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Ann Thorac Surg 2000;69:1663-1668
© 2000 The Society of Thoracic Surgeons
a Division of Thoracic and Cardiovascular Surgery, The University of Texas Southwestern Medical School and Baylor University Medical Center, Dallas, Texas, USA
Address reprint requests to Dr Urschel, 3600 Gaston Ave, Suite 1201, Barnett Tower, LB 161, Dallas, TX 75246
e-mail: urschel1{at}airmail.net
Presented at the Forty-sixth Annual Meeting of the Southern Thoracic Surgical Association, San Juan, Puerto Rico, Nov 4–6, 1999.
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Abstract |
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Methods and Results. Group I (35 extremities) was initially treated with anticoagulants only. Twenty-one developed recurrent symptoms after returning to work, requiring transaxillary resection of the first rib. Thrombectomy was necessary in eight. Group II (36 extremities) was treated with thrombolytic agents initially, with 20 requiring subsequent rib resection after returning to work. Thrombectomy was necessary only in four. Of the most recent 241 extremities (group III), excellent results accrued using thrombolysis plus prompt first rib resection for those evaluated during the first month after occlusion (199). The results were only fair for those if seen later than 1 month (42).
Conclusions. An early diagnosis (less than 1 month), expeditious thrombolytic therapy, and prompt first rib resection are critical for the best results.
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Introduction |
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The availability of thrombolytic agents [7–11], combined with prompt surgical neurovascular decompression of the thoracic outlet [5], have reduced morbidity and necessity for thrombectomy, substantially improving clinical results including the return to work. For those seen late (greater than 6 weeks after the thrombotic episodes), thrombolytic agents followed by first rib resection have not been as successful.
The purpose of this report is to define the evolution of therapy in patients with effort thrombosis secondary to thoracic outlet syndrome (TOS) [12–15] and compare it with the national history of the disease [16–18].
The axillary subclavian vein traverses the tunnel formed by the clavicle and subclavius muscle anteriorly, the scalenus anticus muscle laterally, the first rib posterior-inferiorly, and the costoclavicular ligament medially (Fig 1). Abnormalities of any of these structures, whether congenital, traumatic, or distorted by unusual exercise or physical stress, may narrow the tunnel anatomically. With such an external anatomical constriction, on which is superimposed dehydration, thrombogenicity, excessive exercise, or activity, a patient may have an increased chance of thrombosis.
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Material and methods |
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Diagnosis
In 312 extremities, swelling or venous distention over the chest, arm, or hand occurred, suggesting the clinical diagnosis of venous obstruction (Table 1). Elevation of the arm or hand did not seem to change the configuration of the veins or swelling acutely. Bluish discoloration was observed in 24 arms, and aching pain, which was increased by exercise, occurred in 207. Cervical ribs were noted in 62 instances. The bilateral syndrome occurred in 18 patients (12 females and 6 males), simultaneously in 2 (1 with previous bilateral clavicular fractures), and sequentially in 16. In 24 patients, only minimal symptoms were present.
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The diagnosis was established by clinical history, physical examination, and venogram performed through a medial antecubital vein. An indwelling plastic catheter was inserted into the antecubital vein after 1980 so that serial venograms and thrombolytic injections could be performed.
Substantial narrowing or occlusion of the axillary subclavian vein in the area of the first rib and clavicle was observed in all patients. Some collateral circulation was evident in 280 extremities, although it was obviously not adequate if swelling was present. The pathways for the most efficient collateral communication were between the cephalic, transverse cervical, transverse scapular, and tributaries of the internal jugular veins.
Differential diagnosis included lymphangitis or lymphatic obstruction, intramuscular hemorrhage, and intermittent venous obstruction without thrombosis as well as venacaval or innominate vein obstruction.
Surgical considerations
Indications
Indications for surgery included: (1) persistent symptoms such as aching pain and the feeling of a "heavy" or "fatigued" arm, and swelling of the extremity with or without venous distention; and (2) a venogram positive for significant extrinsic compression (in either the anatomic position or a challenging position such as the Adson’s), and after thrombolytic treatment regardless of whether it was successful or not.
Operative technique: transaxillary resection of the first rib and neurovascular decompression
The patient is placed in the lateral position with the involved extremity gently supported by forearm traction straps attached to an overhead pulley with 1 to 2 pounds of weight. An arm holder provides appropriate traction and relaxation, and prevents hyperabduction of the shoulder beyond 90°. After prepping and draping the axilla and forearm, a transverse incision is made below the axillary hairline between the pectoralis major and latissimus dorsi muscles. The dissection is carried through the skin, subcutaneous tissue to the chest wall, and extended cephalad to the first rib. Care is taken to prevent injury to the intercostal brachial cutaneous nerve, which passes between the first and second ribs to the subcutaneous tissue in the center of the operative field. With gentle dissection, the neurovascular bundle is identified and its relation to the first rib and both scalene muscles is clearly outlined to avoid injury to these structures. The scalenous anticus muscle is divided and resected up into the neck to avoid reattachment to Sibson’s fascia. The lung on the operated side is temporarily collapsed with a double-lumen endotracheal tube to expedite safe dissection. The first rib is dissected subperiostally and carefully separated from the underlying pleura to avoid pneumothorax. The rib is divided and a triangular portion removed with the vortex of the triangle at the scalene tubercle. The anterior portion of the rib is dissected carefully from the vein, the costoclavicular ligament is divided, and the rib is divided at its sternal cartilaginous attachment. All compression bands and adhesions are removed from the axillary subclavian vein, and the anterior venous compartment is thoroughly decompressed.
The posterior segment of the rib is carefully dissected subperiostally from the subclavian artery and brachial plexus posteriorly. The scalenus medius muscle is dissected from the rib. The rib is divided near its articulation with the transverse process of the vertebra. Complete removal of the neck and head of the first rib is achieved by long, specially reinforced double-action Urschel-pituitary and Urschel-Leksell rongeurs. The eighth cervical and first thoracic nerve roots and the brachial plexus undergo careful neurolysis. If a cervical rib is present, it is removed and the seventh cervical nerve root is decompressed. Meticulous hemostasis is accomplished. Only the subcutaneous tissues and skin require closure, because no large muscles have been divided. The patient is encouraged to use the arm normally and can usually be discharged from the hospital on the second day after the surgical procedure.
It is preferable to remove the entire first rib, including head and neck, to avoid future bony or (fibrocartilage) regeneration and irritation of the plexus [19].
For recurrent symptoms, removal of incompletely resected or regenerated rib and lysis of adhesions can best be accomplished through the posterior high thoracoplasty approach.
General surgical observations
Through the transaxillary approach, the vein is one of the landmarks for the dissection of the first rib because of its blue color. In patients with Paget-Schroetter syndrome, the vein is generally occluded or markedly thickened and there is usually no blue color. It is the same color as the other structures, such as the artery, muscle, etc. Because of this, many surgeons who do not routinely perform this operation have difficulty locating the anatomic structures. This is important to understand, and other landmarks must be used to provide the proper orientation. Extremely long surgeries (up to 8 hours) have been reported by surgeons who cannot seem to find the vein when it is totally occluded. This increases the incidence of nerve injuries.
In most patients that have been operated less than 6 weeks after thrombosis, there is usually a severe inflammatory reaction around the neurovascular structures and first rib. These structures may be "plastered down" to the first rib making the dissection hazardous. Several cases that had previous breast implants implanted through the axilla were included in this group of patients. Because of this, structures such as the vein, artery, and brachial plexus may be intimately adherent to the first rib, significantly increasing symptoms and increasing the risk of the operative procedure. This should be suspected in such situations and extra care should be taken not to injure any of the neurovascular structures.
If an interval clot has occurred that was not relieved by the thrombolytic agents, thrombectomy may be necessary. It is difficult in this area because of achieving proximal venous control, and avoiding "air-sucking" into the vein, bleeding, etc. If the external venous compressive structures are removed, the clot usually lyses spontaneously, the patient recanalizes, or it is opened with thrombolytic agents postoperatively (after 10 days). "Roto-rooter" techniques have not been ideal.
Treatment protocols
Group I
Thirty-five extremities were treated before 1980 with anticoagulant therapy (heparin sodium followed by coumadin [crystalline warfarin sodium]). All were seen before 6 weeks from the time of thrombosis. The treatment was usually initiated in the hospital. This group was instructed to elevate their arm and all patients were encouraged to return to work. Recurrent symptoms similar to those of the initial syndrome developed in 26 of those 35 extremities after the patient returned to work. Twenty-one were readmitted to the hospital, and after coumadin therapy was stopped, a transaxillary resection of the first rib was performed with removal of all compressive elements including the congenital or adhesive bands, the costoclavicular ligament, and the scalenus anticus muscle. Eight extremities required thrombectomy. The axillary subclavian vein was opened transversely and a Fogarty catheter was used to remove clot from inside the vein. It was not necessary to patch the venotomy. Proximal control was difficult (Table 2).
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Group II
A second group of 36 extremities was treated after 1980. These were managed initially with heparin and a venogram. Thrombolytic agents were administered in most instances through an indwelling catheter in the antecubital vein. After lysis of the thrombus, first rib resection was performed promptly. In the first 10 patients, the thrombolytic agent was streptokinase with the initiating dose of 250,000 U followed by 100,000 U/h intravenously for 24 to 48 hours or until lysis of the clots occurred. This was monitored with prothrombin times (PT) and partial thromboplastin times (PTT). The operation was "safe" 4 hours after the discontinuation of the streptokinase. No streptokinase was given postoperatively for at least 10 days because of the potential bleeding hazard. If a bleeding state were to develop, reversal of streptokinase was possible with Amicar (aminocaproic acid), fresh-frozen plasma, cryoprecipitate, or fresh whole blood. All patients were tested for the "hypercoagulable" state before surgery.
Streptokinase acts directly on the plasminogen, which is converted to plasmin and activates the body’s thrombolytic mechanism. This may produce a systemic reaction and involves no heparin or antiplatelet therapy. The half-life of heparin is 90 minutes and the half-life of streptokinase is 83 minutes. One drug can be exchanged for another in approximately 4 hours.
Because of the disadvantage of the systemic effect of streptokinase, it was replaced with urokinase in 26 extremities. The loading dose was 4,400 U/kg by bolus, and a dosage of 4,400 U/kg/h was given until clot lysis occurred (mean duration of treatment was 26 hours). Assessment of the therapeutic effect included frequent observation as well as serial venograms through the indwelling catheter. Most patients showed major improvement in less than 24 hours after administration of the thrombolytic agent.
Because of the experience of recurrent symptoms after discharge of group I, the 36 extremities in group II were treated promptly with surgery. Heparin and thrombolytic therapy were stopped and the first rib was removed through a transaxillary incision in 34 extremities, or in a combined transaxillary supraclavicular approach in 2 patients [5, 21, 22]. In addition, complete division of the costoclavicular ligament, the scalenous anticus muscle, and any bands or adhesions was performed. Scalenectomy was extensive to minimize the possibility of muscle fibers reattaching to Sibson’s fascia. Any congenital or compressive bands were removed from the axillary subclavian vein. Venograms were occasionally obtained when the patient was on the operating table, but usually direct observation was adequate to assess patency of the vein. After removing all external compressive structures, if there were any question regarding complete patency, thrombectomy was performed. This was necessary in 4 patients. The apparent suggestion of retained clot and failure of the thrombolytic agents to completely lyse the clot was usually explained at operation by external compressive elements alone, even when there appeared to be "residual clot" on the venogram. The patients were discharged after several days and were followed clinically without anticoagulants. After 6 weeks, they were allowed to return to work.
Group III
The most recent 241 extremities were treated similarly to group II. One hundred ninety-nine extremities (group III) were evaluated within the first 6 weeks after the thrombosis and underwent thrombolytic therapy and prompt transaxillary first rib resection. Thrombectomy was only required in the two extremities.
Group IV
Forty-two extremities were not seen until after 6 weeks from the time of venous occlusion. All were treated similarly to group II. In only 50% of these patients was it possible to decrease the amount of clot with urokinase infusion. However, none could be completely opened. All underwent prompt transaxillary first rib resection. Twenty-four of the 42 subsequently recanalized spontaneously and became "symptomatic." Of the 18 patients with persistent occlusion, 6 demonstrated a severe postphlebetic syndrome, 5 had mild symptoms, and 3 had attempted "Roto-rooter" destruction of the obstruction with only partial relief of symptoms (3 of 6 with postphlebetic syndrome did not respond to "roto-rooter" therapy). No thrombectomy was performed in this group. Three venous bypass procedures were performed without prolonged success in the postphlebetic group (a reversed saphenous vein was sutured from the axillary to jugular vein). Most of these patients were on coumadin before evaluation, and it was continued postoperatively in some of the patients to "improve recanalization."
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Results |
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Group III patients were those seen less than 6 weeks after thrombosis and treated similarly to group II. One hundred eighty-nine had good to excellent results. Eight were fair and two could not be opened.
All patients in group IV were seen 6 weeks or more after thrombosis and also treated similarly to group II. Although none could be opened with thrombolytic agents, all were treated promptly with surgery and 24 recanalized subsequently or developed excellent collateral circulation with good results later, whereas 6 had only fair results, as did 3 more who were opened with "Roto-rooter" treatment and surgery; 9, however, did not open or develop collateral circulation. These were not clinically improved, with 5 exhibiting a severe postphlebitic syndrome. Coumadin did not seem to improve results, but the patients were not randomized for this therapy. None of the three venous bypass grafts remained open after 3 months in the later group.
There was no mortality in any group and no observed evidence of pulmonary embolism.
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Comment |
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DeWeese and colleagues [3] reported long-term results in patients treated conservatively with elevation and coumadin. There was a 12% incidence of pulmonary embolism, which was not observed in any of our patients. Development of occasional venous distention occurred in 18%, and late residual arm symptoms of swelling, pain, and superficial thrombophlebitis were noted in 68% of the patients (deep venous thrombosis with postphlebitic syndrome). Phlegmasia cerulea dolens was present in 1 patient.
These findings substantiate our observations from group I that a more aggressive operative approach after thrombolytic therapy is indicated. This is particularly true for younger patients with "precipitating" factors.
One advantage of urokinase over streptokinase is the direct action of urokinase on the thrombosis distal to the catheter, producing a more localized thrombolytic effect [10, 11]. Streptokinase produces a systemic effect involving the alteration of serum plasminogen and increasing potential complications. A decrease in the need for thrombectomy after use of the thrombolytic agent followed by aggressive surgical intervention is another advantage, as some of the long-term disability is related to more morbidity from thrombectomy as well as recurrent thrombosis [3, 5, 23]. Recently, urokinase has been withdrawn from the market and TPA (Alteplase-Activase; Genetech, Inc, South San Francisco, CA) has been substituted.
The results in group II patients (aggressive thrombolytic therapy with urokinase and surgical resection of the first rib) are in marked contrast to those in group I patients. There was a lack of serious complications in group II, and a high number of patients were able to return to work after 6 weeks.
The natural history of Paget-Schroetter syndrome suggests moderate morbidity [3, 5, 18] with conservative treatment alone. Bypass with vein or other conduits [24–26] has limited application in our experience. Causes other than thoracic outlet syndrome must be treated individually using the basic principles mentioned. Intermittent obstruction of the subclavian vein [27] can lead to thrombosis, and decompression should be employed prophylactically.
In the patients that were seen 6 weeks or more after thrombosis, thrombolytic agents were usually ineffective in completely removing the clot. The more aggressive "Roto-rooter" techniques were helpful in only 3 patients, but usually we are not able to penetrate the hardened fibrotic area. Some of these patients did recanalize at a later stage (24 of 42 patients recanalized spontaneously and became "asymptomatic" after first rib resection). Of the 18 patients with persistent occlusion who did not recanalize after first rib resection, 5 developed severe postphlebitic syndrome, 6 had moderate symptoms, and 3 responded to "Roto-rooter" destruction of the clot with the relief of symptoms. "Roto-rooter" also failed in 4 cases and the clot was unable to be opened.
It is obvious that the earlier that a patient with Paget-Schroetter syndrome ("effort" thrombosis) can be seen by the physician and treated with thrombolytic agents followed by prompt first rib resection, the better the results. The longer the time interval between the acute thrombolytic episode and therapeutic intervention, the less effective the therapy. Efforts at thrombolysis, or "Roto-rooter" techniques, have not been successful after 3 months.
In our experience, there is little evidence that long-term coumadin or heparin therapy has any benefit, either after therapy or for conservative treatment of patients who remain occluded. Certainly, thrombogenic states such as dehydration should be avoided.
A summary of the operative procedures for relieving TOS compression [28] and the reasons for their failures [29] should be reviewed.
Summary
In Paget-Schroetter syndrome, the earlier the diagnosis and treatment, the better the results. Early venogram with thrombolytic urokinase to open the thrombosed axillary subclavian vein is the preferred treatment. Prompt transaxillary first rib resection and neurovascular decompression, particularly in the area of the axillary subclavian venous canal, should be expedited. Anticoagulants did not appear helpful after this procedure.
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