnosis and treatment and prior to the onset of irreversible ischemia. In a review of 137 patients by Sanders and Haug, 84% of patients had successful results after surgical treatment for arterial thoracic outlet syndrome. Recently, percutaneous transluminal angioplasty has gained favor, especially in patients who are poor surgical candidates or in patients with mild arm claudication or weakness. Several groups have investigated the long-term results of angioplasty for the treatment of obstruction or stenosis in the subclavian arteries. Farina et al compared the outcomes in 21 patients undergoing proximal subclavian angioplasty and in 15 patients undergoing surgical carotid-subclavian reconstruction. Patency rates for angioplasty progressively decreased from an initial rate of 91% to 54% at 5 years, but the rates for surgical reconstruction remained unchanged at approximately 86%. A review by Hebrang et al of 45 patients undergoing subclavian angioplasty demonstrated normal blood pressure findings in the treated arm in 91% of patients, with a mean follow-up of 29 months. A review by Wilms et al revealed continued relief of symptoms after 25 months in 86% of 21 patients. Recently, Korner reported a secondary cumulative patency rate of 72% after 100-month follow-up in 28 patients who underwent subclavian and innominate angioplasty. Thrombolysis is a potential treatment option, and it can be considered immediately after a diagnostic angiogram reveals subclavian or axillary occlusion. As part of a larger review of vascular causes of thoracic outlet syndrome, Hood et al recently reported the results in 3 patients with acute upper extremity emboli. Two of these patients received thrombolytic therapy and were without symptoms after 22-month follow-up. Debate continues about the role of angioplasty and thrombolysis versus that of surgery in a patient with acute symptoms of arterial thoracic outlet syndrome. If angioplasty is performed, careful follow-up is important, and Doppler sonography should be performed on a regular basis to evaluate for the recurrence of stenosis. Venous procedures Prognosis and treatment differ for the 2 types of venous thoracic outlet syndrome. Patients with primary venous thrombosis more commonly present with acute symptoms, including arm swelling and pain, which often limit their activity. Anticoagulant therapy reduces further extent of thrombus, as well as the associated minute risk of pulmonary embolism. However, multiple authors have demonstrated minimal long-term benefits of anticoagulation. Intervention is often performed early for rapid symptomatic relief, especially in an otherwise healthy patient who requires full use of the affected arm. In recent years, catheter-directed thrombolysis and mechanical thrombectomy have significantly expanded the nonsurgical options for treatment. With surgical thoracic outlet decompression, its success rate has averaged 81%. Other advantages to catheter-directed thrombolysis include the ability to perform diagnostic venograms at the time of thrombolysis in various positions to provoke the symptoms and the ability to perform postthrombolysis venograms to document the extent of residual thrombus. Disadvantages of thrombolysis include the risk of bleeding, which can be minimized by following prescribed dosing guidelines. Another concern is the fact that the underlying thoracic outlet compression is not addressed; therefore, a surgical procedure may still be needed later. Thrombolysis is most effective if performed within 7-10 days; at least 2 studies have shown a significant decrease in the effectiveness of thrombolysis after 10 days. The timing of thoracic outlet decompressive surgery after thrombolysis has been a source of considerable debate in the literature. The most common approach involves a waiting period of approximately 3 weeks, during which the patient received oral anticoagulation and the vascular endothelium is allowed to heal. Then, decompressive surgery is performed if a postthrombolysis venogram shows focal extrinsic compression. If extrinsic compression resulting from an anatomic cause is not noted on the follow-up venogram, the need for surgical intervention is less clear. In a survey by Rutherford and Hurlbert, 86% of vascular surgeons surveyed opted for a conservative approach in this scenario, with anticoagulation therapy administered for 3-6 months, during which the patient is observed for evidence of recurrent thrombosis. If the postthrombolysis venogram reveals an intrinsic stenosis with or without extrinsic compression, percutaneous venoplasty augmented by stent placement becomes an option, although the precise role of venoplasty and stent use remains an area of considerable disagreement. Most authors do not recommend stent placement without surgical decompression because the stent itself may be compressed or become fragmented by the thoracic outlet narrowing. Dowling et al reported a case of venous thoracic outlet syndrome treated with thrombolysis, angioplasty, and stent placement without immediate first-rib resection. The case was later complicated by stent fracture. Meier et al reported a series of 6 patients who underwent venous stent placement immediately after thrombolysis for venous thoracic outlet syndrome, and 2 patients who underwent delayed stent procedures. Two of the 6 patients who underwent immediate stent placement did not undergo immediate surgical rib resection, and both patients had the complication of stent fracture. Long-term (1- to 3-years) patency was achieved in 6 of the 8 patients. The timing of the interventions continues to be debated. Some authors recommend a phase of venous intimal healing after thrombolysis and before venoplasty or stent placement to reduce further damage to the vessel resulting in recurrent thrombosis. Other authors have proposed the use of venoplasty only after surgical resection of the first rib, when the source of the thoracic outlet compression has been removed. Molina has proposed that thrombolysis be performed first, followed by emergency surgery, for the highest likelihood of avoiding recurrent strictures and chronic symptoms. Kreienberg et al recently reported their outcomes that support early surgical and radiologic intervention. Twenty-three patients with venous thoracic outlet syndrome were treated with thrombolysis, followed by immediate (within 24 h) surgical thoracic inlet decompression (including rib resection and removal of the anterior scalene muscle) and then angioplasty within 24 hours. Stents were placed to treat residual venous stenosis (>50%) in 14 patients. Of the veins treated with only angioplasty, all were patent at a mean follow-up of 4 years. Of those additionally treated with stent placement, 9 were patent at a mean follow-up of 3.5 years. No stent fractures were observed. Occluded stents were associated with longer stenoses and hypercoagulable states. From these findings, Kreienberg et al concluded that early surgical intervention followed by early radiologic intervention is safe and effective and that subclavian venous stent use is effective in short venous stenoses. If the postthrombolysis venogram demonstrates inadequate thrombolysis or residual obstruction, most vascular surgeons favor discontinuation of thrombolysis and initiation of a conservative treatment approach with anticoagulation, elevation, and support of the arm. According to Rutherford and Hurlbert, only one third of surgeons favor an attempt at venoplasty with or without stent placement (see Images 5-6) and only 15% favor surgical decompression. If the patient remains symptomatic with residual stenosis, 80% of vascular surgeons favor surgical intervention with short stenosis (66% favoring jugular vein turndown, 14% favoring claviculectomy) and 48% favor a bypass (either saphenous vein interposition or cephalic vein crossover) with long stenosis. These findings reflect the overall success of surgical intervention. Secondary venous thrombosis, often associated with central venous catheters, usually occurs with an insidious onset and minimal symptoms if any. Anticoagulation with heparin, followed with the long-term administration of warfarin, is the preferred treatment. In uncomplicated cases, thrombolysis has not had a definite benefit, and it does have significant associated complications. In patients with a definite contraindication to anticoagulation, McCarthy et al reported success with simple conservative measures such as arm elevation and compressive dressings, and they propose reserving invasive therapies, such as thrombolysis, surgical thrombectomy, and rib resection, for patients with severe symptoms not responsive to anticoagulation. Interventional therapies, such as angioplasty and thrombolysis, are often more difficult to perform in secondary venous thrombosis because these cases are more likely to be chronic. Medical/Legal Pitfalls:
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