Management of Effort Thrombosis of the Subclavian Vein: Today's Treatment
Article Outline
“Spontaneous” subclavian vein (“effort”) thrombosis is usually related to extrinsic compression of the vein at the costoclavicular junction. Our experience with this entity over the past decade was reviewed with specific focus on the role of thrombolysis and our selection algorithm. All patients treated for this problem at the University of Rochester over the past decade were identified and records retrospectively reviewed. From 1996 to June 2006, a total of 34 patients with spontaneous complete occlusion of the subclavian vein documented by venography were treated, half with a history of an antecedent exertional factor. Catheter-directed thrombolysis prior to planned immediate thoracic outlet decompression (TOD) was performed in 26 patients, while TOD alone was performed in eight. Time since onset of symptoms was the major factor influencing the decision, being a mean of 5.5 days in the 26 referred for lysis but 1 month to many years in the group who underwent surgery alone. In patients undergoing lysis, flow was restored in 16 (62%), of whom nine had a residual lesion. All but two of the 26 who received thrombolysis then underwent TOD with or without angioplasty, and 13 underwent venous reconstruction as well (eight of the 16 in whom patency had been restored and five of eight in whom it had not). Patients not undergoing lysis were managed by TOD, with five (62%) undergoing decompression alone and three (38%) undergoing formal venous reconstruction. Thrombolysis was not attempted (eight) or unsuccessful (four) in all 12 patients whose symptoms had been present for more than 14 days at presentation. At mean follow-up of 33 months, symptom resolution was almost universal. Primary patency at 5 years was 84% in the thrombolysis group and 83% in the TOD-only group. In conclusion, the primary factor influencing treatment choice at our institution has been time since onset of symptoms. Patients presenting soon after symptom onset underwent thrombolysis followed by TOD, while patients presenting with chronic symptoms underwent TOD alone; in both cases venous reconstruction was based on residual findings after lysis. We have not had successful thrombolysis in a patient presenting with 14 days or more of symptoms. While optimal therapy cannot be defined based on this retrospective review, our algorithm resulted in excellent overall long-term patency and symptom relief.
Introduction
Primary subclavian vein thrombosis, also called “effort thrombosis” or Paget-von Schrötter syndrome, refers to thrombosis of the subclavian vein at the junction of the first rib and clavicle in the absence of an inciting factor such as an indwelling catheter. Although sometimes referred to as “idiopathic” or “spontaneous,” it seems to be caused by extrinsic compression by these two bony structures in essentially all cases. Indeed, the term “effort thrombosis” arose from the fact that this is often associated with either prolonged or repetitive upper extremity strain in young, healthy, often muscular and athletic patients.
The management of effort thrombosis has evolved over the last two decades as the utility of thrombolysis was identified. At present, most clinicians and investigators agree that early thrombolysis is the best approach and that subsequently most patients will require thoracic outlet decompression (TOD).1, 2, 3 Issues that remain unsettled include whether decompression should immediately follow lysis or be staged, the role of stents once the thoracic outlet has been decompressed, the indications for direct venous reconstruction versus observation after decompression alone, and the optimal treatment strategy for patients who present in a delayed fashion. We reviewed our experience with patients who presented with complete venous occlusion over the past decade to assess the structure and results of the treatment algorithm that has evolved at our institution, with particular attention to factors influencing the role of thrombolysis and venous reconstruction decision making.
Methods
All patients treated for venous thoracic outlet syndrome (TOS) or upper extremity deep vein thrombosis (DVT) at the University of Rochester Medical Center over the past decade were identified and records retrospectively reviewed. Candidates were identified based on the appropriate combination of ICD-9 and CPT codes and multiple patient lists. Inclusion required that complete absence of flow through the subclavian vein be positively confirmed by ultrasound or venography (all our patients had both) at initial presentation; patients with venous TOS manifest as positional stenosis and/or symptoms only without complete thrombosis were excluded. Intervention varies according to symptom status and anatomy and is further described below. Follow-up visits occur at 1, 3, and 6 months after hospital discharge and then yearly if all is well. Each visit includes ultrasound, and patency is defined by an open vein as seen on duplex examination. Anticoagulation is continued for 6 months, then halted unless (1) a residual defect is seen by ultrasound or (2) a hypercoagulable state is documented. A second episode of thrombosis anywhere in the body is treated with lifelong anticoagulation. Data are presented as means ± standard errors or percentages, as appropriate.
Results
A total of 123 patients were provisionally identified as having potential effort thrombosis with complete venous occlusion treated at the University of Rochester from 1996 to June 2006. Upon review, 50 clearly had neurogenic TOS, 24 incomplete occlusion, four arterial problems only, and 11 occlusion in the presence of an indwelling catheter. Thirty-four patients thus presented with complete venous occlusion at the costoclavicular junction without other causative factors.
Mean age was 33 years (range 18-69); 86% presented with arm swelling and 69% with pain, and half gave a history of an inciting factor such as significant vigorous exercise, employment with arms overhead, or suggestive avocational pursuits such as volleyball, kayaking, and/or guitar/banjo playing.
Thrombolysis prior to planned decompressive surgery was performed in 26 patients, while surgery alone was performed in eight. Time since onset of symptoms was the major factor influencing this decision, being a mean of 5.5 days in the 26 referred for lysis and months to many years in the eight who underwent surgery alone. Twelve patients presented with symptoms beginning more than 14 days prior to treatment, four of whom underwent attempted thrombolysis and eight of whom proceeded directly to TOD.
Decisionmaking and results of treatment are described in Table I, Table II, Table III. Of the 26 patients who were treated with thrombolysis prior to planned surgery, flow was restored by thrombolysis in 16 (62%), all of whom had symptoms that had begun 14 days or less from initiation of lysis. When flow was restored, nine of the 16 (56%) had a residual lesion at rest and all had a demonstrable stenosis or occlusion with arm abduction. All patients with successful lysis then underwent TOD by means of transaxillary first rib excision (n = 9) or claviculectomy (n = 7). Eight of the 16 patients underwent decompression only, while the other eight underwent decompression and venous reconstruction by means of intraluminal debridement and patch venoplasty (n = 5) or jugular venous turndown (n = 3).
Table I. Post hoc treatment groups (n = 34)
| Anticoagulation only | TOD only | TOD + venous reconstruction | % undergoing venous reconstruction | |
|---|---|---|---|---|
| Successfully lysed (n = 16) | 0 | 8 | 8 | 50 |
| Unsuccessfully lysed (n = 10) | 2 | 3 | 5 | 50 |
| Lysis not attempted (n = 8) | 0 | 5 | 3 | 38 |
Table II. Postlysis treatment
| Decompression | Reconstruction | |||
|---|---|---|---|---|
| TA 1st rib | Clavicle | Local/patch | JVTx | |
| Patients undergoing thrombolysis prior to planned TOD (n = 26) | ||||
| Successful (n = 16) (all with stenosis seen with arm abduction) | ||||
 Residual lesion (n = 9) | 5 | 4 | 3a | 2 |
| No residual (n = 7) | 4 | 3 | 2 | 1 |
| Unsuccessful (n = 10) (2 patients anticoagulated only) | 3 | 6b | 1 | 4 |
aOne patient had a patch angioplasty after a transaxillary first rib resection. |
bOne patient underwent medial claviculectomy in addition to first rib resection. |
Table III. Results in the eight patients in whom thrombolysis was not attempted
| Decompression | Reconstruction | ||||
|---|---|---|---|---|---|
| TA 1st rib | Clavicle | Sternal disarticulation + first rib | Local/patch | JVTx | |
| Decompression only (n = 5) | 5 | 0 | 0 | n/a | n/a |
| Decompression and reconstruction (n = 3) | 0 | 2 | 1 | 3 | 0 |
Thrombolysis was attempted but unsuccessful in 10 of the 26 patients (38%). Eight of these patients underwent TOD, three by means of transaxillary first rib resection, four by medial claviculectomy, and one by both. Five of the 10 (50%) underwent venous reconstruction, all after claviculectomy. Two patients declined surgery and were anticoagulated. Mean duration of lysis was 31 ± 14 hr and did not differ between the successful and unsuccessful groups.
Eight patients, all of whom had symptoms for 1 month or more, were not felt to have a potential benefit from thrombolysis. These patients were managed by TOD with transaxillary first rib resection alone (n = 5) or by medial claviculectomy (n = 2) or disarticulation (n = 1) followed by patch venoplasty (Table III). All patients undergoing eventually successful thrombolysis had symptoms of less than 14 days' duration (Table IV).
Table IV. Symptom timing at presentation versus outcome
| Interval from symptom onset to presentation | ||
|---|---|---|
| Intervention | Mean ± SD | Range |
| Successful lysis with planned surgery to follow (n = 16) | 5.5 ± 5.6 days | 1-14 days |
| Failed lysis with planned surgery (n = 10) | Unreportable | 7 days–“months to years” |
| No lysis attempted (n = 8) | Unreportable | “Months to years” |
Eleven complications occurred in nine of the 26 (35%) patients undergoing lysis followed by surgery and in five of the eight (63%) patients undergoing surgery alone (Table V).
Table V. Operative and postoperative complications by intervention
| Lysis (11 complications in 9 patients, 2 reoperations) | TOD alone (5 complications in 8 patients, 1 reoperation) | |||||
|---|---|---|---|---|---|---|
| Lysis alone (n = 2) | TA 1st rib (n = 12) | Clavicle (n = 13) | TA 1st rib (n = 5) | Clavicle (n = 2) | Sternal disarticulation (n = 1) | |
| Hematoma (n = 8) | 0 | 3 | 3 | 0 | 1 | 1 |
| Chylous leak (n = 1) | 0 | 0 | 1 | 0 | 0 | 0 |
| Pneumothorax (n = 3) | 0 | 1 | 0 | 2 | 0 | 0 |
| Suspected pulmonary emboli (n = 2) | 0 | 1 | 0 | 1 | 0 | 0 |
| Stroke-like episode (n = 1) | 1 | 0 | 0 | 0 | 0 | 0 |
| Reoperation (n = 3) | 0 | 1 | 1 | 0 | 0 | 1 |
No differences were seen in initial length of hospital stay (6.8 vs. 6.6 days), number of future admissions (1.2 vs. 1.6), and total time spent in the hospital (7.3 vs. 7.5 days) between those undergoing thrombolysis and those proceeding directly to TOS, respectively.
At mean follow-up of 33 months, symptom resolution was almost universal. Primary and secondary patency rates at 5 years were 84% and 92% in those undergoing planned thrombolysis prior to TOD and 83% and 83% in those referred directly to TOD, respectively. There were no differences in patency rates between the group who initially underwent thrombolysis compared to the group who did not.
Discussion
Our analysis demonstrates that over the past decade we have used time since onset of symptoms to determine whether patients should undergo preliminary thrombolysis or not but that in almost all cases TOD was performed whether or not the vein had been recanalized. Venous reconstruction was performed in about half of our cases, with this decision and specific method being determined by the status of the vein and symptoms at the time of surgery. Notably, in our hands, thrombolysis has never been successful if symptoms have been present for more than 14 days prior to its onset. Using this algorithm, we have obtained essentially universal symptom relief and secondary patency rates of approximately 85% at 5 years.
Sir James Paget first described spontaneous thrombosis of the subclavian vein in 1875.4 In 1884, von Schrötter postulated that this entity resulted from direct damage to the vein caused by stretching that occurred from muscular strain,5 and the English surgeon Hughes then coined the term “Paget-von Schrötter syndrome” in 1949.6 Synonyms for this condition include “primary,” “idiopathic,” “spontaneous,” “traumatic,” and “effort” thrombosis, the last because of its frequent occurrence following physical exertion.
Effort thrombosis is an uncommon but not rare entity, with an annual incidence estimated as about two per 100,000 people.7 It was classically described to be most common in young patients, particularly males, usually after vigorous exercise or activity of the upper extremity, and seemed to be more common on the right.8 The condition is believed to develop primarily as the result of trauma to the subclavian vein from compression by a scissoring action on the vessel between the first rib and the clavicle at the level of the costoclavicular ligament. As first reported in 1939, patients typically present with sudden onset of an aching discomfort with a feeling of heaviness, swelling, and frequently a reddish blue discoloration of the affected upper extremity.9 A prominent venous pattern consisting of dilated superficial collateral veins over the upper arm, base of the neck, and anterior chest wall develops in a majority of patients.
The underlying problem in this patient population is thought to be an anatomically smaller costoclavicular space, which is the result of either hypertrophied muscle (scalenus anterior, subclavius, or pectoralis minor) or abnormal bone morphology (such as that following a fractured clavicle) alone or in one of numerous combinations. The specific precipitating event in this patient population is usually not remembered, but about 40% of patients recall an activity that involves repetitive or prolonged hyperabduction or external rotation of the shoulder joint.1, 10 The process seems to be more complex than simple compression alone. Repetitive trauma is thought to elicit a local inflammatory response, causing eventual formation of intimal hyperplasia, granulation tissue, and intravascular scarring or webs.11 The vein also becomes externally adherent to the surrounding anatomic structures immediately anterior and posterior to the vein, and this less mobile situation then increases the risk for further mechanical trauma, especially with shoulder abduction and arm elevation. The problem seems to be primarily mechanical: hypercoagulable states are relatively rarely found in these patients.7
Even if the vein is not completely occluded, symptoms may arise from recurrent partial occlusion caused by arm positioning, partial thrombosis, or even unnoticed prior occlusion followed by recanalization. Each time the vein is injured or experiences any degree of thrombosis, the sequence of local inflammation, granulation tissue formation, and scar formation progressively adds to the intra- and extraluminal pathology. When the vein is chronically occluded, symptoms are typically milder because of collateral formation.12
Assuming the condition is considered, diagnosis using duplex ultrasonography should be straightforward if the vein is completely occluded. Venography will demonstrate complete obstruction of the vein with prominent collateral veins around the site of occlusion, a pattern referred to by Adams et al.13 as “first-rib bypass collaterals.” This pathway consists of communications between the cephalic vein, the transverse cervical vein, and the transverse scapular vein, tributaries of the terminal subclavian vein as it passes over the first rib. Adams et al. believed that in cases where the first rib collaterals are demonstrated thrombosis very likely began at the first rib level, and this is still the accepted view.
It was demonstrated as early as 1949 that leaving the condition untreated results in permanent disability,6 leading to symptomatic residual upper extremity venous obstruction in up to 75% of affected patients.1, 14 The consensus opinion today is that acute subclavian vein thrombosis should be treated with initial catheter-directed thrombolysis followed by decompression of the venous thoracic outlet (costoclavicular junction).1, 2, 3 The goals of thrombolysis are to reduce clinical morbidity by improving venous outflow from the limb and to halt the vicious cycle of local inflammation and trauma caused by the thrombosis.11, 15 While this will treat the immediate problem, the underlying anatomic defect remains and must be treated if the condition is not to quickly recur. Therefore, once the vein has been cleared (and even if it cannot), “elimination” of the narrowed costoclavicular space by means of removal of the anterior portion of the first rib or clavicle should follow.16, 17, 18, 19 The timing of TOD following thrombolysis remains somewhat controversial. Those who advocate “a trial of anticoagulation” suggest that allowing postthrombotic inflammation to resolve improves outcome,3 but those who advocate immediate decompression point to rates of early rethrombosis as high as 50% during this period.1, 20
A significant number of patients are shown to have intrinsic venous defects on postdecompression venography.15, 21 Whether or not all of these require direct therapy is unknown. One school of thought suggests that elimination of the acute intraluminal process combined with adequate TOD will allow resolution of even gross residual defects with anticoagulation alone,22 while others advocate a more aggressive approach to venous reconstruction, by means of direct repair or jugular venous turndown.11 In general, we and others believe that if venous reconstruction is not to be performed, TOD is best accomplished by transaxillary first rib resection, while claviculectomy offers the best exposure if any direct repair of the vein is contemplated.11, 23, 24 An anterior supraclavicular or subclavicular approach is used by some, but venous reconstruction is very difficult. The clavicle can be disarticulated or rotated upward with a piece of the sternum for better exposure,25 but in our experience morbidity is higher than with simple medial claviculectomy.
The costoclavicular junction is unyielding, and angioplasty prior to decompression has been shown to fail at high rates. Interestingly, it may even directly worsen venous patency,26, 27 perhaps by further damaging the endothelium. Stenting of the vein running through the nondecompressed costoclavicular junction has been shown to be complicated by stent fracture in some, deformation in nearly all, and rethrombosis rates as high as 40%28 and, thus, should be condemned, although the behavior of a venous stent after adequate TOD is as yet unknown.
As a result of the limited number of patients and the nature of this retrospective review of an uncontrolled experience, we cannot make firm recommendations based on these data. Our patients have done well, however; and we would recommend the following algorithm (Fig. 1).
Fig. 1
Suggested algorithm for patient with complete subclavian vein occlusion at the costoclavicular junction.
Patients presenting with symptoms of less than 14 days' duration should undergo thrombolysis regardless of symptom severity. If flow is restored, TOD should be performed acutely (within a day or so), with the specific approach guided by the residual lumen and symptoms. If no residual intraluminal defect exists, transaxillary first rib excision followed by anticoagulation is optimal. If a lesion persists but symptoms are mild (i.e., acute hemodynamic success has been achieved), transaxillary first rib excision will likely allow gradual resolution; but if significant symptoms persist, strong consideration should be given to venous reconstruction. We have been very pleased with medial claviculectomy and jugular venous turndown and believe that this aggressive approach is most likely to result in long-term symptom relief. If patency cannot be restored and more than mild symptoms are present, we advocate reconstruction by means of jugular turndown.
It is our belief that early intervention (a day or two, at most) after attempted thrombolysis offers the best chance at long-term patency. This is due to our belief that the extrinsic compression leads to a persistent intrinsic defect and a perpetual source of inflammation until removed, even after lysis. This approach leads to a higher complication rate at the time of surgery, but such complications cause no mortality and do not seem to affect patency.
If symptoms have been present for more than 14 days, the utility of thrombolysis is less certain. We have not been successful in this situation, but our numbers are very small. We do feel imaging is mandatory prior to reconstruction and, even if chronic, an attempt can be made to cross the lesion with a wire. If it cannot be crossed, claviculectomy and reconstruction should be performed; if it can, an attempt to mechanically debulk the thrombus and/or chemically thrombolyse it can be considered. We would not, however, advocate excessively aggressive attempts to repair this intraluminally as significant complications can and have occurred in this setting. Again, TOD should be performed in all cases.
Finally, if a patient is seen with suggestive symptoms but normal venous flow by ultrasound, venography with the arm abducted should obviously be performed and decompression (transaxillary first rib) performed if a lesion is seen. Additionally, we studied the contralateral side of several of our patients and found venous compression in a significant number. This has warranted us to regularly study the contralateral side of affected patients. When asymptomatic compression is discovered, a discussion occurs, and usually the patient opts for a prophylactic transaxillary first rib resection.
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Presented at the Seventeenth Annual Winter Meeting of the Peripheral Vascular Surgery Society, Steamboat Springs, CO, January 26-28, 2007.
PII: S0890-5096(07)00289-0
doi:10.1016/j.avsg.2007.07.017
© 2007 Annals of Vascular Surgery Inc. Published by Elsevier Inc All rights reserved.
