|Year : 2018 | Volume
| Issue : 2 | Page : 100-104
Management and outcomes of patients with chronic upper limb ischemia secondary to arterial thoracic outlet syndrome
Sandeep Mahapatra, Pinjala Ramakrishna, Muneer Ahmad Para, Venugopal Mustyala, Praveen Kumar Nookala
Department of Vascular Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Web Publication||3-May-2018|
Dr. Sandeep Mahapatra
Department of Vascular Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
Introduction: Post stenotic dilation of the subclavian artery by cervical rib compression is generally seen in young patients with upper limb ischemia. Materials and Methods: We conducted a retrospective study on 26 consecutive patients who underwent surgical decompression for arterial thoracic outlet syndrome (aTOS) with subclavian artery repair from 2010 to 2015. Supraclavicular decompression of the thoracic outlet with cervical rib excission, scalenectomy with subclavian artery reconstruction by aneurysmorrhaphy was performed as per Scher staging of aTOS. The management and post operative outcome with regards to objective changes in the upper limb arterial pressure was studied & followed for 1 year with clinical examination, duplex scan and non invasive segmental vascular pressure. Result: The average age at presentation was 32 years, with equal gender distribution. However, symptomatic right: left aTOS at presentation was 18:8. The Scher classification system for aTOS based on subclavian artery compression identified 14 patients in stage III, 10 patients in stage II and 2 in stage I .8 out of 26 patients had digital ischemia with minor tissue loss and were managed medically by intravenous Alprostadil (Prostaglandin E1) postoperatively for 6 months .The mean above elbow pressure (AEP) before surgery has improved from 62.08±12.97 to 108.46±16.81& the below elbow pressure (BEP) has improved from 48.00±13.13 to 93.46± 32.02 . Above elbow pressure improvement is found statistically significant (p value0.037) across all Scher stages. Complete relief of vascular symptoms was seen in all patients immediately or gradually over a period of 6 months. Minor amputation was carried out in 8 patients of Scher stage 2 & 3 aTOS during follow of 6 months. Conclusion: This study finds its uniqueness in demonstrating the objective improvement of pressure with respect to different Scher stages which which is not reported in the literature.
Keywords: Aneurysmorrhaphy, scalenectomy, Scher staging
|How to cite this article:|
Mahapatra S, Ramakrishna P, Para MA, Mustyala V, Nookala PK. Management and outcomes of patients with chronic upper limb ischemia secondary to arterial thoracic outlet syndrome. Indian J Vasc Endovasc Surg 2018;5:100-4
|How to cite this URL:|
Mahapatra S, Ramakrishna P, Para MA, Mustyala V, Nookala PK. Management and outcomes of patients with chronic upper limb ischemia secondary to arterial thoracic outlet syndrome. Indian J Vasc Endovasc Surg [serial online] 2018 [cited 2022 Jan 27];5:100-4. Available from: https://www.indjvascsurg.org/text.asp?2018/5/2/100/231842
| Introduction|| |
The description “thoracic outlet syndrome” (TOS) was first coined by Peet et al. in 1956 to describe symptoms arising from compression of one or more of the neurovascular structures (brachial plexus, subclavian vein, or subclavian artery) at the thoracic outlet. In the West, neurological TOS (nTOS) is the most common presentation (about 95% in most series). Diagnosis of nTOS can be difficult because of nonspecific presenting symptoms with no objective diagnostic gold standard investigation to confirm the diagnosis.
In India, most vascular surgical centers recognize that patients with arterial TOS (aTOS) form a higher percentage of referrals to their service than nTOS. However, it is often misdiagnosed at the primary care level for some form of neuropathy. The patients are finally referred to a vascular center with advanced ischemia secondary to outflow occlusion from distal thromboembolism. In such patients, the role of noninvasive vascular laboratory testing, imaging, operative intervention, and long-term outcomes is not objectively described in the available literature. We assessed our cohort of patients with aTOS to detail the diagnostic and management protocol, postoperative outcome as per Scher stages with regard to objective changes in the upper limb arterial pressure was studied. The patients were followed with clinical examination, duplex scan, and noninvasive segmental vascular pressure recording of the upper limb for 1 year to check the relief of pain and improvement in upper limb pressure.
| Materials and Methods|| |
Demographic data, clinical presentation, investigation, procedural and outcome data of 26 consecutive patients operated in the Vascular Surgical Unit of Nizam's Institute of Medical Sciences, Hyderabad, with the diagnosis of aTOS from 2010 to 2015 were reviewed retrospectively from the hospital records. The Institutional ethical committee approval was obtained for the study. Patients were diagnosed to have aTOS based on classic symptoms and signs of chronic (>2 weeks) or acute ischemia (<2 weeks) with a directed vascular examination. The loss of pulses either at rest or with provocative maneuvers (Adson's and elevated arm stress test), presence of bruit at supraclavicular or infraclavicular fossa, presence of thromboembolic hand or digit lesions, ulceration or gangrene, and neurological examination were noted. Details of noninvasive vascular laboratory studies (finger–ipsilateral brachial index, digital brachial index (DBI), and pulse waveform analysis) and imaging (anteroposterior neck X-ray, arterial duplex, and computed tomography [CT] angiogram) were recorded for all patients. Critical limb ischemia (CLI), for the upper limb was defined as rest pain, tissue loss (ulceration/gangrene), below-elbow pressure <60 mmHg, finger–ipsilateral brachial index <0.7, or DBI <0.5.
Patients were classified according to the Scher  classification (Stage 0 = asymptomatic compression; 1 = poststenotic dilatation; 2 = subclavian mural thrombus with intimal damage; and 3 = distal embolization) [Figure 1]. All patients were managed based on a protocol-based approach with preoperative assessment of the distal circulation and the nature of the arterial anomaly as per Scher stages. Surgical decompression of the thoracic outlet was performed by removal of a segment of cervical rib from its attachment with the first rib to its proximal attachment with the transverse process of the cervical vertebrae. The compressing band of scalenus anticus muscle overlying the second part of the subclavian artery was excised to relieve the pinchcock effect. The dilated or aneurismal subclavian artery was endarterectomized and a distal embolectomy using Fogarty catheter was done in Scher Stage 2 and 3. Aneurysmorrhaphy of the subclavian artery by approximating the two walls matching the proximal healthy subclavian artery was done using monofilament nylon sutures for 24 patients with subclavian artery aneurysm.
|Figure 1: Distribution of the patients of arterial thoracic outlet syndrome as per Scher's stages|
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General measures such as hand care and physiotherapy were advised in those with minor tissue loss undergoing digital amputation. The patients with digital ischemia with rest pain received 500 micrograms of injection alprostadil  distributed over 5 days, as a daily infusion of 100 micrograms diluted in 50 ml of normal saline in a pump at a rate of 40 microdrops/min, monthly once for 6 months. Operative success was defined as relief of vascular symptoms postoperatively with improvement in the below-elbow pressure above the critical level. Statistical analysis was performed for the Scher stage, presence of critical ischemia, and change in noninvasive tissue perfusion indices pre- and post-operatively. Patients were followed up by noninvasive vascular laboratory testing with upper limb pressure measurements (both below elbow and above elbow level) at 3 weeks, 3, 6, 12 months, and then as needed. All patients received dual antiplatelets (aspirin 75 mg + clopidogrel 75 mg once daily) for a minimum of 1 year, followed by aspirin 75 mg lifelong unless contraindicated.
| Results|| |
There were 26 patients (13 males, 13 females; 26 limbs) diagnosed to have aTOS, undergoing operative management [Table 1]. The average age at presentation was 32 years (range 15–55 years). A total of 8 patients presented with acute-on-chronic upper limb ischemia with an average 7-day duration of symptoms of severe pain in the hand and forearm and bluish discoloration of digits with preexisting claudication lasting from 4 to 10 months. Eighteen others presented with the history suggestive of an undiagnosed episode of upper limb thromboembolism and chronic upper limb ischemia; with an average duration of symptoms of 8 months. In our cohort, 10 patients had upper limb exertional pain, palpable supraclavicular subclavian artery, and supraclavicular bruit documented. Two out of eight patients of acute on chronic ischemia and 6 out of 18 patients of chronic ischemia had single or multiple digital tip gangrene at presentation accounting for minor tissue loss. None of the patients had major tissue loss mandating primary above-elbow amputation of the upper limb.
|Table 1: Demographic distribution of patients of cervical rib with arterial obstruction|
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Complete cervical ribs were detected on X-ray neck in all patients, four patients with bilateral cervical ribs were asymptomatic on the contralateral arm. Arterial duplex and CT angiogram were performed in all patients. Two patients had evidence of compression on provocative maneuvers. A total of 24 patients had arterial poststenotic dilatation, and 14 patients had mural thrombus inside. In total, 14 patients had complete occlusion of the distal outflow with imaging revealing nonopacification of brachial inflow and patchy refilling of radial or ulnar and a single-vessel supply to the hand. The distribution of patients for the subclavian artery pathology associated with cervical rib as per Scher Staging 1, 2, and 3 was 7.7%, 38.5%, and 53.8%, respectively [Figure 1]. Eight patients had distal vessel occlusion with minor tissue loss, treated elsewhere with a diagnosis of neurodegenerative pathology.
The mean preoperative above-elbow pressure (AEP) was 62.08 ± 12.97 mmHg and below-elbow pressure was 48.00 ± 13.13 [Table 2].
|Table 2: Assessment of above-elbow pressure and below-elbow pressure before and after surgery|
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Decompression of the thoracic outlet comprising of cervical rib excision and scalenectomy was performed through a supraclavicular approach in all cases. Subclavian aneurysmorraphy was performed in 24 patients with longitudinal arteriotomy followed by endarterectomy of the subclavian artery in Scher's Stage 2 and 3. Only decompression was carried out in two patients of aTOS in the present series. Longitudinal brachial plexus neuronolysis following complete rib excision was performed as an adjunct in ten patients when the upper plexus was seen to be tented by the cervical rib. Fourteen patients with concomitant distal embolization underwent embolectomy. Brachial embolectomy with directed radial and ulnar embolectomy was successful in 4, and partially successful in 8 (Fogarty catheter could be passed only till the mid-forearm). None of the patients underwent first-rib resection in our series.
Postoperatively, follow-up was clinically supplemented with noninvasive vascular laboratory tests and duplex ultrasound of the upper limb. All patients had symptomatic relief and had regained functional use of the hand at a median follow-up of 12 months. The mean above- and below-elbow pressures improved to 108.46 ± 6.81 and 93.46 ± 32 mmHg and the improvement was statistically significant in comparison to the preoperative values (P < 0.001). This improvement was seen in patients all across the Scher stages [Figure 2]. There was approximately 50 mm increase in AEP head in all patients irrespective of Scher stages that improves the distal circulation even if the inline flow could not be established after surgery. The below-elbow pressure head improved significantly (approximately 70 mmHg) in patients presenting with Scher Stage 1 compared to approximately 46 mmHg improvement in Scher Stage 2 and 40 mmHg in Stage 3. This may be due to the chronic thrombotic occlusion secondary to the distal embolization for a long of period time with luminal obliteration in Scher Stage 3 aTOS. Fogarty catheter (no 2 Fr) could not be passed beyond mid-forearm in those patients. Three patients of Scher Stage 2 and 5 patients of Scher Stage 3 were in CLI (below-elbow pressure <60 mmHg) in spite of surgery [Table 3]. Those patients received alprostadil for digital embolization over 6 months. They then underwent partial digital amputation for digital gangrene; all wounds healed; none of the patients required a major limb amputation. Five patients had postoperative complications that included wound hematoma, seroma, and loss of sensation in the infraclavicular region. However, the symptoms resolved over 1–3 months. There was no postoperative mortality. Postprocedure duplex surveillance of the subclavian artery for 1 year has not shown any significant change in the diameter or luminal irregularities in the artery.
|Figure 2: Improvement of the above-elbow pressures after surgery in relation to the Scher stages|
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|Table 3: Analysis of patients remaining in critical limb ischemia following arterial thoracic outlet obstruction surgery|
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| Discussion|| |
aTOS, although rare, is the most clinically apparent of the TOS. An objective diagnosis is usually possible even when there is a mixed presentation with neurologic or venous TOS. Our cohort of patients presented often with an advanced ischemia of hand or arm. We did not have any patients with asymptomatic compression or neurological symptoms. All patients had an underlying cervical rib that is similar to what is reported in other series.,, Most series describe aTOS to occur in the young as in our study. Although nTOS has a female preponderance, this is not the case in aTOS, as in our series [Table 1]. The clinical presentation and noninvasive vascular laboratories revealed a high rate of advanced ischemia. This late presentation correlated with a corresponding higher number of patients who had thromboembolic occlusion of the distal circulation. Undiagnosed presentation has been reported from 10% to 30% of patients in other series. Late presentation and delayed referral can lead to significant morbidity with hand ischemia, loss of function, or limb amputation. Despite this, improvement of inflow alone resulted in opening up the collateral bed in distribution vessels improving tissue perfusion, aiding ulcer healing, and relieving rest pain. We observe a demonstrable rise (mean 40 mmHg) pressure improvement in below-elbow pressure in Scher Stage 2 and 3. Both the above elbow as well as below-elbow pressures improved significantly postoperatively. The successes of surgical decompression of TOS or outcomes that have been reported in the literature vary among different series and certainly depend on the definition used for the success as well as TOS type. This is reflected in the recent Society for Vascular Surgery reporting standard for the management of TOS.
All patients required arterial repair, which is a little higher than what is reported in other series, but this may again be due to a referral bias. Operative decompression involved complete removal of the offending cervical rib with the arterial pathology tackled by aneurysmorrhaphy with good technical results and clinical success. None of the patients required major amputation, in comparison to a failure rate of 10%, and amputation rate of 3% in other series.
Cervical ribs were first described by Gruber  in 1869. He described four groups of cervical ribs (1) cervical ribs extending just beyond the transverse process, (2) cervical ribs extending beyond the transverse process with a free tip almost touching the first rib,(3) cervical ribs extending beyond the transverse process with fibrous bands or cartilage attaching to the first rib, and (4) cervical ribs completely fused to the first rib [Table 1]. The latter two categories of cervical ribs can present with vascular compromise, including arterial compression, thrombosis, and aneurysm formation.
The three main components of treatment include relieving the arterial compression, removing the source of embolus, and restoring the distal circulation. Cervical rib-associated vascular thoracic outlet obstruction is given a management algorithm by Scher et al. The anatomic and pathophysiologic basis of the syndrome are reviewed in a series of 12 patients with 15 arterial lesions. In 1916, Halsted  evaluated 716 case reports of cervical ribs, in which 125 had vascular symptoms and 27 had aneurysms distal to the point of compression by the cervical rib and its attachments. In 1934, Lewis and Pickering  reported that subclavian artery thrombosis and embolization can occur due to trauma caused by a cervical rib.
The DBI, an “upper extremity ankle–brachial index,” is defined by the ratio of the systolic arterial pressure of the digit to the systolic brachial pressure. Significant pressure gradient between the two segments defines obstructive (structural) disease (stenosis; thrombosis, embolus) in the intervening segment. Each individual segmental arterial systolic pressure can be obtained using the placement of pressure cuffs and a measure of distal flow; for example, Doppler pressure recording above elbow and below elbow, both before and after the surgery and palpation. In general, DBIs >0.7 indicate adequate circulation, whereas values of 0.7 or less suggest inadequate arterial flow to the digit and support intervention. An obstruction between the brachial artery and digital arteries may lower DBI, and for example, a DBI of 0.5 may be indicative of severe disease and imminent digital gangrene defined as the CLI.
An aneurysm is defined as an increase in diameter greater than twice normal. The artery is said to be dilated when the diameter is less than double that of the adjacent normal arterial segment, Poland  was the first to describe this interesting symptom in 1869, in a patient suffering from compression of the subclavian artery by a cervical rib. Various methods described for the arterial reconstruction for subclavian artery dilatation are endoaneurysmorrhaphy, aneurysmorrhaphy plus thromboendarterectomy, resection anastomosis, vein graft interposition, arteriolysis plus arteriotomy, and thromboendarterectomy. In most cases of arterial dilatation, an arteriotomy was carried out to verify the presence of an intimal lesion. The arteriotomy was then closed in such a way as to restore the arterial caliber to normal by a means called aneurysmorrhaphy. This procedure was first advocated by Dr. Rudolph Matas in 1903. The technical simplicity using native artery for repair of the aneurismal subclavian artery has drawn attention for the in situ repair with a promising result. One-year follow-up report from the present study using aneurysmorrhaphy of the subclavian artery, substantiated by duplex scan further emphasizes on Matas's technique of in situ aneurysm repair. Sanders and Pearce  found that only 11 of 2500 TOS operations performed over 40 years were for aTOS in their center. A retrospective analysis in our institution conducted by Singh et al. with 72 patients between 15 and 55 years looked for neurovascular complications of TOS for 15 years. They concluded that scalenectomy with cervical rib excision through supraclavicular approach gives good and lasting results with an acceptable outcome and minimum morbidity.
When a long cervical rib is present, most authors favor a supraclavicular approach that involves complete excision of the cervical rib and additional soft-tissue compressive elements without first rib excision. The justification in favor of not resecting the first rib is that the arterial axis in these cases is quite free after the supraclavicular procedure and that arterial compression has not recurred in cases managed in this way in follow-up.
Long-term, intermittent intravenous infusion of prostanoids, such as prostaglandin E1 (PGE1) or more stable prostacyclin analogs such as iloprost have been shown to reduce rest pain and ischemic ulcerations in masked, placebo-controlled trials. The mechanisms of anti-ischemic effects of PGE1 in patients with Peripheral Arterial Occlusive Disease are probably complex and clearly not limited to a direct vasodilator action. In addition to the known effects on blood flow, viscosity, fibrinolysis, and platelet aggregation, the compound also inhibits monocyte and neutrophil function, suggesting that PGE1 will also have anti-inflammatory effects, inhibition of expression of adhesion molecules, release of inflammatory cytokines (TNF-α, MCP-1), matrix components and decrease endothelin-1 levels, and release of growth factors to help in ulcer healing. All our patients having CLI, even following the surgery for arterial thoracic outlet obstruction received injection PGE1 for 6 months on monthly follow-up along with antiplatelet drugs and peripheral arterial vasodilators. The rest pain subsided and the ulcer healed in all 8 patients with CLI.
In the evaluation of these results, certain limitations merit emphasis. The smaller number (N) is insufficient to give a generalized statement for the community. The data collected from hospital records, being retrospective in nature, carries inherent statistical drawback for generalization.
| Conclusion|| |
Cervical rib presenting with arterial thoracic outlet obstruction managed as per Scher stages focuses on improving the pressure head in the upper limb arteries, thus relieving the ischemic rest pain. Protocol-based approach Scher stages will find its place in objective comparison of different studies with uniform reporting standards in the management of aTOS. This study finds its uniqueness in demonstrating the objective improvement of pressure with respect to different Scher stages even in the presence of advanced ischemia and extensive outflow disease.
We are deeply indebted to Dr. Indrani Sen, vascular surgeon, research scholar in Mayo Clinic, USA, for guidance and able editing in preparation of the manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Peet RM, Henriksen JD, Anderson TP, Martin GM. Thoracic-outlet syndrome: Evaluation of a therapeutic exercise program. Proc Staff Meet Mayo Clin 1956;31:281-7.
Singh D, Pinjala RK, Reddy LR, Vani SP. Thoracic outlet syndrome: Presentation and management. Indian J Surg 2006;68:93-6.
Zimmerman NB. Occlusive vascular disorders of the upper extremity. Hand Clin 1993;9:139-50.
Mahapatra S, Ramakrishna P, Joopalli P, Naqvi Syed M. The effect of prostaglandin therapy on ankle brachial index in non-reconstructable symptomatic peripheral artery disease patients-retrospective analysis. J Vasc Med Surg 2015;3:222.
Scher LA, Veith FJ, Haimovici H, Samson RH, Ascer E, Gupta SK, et al.
Staging of arterial complications of cervical rib: Guidelines for surgical management. Surgery 1984;95:644-9.
Ramakrishna P. Intravenous infusion of prostaglandin E 1 in extremity ischemia. Indian J Vasc Endovasc Surg 2017;4:38-42. [Full text]
Short DW. The subclavian artery in 16 patients with complete cervical ribs. J Cardiovasc Surg (Torino) 1975;16:135-41.
Sanders RJ, Annest SJ. Thoracic outlet and pectoralis minor syndromes. Semin Vasc Surg 2014;27:86-117.
Likes K, Rochlin DH, Call D, Freischlag JA. Coexistence of arterial compression in patients with neurogenic thoracic outlet syndrome. JAMA Surg 2014;149:1240-3.
Meumann EM, Chuen J, Fitt G, Perchyonok Y, Pond F, Dewey HM, et al.
Thromboembolic stroke associated with thoracic outlet syndrome. J Clin Neurosci 2014;21:886-9.
Bhattacharya V, Hansrani M, Wyatt MG, Lambert D, Jones NA. Outcome following surgery for thoracic outlet syndrome. Eur J Vasc Endovasc Surg 2003;26:170-5.
Illig KA, Donahue D, Duncan A, Freischlag J, Gelabert H, Johansen K, et al.
Reporting standards of the society for vascular surgery for thoracic outlet syndrome: Executive summary. J Vasc Surg 2016;64:797-802.
Gruber W. Ueber die halsrippen des menschen, mít vergleíchendanatomischen bemerkungen. Mém Acad Sci 1869;2:7-27.
Halsted WS. An experimental study of circumscribed dilation of an artery immediately distal to a partially occluding band, and its bearing on the dilation of the subclavian artery observed in certain cases of cervical rib. J Exp Med 1916;24:271-86.
Lewis T, Pickering GW. Observations upon maladies in which blood supply to digist ceases intermittently or permanently, and upon bilateral gangrene or digits. Clin Sc 1934;1:327.
Buehner JW, Koontz CL. The examination in the vascular laboratory. Hand Clin 1993;9:5-11.
Poland A. On a case of fusiform and tubular aneurism of the subclavian artery, and its successful treatment by indirect digital compression. Med Chir Trans 1869;52:277-307.
Cormier JM, Amrane M, Ward A, Laurian C, Gigou F. Arterial complications of the thoracic outlet syndrome: Fifty-five operative cases. J Vasc Surg 1989;9:778-87.
Gage IM. The technical simplicity of the matas endo-aneurysmorrhaphy. Ann Surg 1944;119:468-73.
Sanders RJ, Pearce WH. The treatment of thoracic outlet syndrome: A comparison of different operations. J Vasc Surg 1989;10:626-34.
[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]