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Axillary Vein Thrombosis Secondary to Congenital Stricture in a Left-Sided Superior Vena Cava
Axillary Vein Thrombosis Secondary to Congenital Stricture in a Left-Sided Superior Vena Cava L.P. Palerme, MD, Arthur M.T. Chan, MD, and York N.H. Hsiang, MD, Vancouver, Canada
We describe a case of axillary vein thrombosis secondary to unusual venous anatomy. A patient with a strictured left-sided superior vena cava and axillary vein thrombosis was successfully treated with thrombolysis. The underlying stricture was treated with angioplasty and stenting. (Ann Vasc Surg 2000;14:648-651.) DOI: 10.1007/s100169910115
INTRODUCTION Subclavian-axillary vein thrombosis (SAVT) is usually separated into primary and secondary forms.1 Primary SAVT is also referred to as Paget-Schroetter syndrome or “idiopathic” SAVT. Although idiopathic SAVT is usually related to physical activity or arm positioning, there usually is a component of anatomic compression at the thoracic outlet.2 Secondary SAVT implies thrombosis secondary to the abnormal presence of stasis, endothelial injury, and hypercoagulable states. Although by far the most common cause of secondary SAVT is the presence of a central venous catheter, numerous other contributory or independent risk factors exist. Local venous compression of the superior vena cava (SVC) by mediastinal tumors3 is a well-recognized cause of secondary SAVT. Other mechanical causes proximal to the thoracic outlet can lead to venous stasis and thromFrom the Division of Vascular Surgery, University of British Columbia, Vancouver, Canada. Presented at the Annual Meeting of the Canadian Society for Vascular Surgery, Quebec City, Quebec, Canada, September 1719, 1999. Correspondence to: Y.N.H. Hsiang, MD, Division of Vascular Surgery, Department of Surgery, University of British Columbia, Vancouver General Hospital, 910 West 10th Avenue, Room 3100 LSP, Vancouver, B.C., V5Z 4E3 Canada. 648
bosis. In this report, we describe a case of SAVT secondary to anomalous anatomy of the SVC.
CASE REPORT A 63-year-old male presented with a 1 day history of painful swelling of his left arm. There were no precipitating factors and on questioning, he had never had previous symptoms in his left arm. His past medical history was significant for right axillary vein thrombosis 9 months earlier, which was treated with thrombolysis. Completion venography at that time showed complete thrombus clearance and normal SVC anatomy. A stenosis of the right subclavian vein at the thoracic outlet was present, which was successfully treated by angioplasty. Hematologic workup for hypercoagulable states, prior to coumadin administration, showed normal levels of antithrombin III, proteins C and S, factor V Leidin, and homocysteine. The lupus anticoagulant assay was negative. He had completed a 6 month course of oral anticoagulant prior to his latest presentation. Duplex scanning of the left arm showed complete thrombosis of the axillary and subclavian veins, extending into the internal jugular vein. Plain thoracic outlet radiographs did not show cervical ribs. Computer tomography of the chest revealed the presence of a thrombosed left-sided SVC and did not show any intrathoracic pathology, such as mediastinal fibrosis or neoplasm (Fig. 1D). A nuclear lung scan, performed because of mild dyspnea, showed a low probability of pulmonary embolism. Repeat testing for hypercoagulable states was normal. Venography, performed via a left basilic vein ap-
Vol. 14, No. 6, 2000
Axillary vein thrombosis 649
Fig. 1. A Left upper extremity venogram showing in complete occlusion of the axillary and subclavian veins by thrombus (arrow). Note prominent collateral veins around the shoulder. B Following thrombolytic therapy, thrombus clearance has occurred, revealing an underlying left-sided vena cava. There is a long stenosis in the
superior segment of the left SVC (arrow). C The left SVC stenosis has been treated by angioplasty and stenting (arrow). The lumen is now widely patent. D Computer tomogram of the chest demonstrating a thrombosed leftsided SVC. The catheter used for thrombolysis is visible within the lumen (arrow).
proach, confirmed in complete occlusion of the axillary vein and showed several collateral vessels around the shoulder (Fig. 1A). An infusion catheter was advanced through the thrombus into the left brachiocephalic vein and infusion of urokinase was initiated using a pulsespray technique (Fig. 1D). Follow-up venography, performed after 68 hr of thrombolytic therapy, indicated nearly complete thrombus dissolution, revealing a leftsided SVC, with absence of communication with the right SVC. The left-sided SVC drained directly into the right atrium via the coronary sinus (Fig. 1B). A 4-cm-long stenosis was present in the superior aspect of the left SVC. Balloon angioplasty of the stenosis was only partially successful in dilating the stricture. For this reason, angio-
plasty and insertion of a 12 × 60 mm Wallstent were carried out, achieving a good result (Fig. 1C). The patient’s arm swelling diminished progressively following thrombolysis. He was subsequently placed on coumadin prior to discharge. He remains asymptomatic during early follow-up.
DISCUSSION Appearing during the fourth week of embryological development, the precardinal veins are a paired, symmetric bilateral structure, responsible for venous return from the cephalad segment of the em-
650 Palerme et al.
bryo.4 In time, the cephalad portions of the paired precardinal veins become the subclavian and internal jugular veins. By the eighth week of gestation, the left innominate vein develops, connecting the left and right precardinal systems. At this time, the caudal segment of the left precardinal vein undergoes atrophy, the result being that all venous drainage of both the right and left head and neck enters the heart via the right precardinal veins, which enlarge progressively to become the SVC. Failure of the caudal section of the left precardinal vein to regress during embryological development results in a double SVC system (presence of both a right and left SVC).5 A persistent left SVC is present in the general population at a reported incidence of 0.5%. This anomaly is present much more commonly in fetuses and neonates with other congenital cardiac malformations.6,7 Beyond associated cardiac malformations, the clinical implications of this anatomy have mostly concerned on catheter or pacemaker placement into the coronary sinus, a common site of insertion of the left SVC into the right atrium. In particular, the presence of a persistent left SVC draining into the coronary sinus complicates the administration of retrograde cardioplegia during cardiac surgery.8 A variety of anatomic abnormalities causing venous compression and possibly intimal damage to the vein at the thoracic outlet have been described in patients with primary SAVT.3 Most commonly, a narrowing at the costoclavicular space is identified as the precipitating lesion. Possible anomalies causing external compression include abnormal anterior scalene, subclavius and pectoralis minor muscles, abnormal first rib or clavicle, congenital bands, congenital webs, or malformed valves within the vein. These may be associated with identifiable hypercoagulable states. In this report, we describe a case of SAVT secondary to a stricture involving the left cava in a patient with a double caval system. The location of this stenosis is clearly intrathoracic and distant from the thoracic outlet (Fig. 1B). Imaging with CT scanning did not reveal any extrinsic cause, such as neoplasm or mediastinal fibrosis, for compression of the SVC within the chest. The patient did not have a history of precipitating factors such as radiotherapy or central vein cannulation to explain the presence of this stenosis. This idiopathic stenosis likely represents a congenital stricture in this anomalous persistent left SVC. Although it is possible that the stenosis may have been caused by organized thrombus, it would be unlikely for the clot to extend so far proximally without a clear anatomic cause. Treatment options for this problem include con-
Annals of Vascular Surgery
servative management with arm elevation and anticoagulation. This likely would not have resulted in a satisfactory result in this patient given the acuteness of his presentation and the severity of symptoms. Furthermore, patients treated in this fashion have been shown to have a poor functional outcome.2 Percutaneous balloon angioplasty has been found to be disappointing when used for subclavian vein stenosis associated with external compression at the thoracic outlet in patients with primary SAVT.9 Angioplasty of an upper extremity central vein stenosis appears to have a somewhat better short- to medium-term patency.10 Primary stenting does not appear to offer any benefit, compared to balloon angioplasty alone, and is associated with more expense.10 In the patient described in this report, stenting was required because of an unsatisfactory result from angioplasty alone. Subsequent long-term management in the event of recurrent thrombosis in this particular case would depend largely on the patient’s symptoms. If recurrence is minimally symptomatic with little or no functional limitations, conservative treatment may be the best choice. For more severe symptoms, repeat thrombolysis with angioplasty of any residual intrathoracic stenosis would be a reasonable option. In the event of complete occlusion of the left-sided SVC that was not cleared by thrombolysis, a surgical procedure with left-to-right axillary vein bypass may be an option. This lesion was successfully treated using interventional radiology techniques. For this reason, we consider angioplasty and stenting, following thrombolysis, to be the treatment of choice for this previously unreported cause of SAVT.
REFERENCES 1. Adams JT, McEvoy RK, Deweese JA. Primary deep venous thrombosis of the upper extremity. Arch Surg 1965;91:2942. 2. Hurlbert SN, Rutherford RB. Basic data underlying clinical decision making—primary subclavian-axillary vein thrombosis. Ann Vasc Surg 1995;9:217-223. 3. Mason BA. Axillary-subclavian vein thrombosis in patients with lung neoplasms. Cancer 1981;48:1886-1889. 4. O’Rahilly R, Mu¨ller F. Developmental Stages in Human Embryos, Including a Revision of Streeter’s “Horizons” and a Survey of the Carnegie Collection. Washington, DC: Carnegie Institution of Washington, 1987, Publication 637. 5. Nandy K, Blair CB Jr. Double superior venae cavae with completely paired azygous veins. Anat Rec 1965;151:1. 6. Steinberg I, Dubilier W, Lucas DS. Persistence of left superior vena cava. Dis Chest 1953;24:479-488.
Vol. 14, No. 6, 2000
7. Bunger PC, Neufeld DA, Moore JC, Carter GA. Persistent left superior vena cava and associated structural and functional considerations. Angiology 1981;32:601-608. 8. Hanson EW, Hannan RL, Baum VC. Pulmonary artery catheter in the coronary sinus: Implications of a persistent left superior vena cava for retrograde cardioplegia. J Cardiothorac Vasc Anesth 1998;12:448-449. 9. Lee MC, Grassi CJ, Belkin M, Mannick JA, Whittemore AD,
Axillary vein thrombosis 651
Donaldson MC. Early operative intervention after thrombolytic therapy for primary subclavian vein thrombosis: an effective treatment approach. J Vasc Surg 1998;27:11011108. 10. Kalman PG, Lindsay TF, Clarke K, Sniderman KW, Vanderburgh L. Management of upper extremity central venous obstruction using interventional radiology. Ann Vasc Surg 1998;12:202-206.
We describe a case of axillary vein thrombosis secondary to unusual venous anatomy. A patient with a strictured left-sided superior vena cava and axillary vein thrombosis was successfully treated with thrombolysis. The underlying stricture was treated with angioplasty and stenting. (Ann Vasc Surg 2000;14:648-651.) DOI: 10.1007/s100169910115
INTRODUCTION Subclavian-axillary vein thrombosis (SAVT) is usually separated into primary and secondary forms.1 Primary SAVT is also referred to as Paget-Schroetter syndrome or “idiopathic” SAVT. Although idiopathic SAVT is usually related to physical activity or arm positioning, there usually is a component of anatomic compression at the thoracic outlet.2 Secondary SAVT implies thrombosis secondary to the abnormal presence of stasis, endothelial injury, and hypercoagulable states. Although by far the most common cause of secondary SAVT is the presence of a central venous catheter, numerous other contributory or independent risk factors exist. Local venous compression of the superior vena cava (SVC) by mediastinal tumors3 is a well-recognized cause of secondary SAVT. Other mechanical causes proximal to the thoracic outlet can lead to venous stasis and thromFrom the Division of Vascular Surgery, University of British Columbia, Vancouver, Canada. Presented at the Annual Meeting of the Canadian Society for Vascular Surgery, Quebec City, Quebec, Canada, September 1719, 1999. Correspondence to: Y.N.H. Hsiang, MD, Division of Vascular Surgery, Department of Surgery, University of British Columbia, Vancouver General Hospital, 910 West 10th Avenue, Room 3100 LSP, Vancouver, B.C., V5Z 4E3 Canada. 648
bosis. In this report, we describe a case of SAVT secondary to anomalous anatomy of the SVC.
CASE REPORT A 63-year-old male presented with a 1 day history of painful swelling of his left arm. There were no precipitating factors and on questioning, he had never had previous symptoms in his left arm. His past medical history was significant for right axillary vein thrombosis 9 months earlier, which was treated with thrombolysis. Completion venography at that time showed complete thrombus clearance and normal SVC anatomy. A stenosis of the right subclavian vein at the thoracic outlet was present, which was successfully treated by angioplasty. Hematologic workup for hypercoagulable states, prior to coumadin administration, showed normal levels of antithrombin III, proteins C and S, factor V Leidin, and homocysteine. The lupus anticoagulant assay was negative. He had completed a 6 month course of oral anticoagulant prior to his latest presentation. Duplex scanning of the left arm showed complete thrombosis of the axillary and subclavian veins, extending into the internal jugular vein. Plain thoracic outlet radiographs did not show cervical ribs. Computer tomography of the chest revealed the presence of a thrombosed left-sided SVC and did not show any intrathoracic pathology, such as mediastinal fibrosis or neoplasm (Fig. 1D). A nuclear lung scan, performed because of mild dyspnea, showed a low probability of pulmonary embolism. Repeat testing for hypercoagulable states was normal. Venography, performed via a left basilic vein ap-
Vol. 14, No. 6, 2000
Axillary vein thrombosis 649
Fig. 1. A Left upper extremity venogram showing in complete occlusion of the axillary and subclavian veins by thrombus (arrow). Note prominent collateral veins around the shoulder. B Following thrombolytic therapy, thrombus clearance has occurred, revealing an underlying left-sided vena cava. There is a long stenosis in the
superior segment of the left SVC (arrow). C The left SVC stenosis has been treated by angioplasty and stenting (arrow). The lumen is now widely patent. D Computer tomogram of the chest demonstrating a thrombosed leftsided SVC. The catheter used for thrombolysis is visible within the lumen (arrow).
proach, confirmed in complete occlusion of the axillary vein and showed several collateral vessels around the shoulder (Fig. 1A). An infusion catheter was advanced through the thrombus into the left brachiocephalic vein and infusion of urokinase was initiated using a pulsespray technique (Fig. 1D). Follow-up venography, performed after 68 hr of thrombolytic therapy, indicated nearly complete thrombus dissolution, revealing a leftsided SVC, with absence of communication with the right SVC. The left-sided SVC drained directly into the right atrium via the coronary sinus (Fig. 1B). A 4-cm-long stenosis was present in the superior aspect of the left SVC. Balloon angioplasty of the stenosis was only partially successful in dilating the stricture. For this reason, angio-
plasty and insertion of a 12 × 60 mm Wallstent were carried out, achieving a good result (Fig. 1C). The patient’s arm swelling diminished progressively following thrombolysis. He was subsequently placed on coumadin prior to discharge. He remains asymptomatic during early follow-up.
DISCUSSION Appearing during the fourth week of embryological development, the precardinal veins are a paired, symmetric bilateral structure, responsible for venous return from the cephalad segment of the em-
650 Palerme et al.
bryo.4 In time, the cephalad portions of the paired precardinal veins become the subclavian and internal jugular veins. By the eighth week of gestation, the left innominate vein develops, connecting the left and right precardinal systems. At this time, the caudal segment of the left precardinal vein undergoes atrophy, the result being that all venous drainage of both the right and left head and neck enters the heart via the right precardinal veins, which enlarge progressively to become the SVC. Failure of the caudal section of the left precardinal vein to regress during embryological development results in a double SVC system (presence of both a right and left SVC).5 A persistent left SVC is present in the general population at a reported incidence of 0.5%. This anomaly is present much more commonly in fetuses and neonates with other congenital cardiac malformations.6,7 Beyond associated cardiac malformations, the clinical implications of this anatomy have mostly concerned on catheter or pacemaker placement into the coronary sinus, a common site of insertion of the left SVC into the right atrium. In particular, the presence of a persistent left SVC draining into the coronary sinus complicates the administration of retrograde cardioplegia during cardiac surgery.8 A variety of anatomic abnormalities causing venous compression and possibly intimal damage to the vein at the thoracic outlet have been described in patients with primary SAVT.3 Most commonly, a narrowing at the costoclavicular space is identified as the precipitating lesion. Possible anomalies causing external compression include abnormal anterior scalene, subclavius and pectoralis minor muscles, abnormal first rib or clavicle, congenital bands, congenital webs, or malformed valves within the vein. These may be associated with identifiable hypercoagulable states. In this report, we describe a case of SAVT secondary to a stricture involving the left cava in a patient with a double caval system. The location of this stenosis is clearly intrathoracic and distant from the thoracic outlet (Fig. 1B). Imaging with CT scanning did not reveal any extrinsic cause, such as neoplasm or mediastinal fibrosis, for compression of the SVC within the chest. The patient did not have a history of precipitating factors such as radiotherapy or central vein cannulation to explain the presence of this stenosis. This idiopathic stenosis likely represents a congenital stricture in this anomalous persistent left SVC. Although it is possible that the stenosis may have been caused by organized thrombus, it would be unlikely for the clot to extend so far proximally without a clear anatomic cause. Treatment options for this problem include con-
Annals of Vascular Surgery
servative management with arm elevation and anticoagulation. This likely would not have resulted in a satisfactory result in this patient given the acuteness of his presentation and the severity of symptoms. Furthermore, patients treated in this fashion have been shown to have a poor functional outcome.2 Percutaneous balloon angioplasty has been found to be disappointing when used for subclavian vein stenosis associated with external compression at the thoracic outlet in patients with primary SAVT.9 Angioplasty of an upper extremity central vein stenosis appears to have a somewhat better short- to medium-term patency.10 Primary stenting does not appear to offer any benefit, compared to balloon angioplasty alone, and is associated with more expense.10 In the patient described in this report, stenting was required because of an unsatisfactory result from angioplasty alone. Subsequent long-term management in the event of recurrent thrombosis in this particular case would depend largely on the patient’s symptoms. If recurrence is minimally symptomatic with little or no functional limitations, conservative treatment may be the best choice. For more severe symptoms, repeat thrombolysis with angioplasty of any residual intrathoracic stenosis would be a reasonable option. In the event of complete occlusion of the left-sided SVC that was not cleared by thrombolysis, a surgical procedure with left-to-right axillary vein bypass may be an option. This lesion was successfully treated using interventional radiology techniques. For this reason, we consider angioplasty and stenting, following thrombolysis, to be the treatment of choice for this previously unreported cause of SAVT.
REFERENCES 1. Adams JT, McEvoy RK, Deweese JA. Primary deep venous thrombosis of the upper extremity. Arch Surg 1965;91:2942. 2. Hurlbert SN, Rutherford RB. Basic data underlying clinical decision making—primary subclavian-axillary vein thrombosis. Ann Vasc Surg 1995;9:217-223. 3. Mason BA. Axillary-subclavian vein thrombosis in patients with lung neoplasms. Cancer 1981;48:1886-1889. 4. O’Rahilly R, Mu¨ller F. Developmental Stages in Human Embryos, Including a Revision of Streeter’s “Horizons” and a Survey of the Carnegie Collection. Washington, DC: Carnegie Institution of Washington, 1987, Publication 637. 5. Nandy K, Blair CB Jr. Double superior venae cavae with completely paired azygous veins. Anat Rec 1965;151:1. 6. Steinberg I, Dubilier W, Lucas DS. Persistence of left superior vena cava. Dis Chest 1953;24:479-488.
Vol. 14, No. 6, 2000
7. Bunger PC, Neufeld DA, Moore JC, Carter GA. Persistent left superior vena cava and associated structural and functional considerations. Angiology 1981;32:601-608. 8. Hanson EW, Hannan RL, Baum VC. Pulmonary artery catheter in the coronary sinus: Implications of a persistent left superior vena cava for retrograde cardioplegia. J Cardiothorac Vasc Anesth 1998;12:448-449. 9. Lee MC, Grassi CJ, Belkin M, Mannick JA, Whittemore AD,
Axillary vein thrombosis 651
Donaldson MC. Early operative intervention after thrombolytic therapy for primary subclavian vein thrombosis: an effective treatment approach. J Vasc Surg 1998;27:11011108. 10. Kalman PG, Lindsay TF, Clarke K, Sniderman KW, Vanderburgh L. Management of upper extremity central venous obstruction using interventional radiology. Ann Vasc Surg 1998;12:202-206.