The treatment of Paget-Schroetter syndrome or effort

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1 Reoperations After Failed Transaxillary First Rib Resection to Treat Paget-Schroetter Syndrome Patients J. Ernesto Molina, MD Division of Cardiothoracic Surgery, University of Minnesota, Medical School, Minneapolis, Minnesota Background. A series of 15 patients previously treated for Paget-Schroetter syndrome with a transaxillary first rib resection (TARR) were seen with recurrent thrombosis. Methods. Ten were reoperated using an anterior subclavicular approach. The time of reoperation ranged from 5 months to 7 years (mean, 23.4 months). All patients had been subjected to multiple balloon plasties and 4 of them in addition had up to 3 stents implanted, which also failed. Reevaluation was done with venography. Ten patients were considered to be still salvageable and were reoperated, but 5 were inoperable due to progressive obliteration of the venous channel as early as 2 weeks after TARR. Results. All 10 patients had successful reestablishment of the subclavian vein patency and caliber and have remained patent without anticoagulants. Conclusions. The patients who re-thrombose or remain obstructed after TARR should be reoperated instead of resourcing to implanting stents or multiple balloon plasties that invariably fail, and patients should not be kept on anticoagulation indefinitely hoping to maintain the vein open. (Ann Thorac Surg 2011;91: ) 2011 by The Society of Thoracic Surgeons The treatment of Paget-Schroetter syndrome or effort thrombosis of the subclavian vein has undergone significant evolution during the past 20 years. With the advent of thrombolytic agents and thrombectomy catheters the current standard of care entails a prompt implementation of either method to accomplish rapid resolution of the clot [1 4]. After total clearing, the venography shows the extent and the severity of the obstruction in order to plan the surgical intervention which is usually necessary [5 8]. When properly implemented the operation should be conducted as soon as the vein is clear of thrombus [3, 9, 10]. Postponing or delaying the surgical procedure leads to further fibrosis of the vein wall with progressive obliteration involving the axillary and even more distal veins into the arm. The later the surgery is carried out the lower the chances of curing the patient. Waiting is a common mistake incurred by the consulting surgeon unfamiliar with the evolution of this disease. Several surgical approaches have been used in the past with various results. An ideal operation to treat this syndrome is aimed to attain a permanent cure of the situation, rendering a normal caliber of the subclavian vein and eliminating the compression mechanism that causes its obstruction. Because of the popularity of the transaxillary first rib resection (TARR), introduced by Roos back in 1966 [11] to treat thoracic outlet syndromes causing compression of the brachial plexus and in 50% of Accepted for publication Feb 17, Address correspondence to Dr Molina, Division of Cardiothoracic Surgery, University of Minnesota Medical School, 420 Delaware St SE, MMC 207, Minneapolis, MN 55455; molin001@umn.edu. the time also arterial compression [12], this operation has been applied for decompression of the subclavian vein which occupies a much more anterior position in the thoracic outlet. It appears that this approach in some cases may lead, however, to incomplete or ineffective decompression of the subclavian vein due to the inability of the surgeon using this route to remove the costoclavicular ligament, the subclavius tendon, and the most anterior portion of the first rib, leaving the extrinsic mechanism of obstruction intact (Figs 1; 2A). Consequently, some patients after undergoing transaxillary intervention suffer reobstruction of the vein. When this occurs the patients are frequently referred for balloon dilation of the vein and often also for implant of endovascular stent. Most of the time this secondary approach does not solve the problem because the site and the mechanism of the obstruction remain untouched [13]. Balloon dilation alone often fails to maintain the proper caliber of the vein because the fibrous vein wall collapses back as soon as the balloon is deflated. The fibrous process continues and eventually the vein channel reobstructs weeks or months later. If a stent is implanted it often kinks at the site of the obstruction and the vein rethromboses, making further percutaneous interventions more complicated or ineffective (Fig 3A). These patients are often kept on anticoagulants indefinitely. When facing this situation the only solution is to reoperate to correct the problem. Material and Methods The Institutional Review Board of the University of Minnesota under HSC:1010M91832 approved this study. We evaluated 15 patients referred to us from other institutions where no other options were offered to these 2011 by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur

2 1718 MOLINA Ann Thorac Surg REOPERATIONS FOR PAGET-SCHROETTER 2011;91: Fig 1. Computed tomographic scan of the chest with tridimensional reconstruction after transaxillary removal of the first rib. It shows the presence of a significant residual first rib stump in the anterior and posterior portions (arrows). patients. They had undergone transaxillary resection of the first rib after initial successful clearing of the clot using thrombolytics. The transaxillary removal of the first rib was done at various intervals but usually late, ranging from 3 to 10 weeks after the initial lysis of the thrombus (Table 1). The patients were not restudied immediately after surgery by venography to verify the vein status, but only when they continued complaining of edema; usually weeks or months later. Venogram showed obstruction of the subclavian vein at the typical site of the thoracic inlet. Fourteen had been subjected to repeated balloon dilations which were ineffective despite using large diameter balloons. The vein invariably collapsed right back as soon as the balloon was deflated, and as a consequence endovascular stents were implanted in 4 patients. On repeated venography 10 were found to have still acceptable inflow caliber of the axillary vein (about 1 cm in diameter). These patients therefore were reoperated at this time using the subclavicular anterior approach, standard in our institution [3, 14]. Their ages ranged from 15 to 39 years all involved in active occupations or sports (Table 1). There were 7 women and 3 men otherwise in good health. Despite the failure resulting from repeated balloon dilations, patients no. 9 and no. 10 were subjected to 14 and 7 sessions, respectively. The remaining patients were referred to us after 1 to 3 attempts. Four patients (nos. 1, 2, 7, 9) had 1 to 3 stents implanted. All were on chronic anticoagulation with warfarin (Coumadin; Bristol-Myers Squibb Co, Princeton, NJ), which had been implemented continuously from 5 months to 7 years. Among the 15 patients seen, there were 5 who came from 2 weeks to 11 months after their TARR first operation. They were found to have no discernable veins on venography and were therefore inoperable. All were symptomatic with moderate to severe edematous limbs and unable to do their occupation or simple daily normal activities like driving. Briefly, the reoperation entails an anterior subclavicular approach previously published from our institution [14 16]. After reaching the retropectoral space, the stump of the first rib usually measuring from 1½ to 2 inches long, which is mostly cartilage, was found always present. This is resected. In addition the costoclavicular ligament, as well as the subclavius tendon (which insert on the rib), are removed as well (Fig 4). After removing all these structures the subclavian vein is opened lengthwise and a vein patch using a piece of saphenous vein harvested from the upper thigh is laid in to enlarge the vein diameter. If a stent had been previously implanted, the vein which is opened lengthwise requires the use of strong scissors to cut through the metal of the stent. Even though the vein is made wider with the vein patch it invariably collapses back due to the thickness and rigidity of the vein wall, particularly posteriorly, caused by fibrosis and the old stent. In only one instance were we able to remove one of the stents totally. After the patch is implanted the patient undergoes immediate postoperative venography and a final implant of a new stent which, now without any kinking caused by extrinsic compression, functions very well and keeps the vein open. The subclavicular approach with or without transsternal extension, which is sometimes necessary [15, 16], allows adequate exposure of the subclavian vein directly to permit placing vascular clamps medially and distally in order to open the vein and implant a widening patch to restore the proper lumen to the vein at the site of the Fig 2. (A) Patient no. 4. Venogram obtained after a transaxillary resection of the first rib showing the significant indentation and obstruction of the vein caused by the residual first rib stump under the subclavian vein (arrow). (B) Postoperative venogram obtained after the patient was reoperated through a subclavicular anterior approach with removal of the residual rib remnant as well as removal of the costoclavicular ligament and the subclavius tendon. The patient underwent patch angioplasty of the subclavian vein followed by placement of a stent.

3 Ann Thorac Surg MOLINA 2011;91: REOPERATIONS FOR PAGET-SCHROETTER 1719 Fig 3. (A) Patient no. 1. The results after transaxillary resection of the first rib followed by implant of two stents, both of which became obstructed. The venograms show clearly the kinking of the metal stent in the subclavian vein. (B) Results after the patient was reoperated through a subclavicular anterior approach with removal of the residual rib and the ligaments causing the obstruction of the vein. This operation required the implant of a new stent, which functioned quite well. stenosis. Intraoperatively the patients are given heparin at a dose of 100 units per kg weight which is not neutralized at the end of the procedure. Rheomacrodex (Medisan Pharmaceuticals Inc, Parsippany, NJ) is started with a bolus of 50 ml. Postoperatively the patients also are given low molecular weight heparin subcutaneously (40 mg subcut twice-a-day for 48 hours). They are placed on Plavix (Sanofi-Aventis, Kansas City, MO) starting the same evening of the surgery at 75 mg and continued daily with the same dose. Rheomacrodex (low molecular weight dextran 40) as an antiplatelet agent is administered continuously at the rate of 15 cc per hour for 48 hours [3, 8] until the proper level of anticoagulation is attained (international normalized ratio 2-3). In the late postoperative period the patient is kept on anticoagulation only, using Coumadin and Plavix for 12 weeks, repeating an ultrasound at 6 and 12 weeks to verify the patency of the vein. At the end of 12 weeks the patient is allowed to reassume all type of physical activities without restrictions. After the last ultrasound, with the patient being asymptomatic, he is taken off all types of anticoagulation. This method of treatment has been published several times and has proven to be effective in providing 100% permanent patency of the vein with no recurrence of the obstruction [3, 10]. Results As with any reintervention, the conduction of this operation is somewhat difficult, and because the patients have undergone a previous rib resection often enough the pleura is firmly attached to the rib remnant; in 2 patients the pleura was entered in a small area that required a chest tube insertion to reexpand the lung. Because 10 of these 15 patients had adequate inflow from the axillary vein (1-cm diameter) they were considered operable. Upon opening of the vein the flow coming from the arm was always torrential. The other 5 inoperable patients had complete absence of the vein channel and only a network of collaterals around the shoulder and axilla. In 2 operable patients, in whom stents had been originally implanted far into the innominate vein, we had to extend our incision transsternally to the center of the manubrium and up to the sternal notch in a right angle fashion, as described in previous publications, to expose the entire innominate-subclavian venous complex [16, 17]. When the manubrium of the sternum is cut, the patient must have the arm immobilized postoperatively with an arm sling for 4 to 6 weeks until the sternum is firmly healed. This measure is very important in order to prevent sternal dehiscence. There were no bleeding complications. The venous stents previously implanted usually could not be removed. We were able to remove Table 1. Patients With Previous Transaxillary First Rib Removal Who Subsequently Reoccluded the Vein and Were Reoperated by the Authors Patient Number Age (Years) Sex Time of TARR After Lytic Therapy Time Found Re-occluded Number of Repeated Percutaneous Manipulations Stents Implanted Time Before Reoperation 1 26 F 5 weeks Immediately 3 2 2y9mo 2 39 F 4 weeks 1 month 3 1 2y6mo 3 31 F 10 weeks 2 days 1-5 mo 4 15 M 4 weeks 2 days 2-9 mo 5 42 F 6 weeks 6 months 0-1 ½ y 6 38 F 3 weeks 2 weeks 2-10 mo 7 39 F 3 weeks 1 month 2 1 1y6mo 8 33 M 5 weeks 4 months 1-1y3mo 9 26 F 2 weeks 2 months y M 1 month 2 months 7-1 y mo month(s); TARR transaxillary first rib resection; y year(s).

4 1720 MOLINA Ann Thorac Surg REOPERATIONS FOR PAGET-SCHROETTER 2011;91: Fig 4. Patient no. 10. Typical findings at time of reoperation after the left first rib had been removed using the transaxillary approach. First rib remnant measured 1 3/4 inches (dotted line between arrows). Intact subclavius tendon inserting on the rib (isolated arrows). Patient s sternum is on the left side of the photograph. (Pec: pectoralis major muscle; Vein: subclavian.) only one in patient no. 2. Stents are usually firmly integrated into the scar tissue of the vein wall. All patients required a new stent, even if they had not received one previously, because the thick fibrous walls of the vein collapsed again even after implant of the enlarging vein patch and dilating the vein with large diameter balloons. This is explainable because the vein had already lost its normal structure; the walls had been replaced by heavy scar tissue which tends to close back if no supporting device is placed. So far the follow-up, which has been 100%, has shown normal flow and good patency rate of the vein up to over 5 years (Figs 2B; 3B). The range of follow-up extends now from 6 months to 5 years 4 months, showing good flow of patent subclavian veins with the patient completely asymptomatic and without anticoagulants. Comment Significant progress has been made in the treatment of the subclavian vein effort thrombosis at the time of the acute event and also in selected cases of chronic obstruction. However, one of the approaches implemented by many surgeons is the transaxillary resection of the first rib as introduced by Roos in 1966 [11]. As we have pointed out before, the Roos operation has its place in treating neurogenic or arterial thoracic outlet syndrome. However, it is not as effective in decompressing the subclavian vein. In the 1990s, when reports started to appear in how to treat Paget-Schroetter syndrome [18, 19], it was shown that in using the transaxillary route the incidence of veins remaining occluded was significantly high. Machleder, in 1993 [18], showed that 13 out of 35 patients undergoing this type of resection remained occluded. In the report by Azakie and colleagues [7], of 8 patients operated using this route 5 had recurring thrombosis at the same site [13]. Although the report by Urschel and Razzuk [8] of 34 patients operated using the transaxillary approach, where thy claimed excellent results in 32, none of those patients were subjected to venography postoperatively. Even in more recent publications by De Leon and colleagues [20], where they reported 67 patients treated at different times divided in 4 groups, all of them operated by the transaxillary route, their results showed the following. In the first group of 3 patients in acute stage, one acutely rethrombosed. In group 2 of 39 patients, venograms done 2 weeks postoperatively showed that 64% of them showed still significant stenosis that had to be treated with balloon plasty and anticoagulation. The incomplete decompression attained using the TARR was already observed and reported by Azakie and colleagues years ago [7]. A recent publication by Guzzo and colleagues [21] showed 49% persistent stenosis and 16% total occlusion of the subclavian vein after TARR, requiring balloon plasties and prolonged anticoagulation. Because of these less than optimal results of using transaxillary operations to decompress the subclavian vein, many surgeons began using what they call the paraclavicular approach [4, 21], which involves two incisions; one is infraclavicular and one supraclavicular, which gives a more direct approach to the anterior portion of the thoracic outlet where the vein is located. The report by Melby and colleagues [4] using this approach in 18 patients, as well as the publication by Schneider and colleagues [22], showed intraoperative patency of 92% even though 8% had recurring thrombosis and one of them suffered phrenic nerve injury. Nevertheless, in the Melby group intraoperative venography showed persistent stenosis in 16 patients (64%). It is interesting to see that the initial 23 patients were done using the two incisions of the paraclavicular approach, but in the last 2 patients they used only the infraclavicular which is our preferred technique. The report by Thompson and colleagues [23] also proposes the use of the paraclavicular approach which offers better exposure of the site of obstruction. There is a limitation in applying this technique when stents are already present as far as the innominate vein because placement of a clamp on the normal portion of the vein is beyond reach. With the significant incidence of residual stenosis of the subclavian vein after transaxillary removal of the first rib, patients are frequently subjected to percutaneous balloon angioplasties and placed on anticoagulation for prolonged periods of time with the hope of keeping the vein open. However, often enough balloon angioplasty alone does not result in a durable solution mainly because the extrinsic compression still persists, aggravated by the fact that the vein gradually re-fibroses again and reoccludes sometimes within a few months, sometimes up to a year. Stretching the lumen of this fibrotic stricture only leads to more fibrosis and eventual reocclusion of the vein. In order to overcome this problem the patients are treated with endovascular stent implants. When the stents kink and the vein reoccludes again, more stents are implanted in the same area as occurred in patients no. 1 and no. 9 seen in this series. All surgeons are aware that

5 Ann Thorac Surg MOLINA 2011;91: REOPERATIONS FOR PAGET-SCHROETTER ideally in the transaxillary approach the rib should be divided anteriorly at the sternal level accompanied by the resection of the subclavius tendon and the costoclavicular ligament [24]. But it appears that these goals are not always attained, particularly in large or muscular individuals because the pectoralis major muscle that needs to be retracted anteriorly to reach that level can almost never be accomplished. In our present report every case we reoperated had the costoclavicular ligament, the subclavius tendon, as well as the anterior portion of the first rib intact, and this is the site of the obstruction. The report by Urschel and Patel [24] in 2008 stated clearly that Paget-Schroetter syndrome is a surgical disease and should be treated accordingly prompt and properly. The transaxillary approach, however, does not allow access for direct repair of the vein which is frequently needed in these cases. Three factors are important in treating the subclavian vein effort thrombosis. (1) The time of surgery after the thrombolysis is completed should be done promptly lest the patient may become inoperable later. (2) The type of operation offered to the patient should entail complete anterior decompression of the vein tunnel. (3) The original caliber of the vein should be reestablished. The interventional radiologist generally does an outstanding job clearing the vein of thrombus with either thrombolytic agents or using the thrombectomy catheter. However, the surgical treatment that follows needs to be conducted promptly and appropriately. The anterior decompression seems to be the route of choice. When a transaxillary operation is undertaken a venogram, ultrasound, or both should be done postoperatively to verify patency of the subclavian vein. If the patient shows reocclusion or persistent obstruction of the subclavian vein, the patient should be reoperated by an anterior approach instead of repeated balloon dilations, stent placements, or indefinite periods of anticoagulation. References 1. Rutherford RB, Hurlbert SN. Primary subclavian-axillary vein thrombosis: consensus and commentary. Cardiovasc Surg 1996;4: Rutherford RB. Primary subclavian-axillary vein thrombosis: The relative roles of thrombolysis, percutaneous angioplasty, stents, and surgery. Semin Vasc Surg 1998;11: Molina JE, Hunter DW, Dietz CA. Paget-Schroetter syndrome treated with thrombolytics and immediate surgery. J Vasc Surg 2007;45: Melby SJ, Vedantham S, Narra VR, et al. Comprehensive surgical management of the competitive athlete with effort 1721 thrombosis of the subclavian vein (Paget-Schroetter syndrome) J Vasc Surg 2008;47: Urschel HC Jr, Razzuk MA. Improved management of the Paget-Schroetter syndrome secondary to thoracic outlet compression. Ann Thorac Surg 1991;52: Molina JE. Need for emergency treatment of subclavian vein effort thrombosis. J Am Coll Surg 1995;181: Azakie A, McElhinney DB, Thompson RW, Raven RB, Messina LM, Stoney RJ. Surgical management of subclavian vein effort thrombosis as a result of thoracic outlet compression. J Vasc Surg 1998;28: Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000;69: Molina JE. Surgery for effort thrombosis of the subclavian vein. J Thorac Cardiovasc Surg 1992;103: Molina JE, Hunter DW, Dietz CA. Protocols for Patet- Schroetter syndrome and late treatment of chronic subclavian vein obstructions. Ann Thorac Surg 2009;87: Roos DB. Transaxillary approach for first rib resection to relieve thoracic outlet syndrome. Ann Surg 1966;163: Molina JE. The vascular component in neurogenicarterial thoracic outlet syndrome. Intl J Angiol 2008;17: Urschel HC Jr, Patel AN. Paget-Schroetter syndrome therapy: failure of intravenous stents. Ann Thorac Surg 2003;75: Molina JE. Operative technique of first rib resection via subclavicular approach. J Vasc Surg 1993;27: Molina JE. Treatment of chronic obstruction of the axillary, subclavian and innominate veins. Intl J Angiology 1999;8: Molina JE. A new surgical approach to the innominate and subclavian vein. J Vasc Surg 1998;27: Molina JE. Approach to the confluence of the subclavian and internal jugular veins without claviculectomy. Semin Vasc Surg 2000;13: Machleder HI. Evaluation of a new treatment strategy for Paget-Schroetter syndrome: spontaneous thrombosis of the axillary-subclavian vein. J Vasc Surg 1993;17: Kunkel JM, Machleder HI. Treatment of Paget-Schroetter syndrome. Arch Surg 1989;124: de León RA, Chang DC, Hassoun HT, et al. Multiple treatment algorithms for successful outcomes in venous thoracic outlet syndrome. Surgery 2009;145: Guzzo JL, Chang K, Demos J, Black JH, Freischlag JA. Preoperative thrombolysis and venoplasty affords no benefit in patency following first rib resection and scalenectomy for subacute and chronic subclavian vein thrombosis. J Vasc Surg 2010;52: Schneider DB, Dimuzio PJ, Martin ND, et al. Combination treatment of venous thoracic outlet syndrome: Open surgical decompression and intraoperative angioplasty. J Vasc Surg 2004;40: Thompson RW, Schneider PA, Nelken NA, Skioldebrand CG, Stoney RJ. Circumferential venolysis and paraclavicular thoracic outlet decompression for effort thrombosis of the subclavian vein. J Vasc Surg 1992;16: Urschel HC Jr, Patel AN. Surgery remains the most effective treatment for Paget-Schroetter Syndrome: 50 years experience. Ann Thorac Surg 2008;86: INVITED COMMENTARY Management of thrombosis of the subclavian vein has remained challenging over the years, with a variety of approaches being promoted. Articles from the 1960s [1] reported transaxillary resection of the first rib; articles from the 1980s [2] extolled the virtues of resection of the clavicle; and articles in the 1990s [3] reported the introduction of thrombolytic therapy followed by first rib resection. Although the idea of resecting the clavicle never really caught on with most surgeons treating patients with this entity and although virtually all agree that lytic therapy is the correct first step in treating these thrombosed veins, controversy remains about what the best 2011 by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur