Ann Thorac Surg 2009;88:295-297. doi:10.1016/j.athoracsur.2008.12.039
© 2009 The Society of Thoracic Surgeons
Case Reports
Limb-Threatening Ischemia Secondary to a Congenital Acromioclavicular Remnant
Jonathan M. Enlow, DO*,
Walter E. Mcgregor, MD
Department of Thoracic Surgery, Riverside Methodist Hospital, Columbus, Ohio
Accepted for publication December 1, 2008.
* Address correspondence to Dr Enlow, Department of Surgery, Riverside Methodist Hospital, Columbus, Ohio 43214 (Email: je633701{at}ohio.edu).
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Abstract
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Upper extremity vascular compromise from thoracic outlet syndrome is rare and is usually the result of a "cervical rib," anterior scalene muscle abnormality, or clavicular trauma. We report a case of acute axillary artery thrombosis secondary to a congenital acromioclavicular remnant in a 40-year-old woman.
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Introduction
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Thoracic outlet syndrome is most commonly associated with neurogenic or venous impingement, and more rarely produces arterial compromise. Traumatic and congenital clavicular abnormalities both have been associated with acute axillary artery thrombosis and subsequent upper limb ischemia. These conditions traditionally have been treated with emergency surgical decompression and interposition grafting. As experience with endovascular operations continues, treatment algorithms now include acute limb salvage with endovascular therapies, followed by surgical decompression.
A 40-year-old woman with a 50 pack-year smoking history, presented to another facility with right hand pain and cyanosis of the fourth and fifth fingers She underwent right ulnar artery thrombectomy with vein graft reconstruction. Her postoperative course was uneventful. She was discharged without anticoagulation or antiplatelet therapy, with instructions to stop smoking.
The patient presented to our facility 7 weeks later with a painful, cyanotic, and pulseless right upper extremity. The patient was noted to have congenital absence of the right clavicle. Angiography demonstrated a thrombus in the axillary artery. Abduction of the right shoulder failed to show any angiographic abnormality to suggest thoracic outlet compression. An arteriogram of the left upper extremity did not demonstrate any signs of an underlying vasculitis.
Catheter-directed thrombolysis was given for the next 12 hours, with postlytic angiography still showing a 2- to 3-mm wall-adherent filling defect in the right axillary artery. A heparin drip was continued, and her symptoms resolved during a 3-day period.
Repeat angiography demonstrated thrombus resolution but found a filling defect in the right axillary artery producing 50% focal stenosis. The defect was of unclear cause and was presumed to be intraluminal. In the interventional radiology suite, a bare metal stent was placed across the stenosis, with complete radiographic resolution of the filling defect. Follow-up computed tomography angiography identified a distal clavicular remnant located approximately 6 mm anterior to the right subclavian artery stent causing potential compression (Fig 1). The result of a hypercoagulability/vasculitis workup was negative.
Thoracic surgery was consulted, and resection of the distal acromioclavicular remnant was done to decrease the risk of repeat thromboembolism. A transverse incision was made below the clavicle from 3 cm lateral to the sternum to the deltopectoral groove. The pectoralis muscle fibers were split, the underlying clavipectoral fascia was incised, and proximal and distal vascular control was achieved. The subclavian/axillary artery was attached to the clavicular remnant by an encasement of fibrous, chronic-appearing scar tissue. The remnant was removed (3-cm specimen), and the stented portion of the artery was mobilized. The patient was discharged on postoperative day 4, with palpable pulses, on warfarin and clopidogrel therapy.
The patient discontinued her medications approximately 1year postoperatively. She returned to our facility 23 months later with limb-threatening ischemia due to stent thrombosis. Catheter-directed lysis was successful in achieving limb salvage. Because of the stent thrombosis, the patient underwent anticoagulation education and was discharged home on lifelong warfarin and clopidogrel.
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Comment
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Acute upper limb ischemia can be a result of many different disease processes, including embolic shower, thoracic outlet syndrome (and other local thrombosis), arterial aneurysm, and trauma. In a series of 97 patients with acute upper limb ischemia, Pentti and colleagues [1] reported embolus originating from the heart was responsible for acute upper extremity ischemia in 73%, local thrombosis was the cause in 12%, and a definite source of emboli could not be found in 15%. The right limb was more often affected (59 vs 38, P < .05). Although rare, patients with an underlying hypercoagulable state have been reported to present with acute upper limb arterial thrombosis [2].
Thoracic outlet syndrome (TOS) may initially present with upper extremity ischemia, although much less frequently than venous/neural involvement [3]. In a series of 30 patients with TOS with vascular compromise, a cervical rib was responsible in 53%, soft tissue anomalies in 40%, and scar tissue after clavicle fracture was the cause in 7%. Subclavian arterial aneurysms developed in 10 patients as a result of the extrinsic forces [4]. Surgical treatment is aimed at decompression of affected vasculature, by cervical/first rib resection or soft tissue division, or both, with distal embolectomy. Aneurysmal disease should be resected with placement of an interposition vein graft. Occasionally, subclavian–axillary or axillary–brachial bypass is necessary to provide upper extremity perfusion.
Currently, little data are available to guide the use of endovascular therapy in subclavian/axillary artery stenosis and aneurysmal disease. Malliet and colleagues [5] reported 3 subclavian artery aneurysms, all as a result of TOS, that were initially treated with a stent and subsequently received a first rib resection. At 37.3 months, all patients were free of symptoms and without late complications [5]. In cases of venous TOS, combination first rib resection with intraoperative endovascular balloon therapy may reduce the incidence of rethrombosis or the need for open venous repair or venous stent placement [6].
Our patient's case is very rare. Only 2 patients with documented congenital clavicular abnormalities causing arterial compromise have been reported, and both had cleidocranial dysostosis [7, 8]. Our patient failed to exhibit the skull-based features or dental abnormalities of this rare syndrome of delayed or absent ossification. This patient's treatment algorithm would have proceeded differently if the extrinsic compressive force had been identified before stent placement by interventional radiology. Hopefully, surgical consultation would have been requested earlier, and coordination of a decompressive procedure combined with intraoperative endovascular evaluation/therapy would have progressed in lieu of the initial stent placement.
We advocate attempting initial limb salvage by catheter-directed thrombolysis, with concurrent imaging studies to rule out thoracic outlet abnormalities in all patients with acute upper limb ischemia and no clear etiology. Further studies are needed to determine the best multidisciplinary management algorithm, as well as the timing of surgical decompression to treat TOS and variants with arterial compromise.
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References
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- Pentti J, Salenius JP, Kuukasjarvi P, Tarkka M. Outcome of surgical treatment in acute upper limb ischaemia Ann Chir Gynaecol 1995;84:25-28.[Medline]
- Dorweiler B, Neufang A, Kasper-Koenig W, Schinzel H, Schmiedt W, Oelert H. Arterial embolism to the upper extremity in a patient with factor V Leiden mutation (APC resistance)- a case report and review of the literature Angiology 2003;54:125-130.[Medline]
- Urschel JD, Hameed SM, Grewal RP. Neurogenic thoracic outlet syndromes Postgrad Med J 1994;70:785-789.[Free Full Text]
- Deguara J, Ali T, Modarai B, Burnand KG. Upper limb ischemia: 20 years experience from a single center Vascular 2005;13:84-91.[Abstract/Free Full Text]
- Malliet C, Fourneau I, Daenens K, Maleux G, Nevelsteen A. Endovascular stent-graft and first rib resection for thoracic outlet syndrome complicated by an aneurysm of the subclavian artery Acta Chir Belg 2005;105:194-197.[Medline]
- Scneider D, Dimuzio P, Martin N, et al. Combination treatment of venous thoracic outlet syndrome: Open surgical decompression and intraoperative angioplasty J Vasc Surg 2004;40:599-603.[Medline]
- Qureshi K, Lees T. Acute upper limb ischemia resulting from cleidocranial dysostosis J Vasc Surg 1997;26:888-890.[Medline]
- Short DW. A case of craniocleidal dysostosis presenting with vascular complications Br J Surg 1979;66:596-598.[Medline]
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Abstract
Introduction
