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      Digital ischaemia of the upper limbs in middle age: consider arterial thoracic outlet syndrome until proven otherwise!

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          Abstract

          Objectives

          Patients presenting with digital upper limb ischaemia are occasionally referred to rheumatology services to rule out vasculitis. We aimed to present two cases of delayed diagnosis of arterial thoracic outlet syndrome (aTOS) in middle-aged patients presenting with digital ischaemia in order to raise awareness of this important pathology that requires timely surgical intervention.

          Methods

          Two cases of progressive ischaemia of the right upper extremity caused by primarily undiagnosed compression of the subclavian artery by an accessory cervical rib are presented. The case notes, radiological images, intra-operative and postoperative findings for both patients were reviewed. Patients were followed up after ≥6 months to assess prognosis.

          Results

          Both patients had a working diagnosis of Buerger’s disease and had been treated with prostaglandin infusions before establishment of the diagnosis of arterial thoracic outlet syndrome. Both patients were heavy smokers, and one patient had bilateral symptoms and a history of axial SpA and positive HLA-B27. Late presentation in one patient led to the loss of three fingers and the need for plastic reconstructive surgery after cervical rib resection and revascularization. In the other patient, surgical thrombectomy of the upper limb arteries along with resection of a cervical rib and repair of the subclavian artery with an interposition graft were necessitated to heal digital ulcers successfully.

          Conclusion

          A high index of suspicion of aTOS should be maintained in middle-aged patients presenting with digital or upper limb ischaemia even in the presence bilateral symptoms or relevant risk factors of other diagnoses, such as smoking or a positive rheumatological history.

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          Most cited references14

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          Thromboangiitis obliterans (Buerger's disease).

          J W Olin (2000)
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            Thoracic-outlet syndrome: evaluation of a therapeutic exercise program.

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              The spectrum of arterial compression at the thoracic outlet.

              In the absence of ischemic events, arterial pathology at the thoracic outlet (TO) is rarely identified because findings of chronic arterial pathology may be masked by symptoms of neurogenic compression. This study describes the clinical presentations and significance of arterial compression at the TO. This was a retrospective analysis of the clinical records and imaging studies of 41 patients with objective findings of arterial compression at the TO. Sixteen were diagnosed from 1990 to 2003, during which 284 patients underwent surgery for TO decompression with selective arterial imaging; 25 were diagnosed from 2003 to 2009, and 62 underwent TO surgical decompressions. Subclavian artery stenosis, with or without poststenotic dilatation (PSD), was found in 26 patients (63%), subclavian artery aneurysms in 12 (29%), chronic subclavian occlusion in 1(2.4%), and axillary artery compression in 2 (5%). Chronic symptoms difficult to discern from neurogenic compression were present in 27 patients (66%; 24 had subclavian stenoses or PSD, or both, 1 had subclavian occlusion, and 2 had axillary artery compression); 13 (32%) presented with acute ischemia (11 had aneurysms and 2 had PSDs), and 1 asymptomatic patient had a subclavian aneurysm. Osteoarticular anomalies were found in 27 patients (66%), including 19 cervical ribs, 4 first rib anomalies, and 4 clavicular or first rib fractures, or both. Among 27 patients with subclavian aneurysms or PSD, 21 (78%) had a bone anomaly. Arterial pathology was deemed significant in 30 patients (73%) and mild or moderate in 11 (21%). Symptoms in 23 of these patients were compatible with neurogenic compression without clinical suspicion of arterial pathology, but 13 (56%) harbored a significant arterial anomaly. The incidence of arterial pathology secondary to compression at the TO may be underestimated, and in the absence of obvious ischemia, significant arterial pathology may not be suspected. Two-thirds of patients with arterial compression have associated bone anomalies. Therefore, routine arterial imaging seems advisable for patients evaluated for TO syndrome in the presence of a bone anomaly at the TO or an examination that shows an arterial abnormality. In the absence of these signs, however, arterial pathology may be overlooked in patients with symptoms suggestive of neurogenic compression. Further study is needed to elucidate the incidence, natural history, and clinical relevance of arterial compression and PSD at the TO. Copyright (c) 2010 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.
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                Author and article information

                Journal
                Rheumatol Adv Pract
                Rheumatol Adv Pract
                rheumap
                Rheumatology Advances in Practice
                Oxford University Press
                2514-1775
                2021
                01 December 2020
                01 December 2020
                : 5
                : 1
                : rkaa045
                Affiliations
                [1 ] Norwich Vascular Unit, Norfolk and Norwich University Hospital NHS Trust , Norwich, UK
                [2 ] Heart and Vascular Institute, Cleveland Clinic Abu-Dhabi , Abu-Dhabi, United Arab Emirates
                Author notes
                Correspondence to: Wissam Al-Jundi, Norfolk and Norwich University Hospital NHS Trust, Colney Lane, Norwich NR4 7UY, UK. E-mail: Wissam.Al-Jundi@ 123456nnuh.nhs.uk
                Article
                rkaa045
                10.1093/rap/rkaa045
                7884024
                0898c6e3-43ba-40fb-90f6-6f901e06957b
                © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 02 June 2020
                : 05 August 2020
                Page count
                Pages: 5
                Categories
                Concise Report
                AcademicSubjects/MED00010

                digital ischaemia,arterial thoracic outlet syndrome,cervical rib

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