Venous Thoracic Outlet Syndrome: The Role of Early Rib Resection

Transcription

1 MILITARY MEDICINE, 181, 11/12:e1706, 2016 Venous Thoracic Outlet Syndrome: The Role of Early Rib Resection LT Vienna G. Katana, MC USN*; CAPT Jeffrey S. Weiss, MC USN ABSTRACT The upper extremity is an uncommon site for deep vein thrombosis and, although most of these thrombotic events are secondary to catheters or indwelling devices, venous thoracic outlet syndrome is an important cause of primary thrombosis. Young, active, otherwise healthy individuals that engage in repetitive upper extremity exercises, such as those required by a military vocation, may be at an increased risk. We present the case of a Naval Officer diagnosed with venous thoracic outlet syndrome whereby a multimodal approach with early surgical decompression was used. Although thoracic outlet decompression by means of first rib resection is the standard of care, timing of first rib resection after thrombolysis is debated. With respect to the active duty service member, the optimal timing of additional postoperative interventions for residual venous defects and duration of anticoagulation remain in question. A more streamlined perioperative treatment regimen may benefit the military patient without jeopardizing the quality of care and allow more expeditious return to full duty. CASE PRESENTATION A 26-year-old Surface Warfare Officer presented to his ship s medical department with 5 days of right shoulder pain and arm weakness following strenuous upper body exercise in preparation for his physical readiness test. He endorsed concurrent paresthesiae within the T1/C8 dermatomal distribution. He had previously been evaluated by multiple providers for similar symptoms and diagnosed with bicep tendonitis 2 weeks before this presentation; of most concern to him was the new onset of nocturnal pain and inability to do push-ups. He served as his ship s weapons officer, but he denied consistent use of firearms preceding this event or any other repetitive occupational activities. He had no personal or family history of thrombophilia. His review of systems was negative for chest pain or shortness of breath. Physical examination was notable for right upper extremity edema, cyanosis, and engorged venous collaterals across his shoulder, axilla, and chest. The neurovascular examination demonstrated equally palpable upper extremity arterial pulses bilaterally with normal motor function and sensation. His skin was devoid of ulcers or wounds. He was immediately referred to the local military medical treatment facility where a duplex venous ultrasound demonstrated right subclavian and axillary vein thrombosis. He was heparinized, admitted to the vascular surgery service, and taken for venography (Fig. 1) which confirmed the ultrasound findings. Using a right basilic vein approach, catheterdirected thrombolysis with tissue plasminogen activator was initiated. After approximately 24 hours, his venogram showed resolution of thrombosis but the presence of a *USS Somerset (LPD-25), Medical Department, Naval Base San Diego, FPO AP , San Diego, CA, Division of Vascular Surgery, Department of Surgery, Naval Medical Center San Diego, Bob Wilson Drive, San Diego, CA The views expressed herein are those of the author and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the U.S. Government. doi: /MILMED-D severe subclavian vein stenosis at the level of the first rib. Percutaneous transluminal angioplasty (PTA) was attempted within the proximal subclavian vein with little effect (Fig. 2). He was discharged on therapeutic subcutaneous enoxaparin as a bridge to warfarin with a plan for an elective first rib resection (FRR) with scalenectomy in 3 weeks. Following successful thoracic outlet decompression with FRR via a transaxillary approach, intraoperative venography with PTA (Fig. 3) was performed for a residual subclavian vein stenosis with good results. He was discharged by postoperative day 2 with the plan to complete a 3-month course of anticoagulation therapy. Just before his final follow-up (2 months post-frr with intraoperative PTA), the patient complained of mild, intermittent arm swelling associated with pain that coincided with an increase in upper extremity activity. Venography was devoid of thrombus, but showed restenosis unresponsive to serial PTA; therefore, a 14-mm self-expanding bare metal stent was deployed across the stenotic region with restoration of flow (Fig. 4). He was continued on oral anticoagulation for an additional 2.5 months (5.5 months total). At his 6-month follow-up, right upper extremity duplex ultrasound was negative for thrombus or wall thickening in the venous system, therefore warfarin was discontinued and he was transitioned to oral antiplatelet therapy (aspirin 81 mg daily) for 3 months. DISCUSSION Upper extremity deep vein thrombosis is an uncommon entity; although most of the thrombotic events occurring at this site are secondary to catheters, indwelling devices, and cancer, venous thoracic outlet syndrome (VTOS) is an important cause of primary thrombosis. 1 VTOS is caused by extrinsic compression of the subclavian vein between the anterior scalene muscle and the junction of the first rib, clavicle, and subclavius muscle. 2 Persistent compression injury (i.e., strenuous exercise or repetitive movement) causes intrinsic endothelial damage e1706

2 FIGURE 1. Demonstrating complete occlusion of right subclavian and axillary veins (arrows). Wire transverses occluded segment. First rib (dashed lines). leading to thrombus formation. 3 This effort thrombosis, also known as Paget-Schroetter Syndrome, classically occurs in the dominant arm of young, active individuals. 3 VTOS has a yearly estimated incidence of 2 per 100,000 and accounts for 4% of all thoracic outlet syndromes. 1 Not only competitive athletes but also active duty military members could be at an increased risk given occupational habits and vocational demands; the diagnosis can be derailing to an operational tour and it adversely impacts medical and command readiness for the Fleet. Delayed presentation is common as distal tributaries form in response to venous congestion from luminal compression and initial thrombus; acute symptoms are hypothesized to FIGURE 3. Intraoperative venogram following first rib resection (circle denotes area of rib resection) and percutaneous transluminal angioplasty for residual stenosis. Although stenosis is not clearly visualized in this unsubtracted image, the presence of venous collaterals (arrows) may infer a residual stenosis. occur after propagation of the clot obstructs these distal collaterals. 3 Given the natural history of this disease, most patients with VTOS have elastic fibrous scarring within the vessel lumen. 4 Our patient likely experienced symptoms of chronic occlusion which are vague and can be easily mistaken for common musculoskeletal/athletic injuries that plague active duty members. This condition is often misdiagnosed or underdiagnosed; prompt recognition and treatment within 14 days of the acute thrombus is one FIGURE 2. Venogram approximately 24 hours after successful thrombolysis. Significant subclavian vein stenosis (arrows) at the level of the first rib (dashed lines) persists after initial attempts at percutaneous transluminal angioplasty. FIGURE 4. Full venous patency across deployed stent (arrows). e1707

3 of the most important predictors of outcome. 3 The risks of organized clot propagating to pulmonary emboli and postthrombotic syndrome increase with a delay in diagnosis. 1 Treatment algorithms are highly varied given the relative rarity of this disease and lack of quality-randomized controlled studies. Most protocols are guided by single institutional reports, retrospective reviews, and expert opinion. 5 Hence, various operative and nonoperative strategies exist. One of the most commonly used operative algorithms was developed by Kunkel and Machleder in the 1980s; this protocol included early thrombolysis and 3 months of anticoagulation before transaxillary FRR. 2,6 Even though surgical decompression after thrombolysis has now become the standard of care, some experts still argue that FRR may be overutilized as a broad treatment strategy. 7 Conservative, selective surgical algorithms have been described. For example, Lee et al from Stanford University performed rib resection only on those patients that had recurrent or persistent symptoms, had ultrasound evidence of wall thickening, or had rethrombosis after the initial clot lysis. 8 Of note, 48% (10/21) of the competitive athletes in this small series had recurrent symptoms/rethrombosis following 1 month of oral anticoagulation. Moreover, 27% (3/ 11) of the remaining athletes that were symptom free at the 1-month follow-up and therefore selected for the nonoperative group had rethrombosis during a mean follow-up of 10 months (range 6 14 months). 8 Although this study concluded that competitive athleticism did not mandate surgical intervention, nor was this a characteristic found to be associated with long-term thrombotic recurrence, 62% of the athletes in this study ultimately required FRR. Furthermore, this need for FRR increased with younger age, a defining characteristic of the active duty population. While claiming that high rates of total complications (18% in their series and up to 20% in other series) was a critical point for a selective surgical approach, 8 recent literature claims that immediate or early FRR decreases the duration of symptoms without increasing the complication rates 2 ; serious complications associated with this surgery, such as hemothorax and nerve injury, are reported to be rare (1 2%). 5 To contrast their initial conservative approach, a recent study from this same group focused on the ability of competitive athletes to recover from VTOS following an early and standardized surgical intervention. All athletes in this VTOS cohort underwent FRR with 93% expeditious return to sport at a mean time of 4.7 months (range 2 12 months). 9 They therefore conclude that young athletes with VTOS must have surgical decompression to reduce the risk of prolonged symptomatology, rethrombosis, and athletic disability. 9 Not only are there disparities between surgical and nonsurgical algorithms as discussed above, but there is also a lack of consensus on the appropriate timing of FRR, PTA, and stenting. 1 In one retrospective review from the Naval Medical Center San Diego, Colleta et al described an early synergistic approach whereby most FRR procedures were performed during the initial hospital admission (usually within 5 days of thrombolysis). Repeat venography was obtained at approximately 10 days postoperatively; if residual stenosis was present and not responsive to PTA, stenting was performed. Standard postintervention oral anticoagulation duration was for 3 months. 10 Schneider et al highlighted an expedient strategy where thrombolysis was immediately followed by decompression surgery. As residual intrinsic venous defects were a common finding (present in 64% of their subjects), extrinsic and intrinsic pathology was addressed concurrently by performing an intraoperative venogram and PTA as indicated. This one-stage operation was hypothesized to decrease the risk of rethrombosis and need for stent placement in the postoperative period. Patients were followed with serial ultrasounds (at discharge, 1, 6, and 12 months postoperatively) confirming a primary patency rate of 92% at 1 year. 11 The largest retrospective surgical series to date is from Johns Hopkins Medical Institutions; 261 patients with VTOS were treated with a standardized surgical protocol and achieved a long-term patency rate of 97%. 12 Their protocol uses anticoagulation with or without thrombolysis (reserved for acute thrombosis identified within 2 weeks of symptom onset) followed by FRR. Patients that presented to their institution with chronic subclavian vein thrombosis or stenosis proceeded directly to FRR 13,14 ; particularly in subacute or chronic thrombosis, they claim that preoperative endovascular intervention (thrombolysis and/or venoplasty) offered no benefit to anticoagulation alone with regard to patency following definitive treatment with FRR. 15 They also hypothesized that high rates of early restenosis after FRR may be the result of postoperative manipulation and subsequent inflammation within the subclavian vein 14 ; therefore, regardless of symptoms, all patients received a routine venogram 2 weeks post-frr rather than intraoperatively. The results of the venogram dictated additional management (anticoagulation plus PTA or anticoagulation alone). PTA was performed for residual stenosis with greater than 50% luminal narrowing with repeat attempts for greater than 30% stenosis. 14 Stents were explicitly not used at the beginning of the series 13 and there was no mention of use at the end of their 10-year retrospective review. Other literature suggest that PTA may be used before or after surgical decompression, whereas stent placement is typically reserved for residual stenosis, not responsive to PTA, only after the extrinsic compression has been removed. 1 Symptomatology and physical examination alone have not been shown to be a sufficient method for determining longterm subclavian vein patency 14,16 ; therefore, the John Hopkins standard protocol used early post-frr venogram in conjunction with routine ultrasonic imaging at 6-month intervals up to 1 year; recurrence after this time was shown to be rare. 12 This method claimed alignment with best practices and has been promulgated as the key factor for ensuring longterm patency in VTOS patients. 12 e1708

4 Reviewing literature for other military case reports, FRR was delayed 8 months after presentation, 17 there was no mention of time course to FRR, 18,19 or patients received conservative, nonsurgical management only We argue that definitive treatment with FRR at the time of admission or within weeks of thrombolysis is prudent in military patients with high vocational demands; rethrombosis rates have been estimated at 30 to 50% when delaying FRR to 6 weeks post thrombolysis. 5 Symptom-free rates are only 63% for those without FRR or delayed FRR versus 93 to 95% in early, routine FRR. 5 Presurgical intervention (beyond thrombolysis providing immediate symptom relief in the acute phase) may have little impact on the long-term patency and recovery as long as FRR is performed promptly. Therefore, we could have considered thrombolysis alone followed by FRR during the initial hospitalization to reduce his total course of treatment. Predecompression PTA may be a superfluous attempt as the extrinsic forces on the vein have not been removed. Moreover, it is unknown if intraoperative PTA reduces the risk of postsurgical restenosis/rethrombosis or contributes to a proinflammatory and pro-occlusive environment. More importantly, it may be difficult to ascertain the final disposition of the treated vein following surgery as this area can be challenging to duplex and the patient may not be active enough to induce symptoms of venous obstruction related to residual intrinsic defects. Reported symptomatology and physical examination alone have been shown to be an inaccurate means of determining venous patency. Therefore, accurate and early postoperative imaging is key; routine venography, as described by the Johns Hopkins series, has been justified in the absence of symptoms and has been proclaimed as best practice for long-term success. In hindsight, we may speculate that this routine venogram 2 weeks post-frr may have elucidated our patient s restenosis amenable to PTA and possibly averted future symptoms and/or stent placement. Although, we did perform a routine ultrasound at his 6-month follow-up to support discontinuation of oral anticoagulation, we should additionally consider a routine ultrasound at the 12-month mark. CONCLUSION Diagnosis and treatment of VTOS is a clinical challenge. Primary care providers in the Fleet must maintain a high index of suspicion when a patient presents with upper extremity complaints related to recent vigorous exercise. To avoid long-term disability in this unique patient population, recognition and prompt referral for specialized surgical intervention is paramount. Immediate or early decompression with FRR should be considered as this has shown to reduce the risk of pulmonary embolism, rethrombosis, and the debilitating sequelae of post-thrombotic syndrome. As early restenosis may be inherent from the decompression surgery, repeating venography early in the postoperative course for the asymptomatic patient may elucidate residual stenosis treatable by additional intervention without extending the total time of oral anticoagulation; therefore, total time of disability may be dramatically reduced allowing early and full return to premorbid vocation and/or operational duty. Routine ultrasound examination should be performed in the asymptomatic patient up to 12 months postsurgery. ACKNOWLEDGMENTS VG Katana and JS Weiss had full access to all the data in the study and take responsibility for the integrity of the data, and VG Katana and JS Weiss interpreted the data and take responsibility for the accuracy of the data analysis. Drafting of the manuscript and critical revision of the manuscript for important intellectual content was performed by VG Katana and JS Weiss. REFERENCES 1. 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