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2.1 Guidelines
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– Results of thrombolysis. – Any complications.
• Operative thoracic outlet decompression or venous intervention.
– Duration of symptoms in days and classication as before. – Whether the patient has received thrombolysis before surgery and results
thereof. If so, interval in days between the cessation of thrombolysis and
surgery. – Status of the subclavian vein at the time of operation. – Surgical approach and structures removed. – Presence or absence of occult anterior rst rib fracture or osteophytic
degeneration. – Use of external axillary-subclavian venolysis and status of vein thereafter. – Whether any adjunctive procedures were performed.
Intraoperative venography. Endovenous intervention. Surgical repair of the subclavian vein, including details of repair and mate­rial used. Other reconstruction (jugular turndown, arteriovenous stula creation).
– Pleural entry, use of chest drainage. – Intraoperative complications. – Postoperative pain control and methods used. – Length of hospital stay. – All postoperative complications or readmissions within 30days. – Postoperative anticoagulation or antiplatelet therapy, with duration.
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ATO S
Description of treatment should be categorized into the three goals of treatment for patients with ATOS:
1. repair/replacement of the damaged subclavian artery (local disease);
2. correction of the original inciting cause at the thoracic outlet (cause); and
3. correction of any distal embolic or other problems (distal disease).
As many details as possible should be documented:
• Catheter-directed thrombolysis.
– Any endovascular intervention done at this time should be recorded. – Any complications should be recorded. – Status of arm and hand at cessation of lysis should be recorded.
• Operative management.
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– Whether the patient has received thrombolysis before surgery. If so, interval
(in days) between cessation of thrombolysis and surgery. – Status of the artery at the time of operation (at scalene triangle and in arm). – Surgical approach and structures removed. – Whether any adjunctive procedures were performed.
Endoluminal intervention. Arterial repair, replacement, or bypass. [For either, conduit or material used] Distal embolectomy. Fasciotomy.
– Completion arteriography or duplex ultrasound. – Neurological status before and after surgery. – Pleural entry, use of chest drainage. – Intraoperative complications. – Postoperative pain control methods used. – Length of hospital stay. – All postoperative complications or readmissions within 30days.
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2.1.2 American College ofRadiology Appropriateness Criteria
The American College of Radiology has published criteria for appropriate imaging in NTOS, VTOS and ATOS [3].
• Radiography of the chest and either MRI without and with IV contrast of the chest or MRI without IV contrast of the chest are usually appropriate for the initial and follow-up imaging after surgery or intervention for patients with NTOS.
• Radiography of the chest and US duplex Doppler of the subclavian artery and vein, CT with IV contrast of the chest, or catheter venography of the upper extremity are usually appropriate for the initial and follow-up imaging after sur­gery or intervention for patients with VTOS.
• Radiography of the chest and CTA with IV contrast of the chest, MRA without and with IV contrast of the chest, US duplex Doppler of the subclavian artery and vein, or arteriography of the upper extremity are usually appropriate for the ini­tial and follow-up imaging after surgery or intervention for patients with ATOS.
2.2 Results
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2.2 Results
2.2.1 Meta-Analyses/Systematic Reviews
2.2.1.1 Surgical Procedures forNTOS
In a systematic review, Yin et al. [4] compared the treatment outcomes of three alternative surgical procedures for NTOS: transaxillary rst rib excision, 17 studies/ supraclavicular rst rib excision with scalenectomy, 9 studies/and supraclavicular release leaving the rst rib intact, 14 studies. In this analysis, supraclavicular relase had the highest point estimates of success rate and complete relief rate (85% and 61%, respectively), followed by supraclavicular rst rib excision (77% and 57%, respectively) and transaxillary rst rib excision (76% and 53%, respectively). The probabilities of complete relief rate of 50% or greater were 67%, 71%, and 69% for transaxillary rst rib excision, supraclavicular rst rib excision, and supraclavicular release, respectively. Sensitivity analyses showed similar results. The complication rates for transaxillary rst rib excision, supraclavicular rst rib excision, and supra­clavicular release were, respectively, 22.5%, 25.9%, and 12.6%. These ndings sug­gest that resection of the rst rib is not necessary for surgical treatment of patients with neurogenic TOS.
In a second meta-analysis, Blondin etal. [5] came to a similar conclusion: rib-
sparing scalenectomy (RSS) is an effective option for the treatment of NTOS, RSS is sufcient for treating NTOS without the added morbidity of rst rib resection.
2.2.1.2 Procedures forPaget-Schrötter Syndrome (VTOS)
For a meta-analysis on the treatment of Paget-Schrötter syndrome, Karaolanis etal. [6] found 25 studies with 1511 patients. Thrombolysis was performed in 1177 (77.9%) patients, anticoagulation in 658 (43.5%) and decompression of the thoracic outlet in 1293 (85.6%). The complete thrombus resolution was estimated at 78.1% after thrombolysis, and the respective pooled proportion for partial resolution of thrombus was 23.7%. Despite thrombolytic therapy, additional balloon angioplasty was performed in 212 patients for residual stenosis, although only 36 stents were implanted. After anticoagulation, complete thrombus resolution was seen in 40.7% and partial resolution in 29.1%. During follow-up, 51.75% of patients with any initial treatment modality had no remaining thrombus and 84.7% of these patients were free of symptoms. A subgroup meta-analysis with 20 studies and 1309 patients, showed signicantly improved vein patency and symptom resolution in patients who had rst rib resection with or without venoplasty, compared with those who had only thrombolysis. The data suggest that resection of the rst rib after throm­bolysis is the treatment of choice in the majority of patients with Paget-Schrötter syndrome.
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2.2.2 Studies
2.2.2.1 TOS: Incidence/Registry Data
Based on a prospectively maintained database of an academic TOS centre with 526 admitted patients in 47months, Illig etal. [7] reported the ratio of NTOS, VTOS and ATOS in the admission diagnoses as 82%, 16% and 2%, respectively. NTOS was also the TOS most commonly treated surgically (73%, 25%, 3%). They esti­mated the annual incidence of NTOS to be approximately 3 cases/100,000 popula­tion, and that of VTOS to be 1/100,000. Although TOS is considered a rare disease, the incidence according to this calculation was not lower than that of amyotrophic lateral sclerosis, for example.
George etal. [8] analysed 3547 TOS operations recorded in the National Inpatient
Sample for the years 2010 to 2015 (89.2% NTOS, 9.9% VTOS and 0.9% ATOS). They estimated that a total of 18,210 TOS surgeries were performed in the US dur­ing this time, with an upward trend. Higher volume centres (≥10 cases/year) repre­sented 5.2% of hospitals, but 37% of cases, and achieved signicantly lower overall major complication rates, but no difference in neurologic complications. Overall mortality was less than 0.6% and was highest after procedures for VTOS (4.2%), followed by 0.2% for NTOS and 0% for ATOS. Neurological complications occurred in 0.34% of cases, brachial plexus injuries in 0.11%, iatrogenic pneumo­thorax in 3.3% and vascular complications in 8.7% in VTOS, 9.7% in ATOS and only 2.1% in NTOS.Higher volume centers delivered higher value care: less or similar operative morbidity with lower total hospital charges.
In a retrospective analysis of the National Surgical Quality Improvement Program
database from 2005 to 2014, Jubbal etal. [9] found 1853 patients with TOS.The purpose of this study was to identify the most common techniques used to treat TOS and compare complication rates. The most common procedures were rst rib resec­tion (64%), followed by anterior scalenectomy with cervical rib resection (32.9%), brachial plexus decompression (27.2%) and anterior scalenectomy without cervical rib resection (8.9%). The authors reviewed complication rates and concluded that surgical treatment of TOS most commonly involves rst rib resection, which is not associated with an increased risk of medical or surgical complications. Factors that demonstrated association with medical complications included female gender, ASA classication of 3 or greater, and prolonged operative time. No factors demonstrated statistically signicant association with surgical complications.
2.2.2.2 VTOS: Outcome
The outcome in 36 patients with chronic VTOS was presented by Pesser etal. [10]. VTOS was considered chronic when symptoms and venous stenosis or occlusion were present for >3 months after the initial primary upper extremity deep vein thrombosis event. Treatment algorithm consisted of transaxillary rst rib resection
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for thoracic outlet decompression, external venolysis, and intraoperative venogra­phy, followed by percutaneous low-pressure diagnostic balloon ination, in a single setting. Chronic occlusions that could not be recanalized with a guidewire were excluded. At a mean follow-up of 24months, 83% of patients were free of symp­toms. The functional outcome measures and quality of life had all improved signi­cantly (P< .01). A return to daily activities was achieved by 93% of the patients. The treatment of patients with chronic venous thoracic outlet syndrome using a same admission treatment algorithm consisting of transaxillary thoracic outlet decompression, external venolysis, and percutaneous transluminal angioplasty is effective and leads to signicant improvement in functional outcome and physical quality of life.
Another case series on patients with VTOS included 15 patients each with supra-
clavicular and 15 patients with infraclavicular decompression [11]. Infraclavicular thoracic outlet decompression was associated with fewer patients with postopera­tive symptoms, 0 of 15 (0%) versus 8 of 15 (53.3%), (P=0.0022), and infraclavicu­lar thoracic outlet decompression demonstrated improved patency, 15 of 15 (100%) versus 8 of 15 (53.3%), (P = 0.028) at a mean combined follow-up of
8.47±10.8months. The supraclavicular approach was also compared with the infraclavicular
approach in patients with VTOS by Dua et al. [12]. 109 rib resections were per­formed, 54 via a supraclavicular and 55 via an infraclavicular approach. There was a signicant decrease in the number of complications in the infraclavicular cohort compared with the supraclavicular group, but the long-term outcomes in terms of venous patency and quality of life did not differ between the two groups. Dua etal. [13] also reported long-term functional outcomes in 170 patients with 188 rib resec­tions for all forms of TOS (102 NTOS, 82 VTOS, 4 ATOS) with a mean follow-up time of 5.3years. 167 (88.9%) patients returned to baseline activities postopera­tively, this included active athletes, and 96% of patients stated they were satised and would undergo the procedure again.
Silverberg etal. [14] chose a different approach. They reported the long-term
results in 18 patients with Paget-Schrötter syndrome who were treated exclusively with anticoagulation and catheter-directed thrombolysis (up to 72h) without surgi­cal management. Patients excluded were those with a clinical presentation exceed­ing 14days from the development of symptoms to hospital admission and patients with an existing contraindication to thrombolysis. After thrombolysis, the treated veins were found to be patent on venography in 16 of 18 patients. Two patients had occluded subclavian/axillary veins. All patients with successful catheter-based thrombolysis were found to have focal stenotic lesions in the subclavian vein; PTA was performed in only 4 patients with high-grade stenosis >80%. After thromboly­sis, patients were anticoagulated for a mean of 26 months. Mean time from the index event to the follow-up clinic visit was 109 (37–176) months. 17 patients (94%) had a Villalta score of 0–3, equivalent to non-existent post-thrombotic syn­drome. 14 patients (78%) were completely asymptomatic. None of the patients suf­fered from impaired quality of life. The study demonstrates that patients with Paget-Schroetter syndrome treated with catheter-directed thrombolysis and
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anticoagulation, but no rib resection did not develop post-thrombotic syndrome and did not suffer from an impaired quality of life.
2.2.2.3 NTOS: Outcome
Balderman etal. [15] assessed the results of physical therapy management and sur­gical treatment in a prospective observational cohort of patients with neurogenic thoracic outlet syndrome (NTOS) using patient-reported outcomes measures. During a median follow-up >12 months of 130 patients with NTOS, 40 (31%) obtained symptom improvement with physical therapy alone and 90 (69%) under­went surgery. Surgery for NTOS consisted of supraclavicular decompression (with complete anterior and middle scalenectomy, rst rib resection, and brachial plexus neurolysis) or subcoracoid decompression (pectoralis minor tenotomy), or both. Patients rated the outcome after surgery as excellent in 27%, good in 36%, fair in 26% and poor in 12%. Despite substantial pretreatment disability, surgery for NTOS can be effective when physical therapy is insufcient, with substantial symptom improvement in approximately 90% of patients. These outcomes were reected by changes in Disability of the Arm, Shoulder, and Hand scores, but no specic factors predicted which patients will benet from physical therapy alone or will require surgery.
The rst single centre prospective cohort study to describe the implementation of
a multidisciplinary diagnostic care pathway for patients with NTOS based on the SVS reporting standards was published by Pesser etal. [16]. NTOS was diagnosed in 476 of 856 (55.6%) referred patients. Dedicated physiotherapy as the primary treatment was successful in 186 patients (39.1%). Surgical treatment was performed in 290 (60.9%) patients, of whom 274 were included in the follow up (mean follow­ up 16.9±9.2months). Surgical treatment consisted of thoracic outlet decompres­sion, which included a complete resection of the rst rib, partial anterior and medial scalenectomy and a thorough neurolysis of the branches of the lower brachial plexus. At follow-up, surgical outcome was considered excellent in 83 patients (30.3%), good in 114 (41.6%), fair in 43 (15.7%) and poor (recurrent or persistent NTOS) in 34 (12.4%). The results suggest a prominent role for multidisciplinary care pathways in NTOS diagnostics, underline the role of dedicated physiotherapy as the primary treatment, and show good to excellent short term treatment outcomes in the majority of the surgically treated NTOS patients following previous unsuc­cessful physiotherapy.
Johansen [17] questioned the indication for rst rib resection in NTOS. He
reported on a retrospective series of 504 thoracic outlet decompression procedures in 442 patients with scalene block-proved neurogenic thoracic outlet syndrome. Patients underwent anterior, middle and minimus scalenectomy and brachial plexus neurolysis via a supraclavicular incision, combined with pectoralis minor tenotomy through a small vertical infraclavicular incision. No rst ribs were excised, and all procedures were performed by a single surgeon. All patients had a positive preop­erative response to scalenus blockade using local anaesthetic. The average operative
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time was 1.15h, and the average length of stay was 1.05days. Major complications (intraoperative arterial injury, wound haematoma requiring reoperation, and chylo­thorax) were seen in 7 patients (1.4%). All but two patients (99.6%) had symptom­atic improvement, with 90.9% experiencing ≥50% improvement in preoperative symptoms and function. These results make it questionable whether the rst rib should actually be resected in NTOS.
Ruopsa etal. [18] also pointed out that rib resection was probably unnecessary
in most of their patients with NTOS. They reported long-term outcomes in 94 patients with NTOS and scalenectomy with a mean follow-up of 12.9years. 82% of women and 57% of men reported that aid from surgery had been excellent or good. 69% reported that surgery helped considerably for at least a mean of 9.9years. The weak point of this study is the fact that of 210 patients operated on, only 94 (47%) ultimately accepted the invitation for a follow-up examination.
The learning curve for supraclavicular thoracic outlet decompression with rst
and/or cervical rib resection of a single surgeon was reported by Panda etal. [19]. Indication was an NTOS.The mean operative time was 201.5 (SD 56.7) minutes for 114 patients. A plateau was reached after 45 to 55 operations. The operative times before and after the rst 51 operations (rst and second collectives) were 231.1 (213.6–248.6) vs. 178.3 (168.4–188.3) minutes (p<0.001). There were no signi­cant differences between the two groups regarding the length of postoperative stay, duration of closed-suction drainage, and frequency of complications. At follow-up after a mean of 90days, patients in both the early and late cohorts reported a signi­cant improvement in their symptoms (74% vs. 73%). Certain preoperative consider­ations (ASA classication and failed preoperative botulinum injection) were associated with minimal improvement in postoperative symptoms.
Goeteyn etal. [20] highlighted the controversial surgical treatment of NTOS and
conducted the rst (small) randomised trial to objectify the effect of thoracic outlet decompression (TOD). Patients with a diagnosis of NTOS refractory to conserva­tive therapy were randomised to one of two intervention arms, receiving either a transaxillary thoracic outlet decompression (complete resection of the rst rib and partial scalenus resection) (n=25) or continued conservative treatment (n= 25). After 3 months, the conservative treated group was also offered a transaxillary tho­racic outlet decompression. The primary outcome was change in the Disability of the Arm, Shoulder and Hand (DASH) questionnaire score. At 3months, there was a statistically signicant difference in DASH scores (transaxillary decompression mean 45.15, conservative treatment 64.92; p<0.001). All patients in the conserva­tive treatment group applied for surgery 3 months after randomisation. After surgery of the conservative treatment group, there was no statistically signicant difference between the groups for all primary and secondary outcome measures. The conclu­sion was that surgical thoracic outlet decompression is effective in improving symp­toms in patients with NTOS who do not respond to conservative treatment.
In addition, Goeteyn etal. [21] reported the results of redo thoracic outlet decom-
pression surgery through the supraclavicular approach for persistent or recurrent NTOS.Recurrent NTOS was assumed to occur when symptoms reappeared after an initial successful period following a rst thoracic outlet decompression. The redo
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procedure consisted of complete (cartilage-cartilage) resection of the rst rib, any bony and brous anomalies, complete anterior and middle scalenectomy, and com­plete neurolysis of the brachial plexus (complete anatomical decompression of the brachial plexus). In total, 45 patients had redo surgery. Postoperative complications were seen in eight patients (18.18%). One patient had postoperative complications with permanent impairment (Horner syndrome). Seven patients had postoperative complications with full recovery (three patients had a chylous leakage that was treated with a median-chain triglycerides diet for 6weeks, three patients had tran­sient phrenic nerve palsy with full recovery <6weeks, and one patient had a discrete Horner syndrome that resolved in 6 weeks). The median time of follow-up was
19.50months (interquartile range, 14.00months). Postoperatively, there was a sig-
nicant decrease in the DASH score. Patients with rst rib remnants showed a sig­nicant better response compared with patients without rst rib remnants.
2.2.2.4 ATOS: Outcome
ATOS is the least common presentation of TOS, accounting for 1% to 2% of cases. Pantoja etal. [22] saw only 51 cases with ATOS among 2200 patients with TOS between January 1986 and March 2021. Subclavian artery pathology included 16 aneurysms (31%), 15 stenoses (29%), and 19 occlusions (37%). Transaxillary decompression was performed in 50 patients, including 50 rst rib resections and 23 transaxillary cervical rib resections. Among the 51 decompression cases, approxi­mately 41% (21 cases) required arterial reconstruction: 13 open surgical reconstruc­tions (11 bypass grafts, 2 primary aneurysm repairs) and 8 stentgraft repairs. Comparing two time periods (1986–2003 vs 2003–2021), thrombolysis (5 vs 10) and stentgraft repairs (0 vs 8) increased, whereas open subclavian artery repair (11 vs 2) decreased. Return-to-work rates (74% vs 96%) and good-excellent Derkash scores (81% vs 96%) were comparable. The study described the evolving role of endovascular management of ATOS over the past two decades and documented the expanded role of vascular surgeons in the endovascular management of ATOS at a single institution. Compared with open repair, stent graft repair of the subclavian artery may be associated with shorter operative times, less blood loss, but decreased patency, without changes in long-term functional outcomes.
2.2.2.5 Transaxillary Access
Stilo etal. [23] reported a single surgeon’s technique and experience with rst rib resection through transaxillary approach, video-assisted in recent years, in 103 patients with TOS (NTOS 58.2%, VTOS 31.1%, ATOS 7.76%). Thirteen (12.6%) patients had cervical rib and sixteen cases (15.5%) had bilateral TOS.The technical success rate was 100%. Three patients (2.9%) presented with hand ischemia and needed an arm vein bypass after rib resection. One (0.9%) intraoperative arterial injury was reported, and nerve injury rate was 1.8%. At 30-day re-intervention rate
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was 0.9%: one patient experienced hemothorax solved by thoracoscopic drainage. Restrict pneumothorax was reported in 42 patients (40.8%) treated with pleural drainage. At mean follow-up of 93± 9 months partial symptoms recurrence was present in 6 patients (5.8%). The authors advocated the transaxillary approach with resection of the rst rib as a safe standard procedure for TOS.
Experience with a modication of the transaxillary rst and cervical rib resection
technique was described by Moridzadeh etal. [24]. Instead of transecting the ribs en bloc, this approach relied on dividing the junction at the rst and cervical ribs and then removing the individual segments. The separated segments of the rst rib could then be individually distracted, allowing for improved mobilization, retraction, dis­section, and removal of the individual segments. All cases were performed with the assistance of high-denition video endoscopy. 38 patients had undergone complete transaxillary resection of 40 fully formed cervical ribs (10 class 3 and 30 class 4). Of these 38 patients, 74% were women. The presentations had been neurogenic (65%), arterial (31%), and venous (5%). The duration of surgery averaged 141min, blood loss was 65mL, and length of stay was 2.1 days. None of the patients had experienced brachial plexus, phrenic, or long thoracic nerve injury. The average follow-up period was 65months. The nal mean postoperative SPS and QuickDASH scores were lower than the scores at presentation (SPS score, 6.4 vs 1.2; P<.001; QuickDASH score, 50 vs 17; P< .001). Transaxillary resection of fully formed cervical ribs should be considered a safe and effective operation.
2.2.2.6 Robotic Surgery
Martinez etal. [25] presented a retrospective review and analysis of data collected from a 16-year experience of a single surgeon using a robotic surgical system and technique for TOS surgery. 412 transaxillary decompression procedures using the robotic da Vinci Surgical System were performed in 306 patients for rst rib resec­tion and cervical band removal. In 204 (49%) procedures, the transaxillary approach was used alone, and in 174 (41%) operations an open supraclavicular approach preceded the robotic transaxillary approach (for cervical rib excision (n=48) and anterior scalenectomy). In 126 of 412 (31%) operations, the supraclavicular approach was for total anterior scalenectomy in patients who had predominantly had cervical C5, C6, and C7 upper plexus syndrome. None of the patients died, and no patient experienced permanent neurovascular damage of the extremity. Of the 306 patients, 22 (5% of 441 operations) experienced complications. One patient developed postoperative scarring that required a redo operation with a robotic­assisted transaxillary approach. The authors concluded that the endoscopic robotic­assisted transaxillary approach offers a safe and effective approach for performing thoracic outlet syndrome surgery because it provides a three-dimensional visual magnication of the anatomic area and facilitates observation of the cervical bands and the mechanism of neurovascular compression causing TOS.
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2.2.2.7 Botulinum Toxin Injections inPatients withNTOS
In a retrospective analysis, Donahue etal. [26] examined the role of botulinum toxin (BTX) injections of anterior scalene (AS) and pectoralis minor (PM) muscles in patients undergoing surgery for NTOS. They hypothesized that symptomatic improvement from BTX injections correlates with favorable long-term response to surgery for NTOS.Data of NTOS patients who underwent sonographically guided chemodenervation of AS and PM using BTX type A followed by scalenectomy and rst rib resection were analysed. A supraclavicular approach was chosen for the surgical procedure. There were 157 patients with 178 BTX injections. A high pro­portion of responders to BTX injections also responded to surgery (positive predic­tive value of 99%), and BTX injections showed high specicity (90%). BTX injections were moderately sensitive (66%) and accurate (67%) to determine surgi­cal response and had low negative predictive value (14%). The study demonstrated that favorable response to sonographically guided BTX injections is associated with favorable surgical outcomes at 2years follow-up and has high positive predictive value. While the positive predictive value of BTX injection was high, the negative predictive value was low. Only 14% of patients who did not respond to BTX injec­tion also did not respond to surgery. The low negative predictive value obviously limits the value of the test.
2 Thoracic-Outlet-Syndrome
2.3 Conclusions forClinical Practice
1. Very few (randomised) studies are available on the treatment of TOS, evidence-
based statements on therapy cannot be made. The Society for Vascular Surgery has adopted reporting standards. The standards dene terminology, local nd­ings, diagnostics, and severity assessment and describe the treatment and the requirements for the presentation of results.
2. The therapy must be based on the ndings, whereby a strict distinction must be
made between neurogenic (NTOS), venous (VTOS), arterial (ATOS) TOS, and their combinations.
3. In the major registries, rst rib resection is favoured as therapy, which is proba-
bly also justied if a cervical rib is in addition resected.
4. In NTOS, rst rib resection is controversial; supraclavicular decompression
without resection of the rst rib probably has the highest success rate in NTOS.In VTOS (Paget-Schrötter syndrome), the data suggest that rst rib resection after thrombolysis is the treatment of choice in the majority of patients.
5. It is undisputed that the care of TOS patients should be reserved for specialised
centres.