link
Bookmarks
Upper Extremity: Vasculitis and Revascularization
T. Gregory Walker, MD, FSIR; Brandt C. Wible, MD; Zubin Irani, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe

KEY FACTS

  • Terminology

    • Procedure

      • Outcomes

        TERMINOLOGY

        • Definitions

          • Buerger disease (thromboangiitis obliterans): Nonatherosclerotic inflammatory disease affecting small to medium-sized arteries & veins of extremities
            • Strongly associated with heavy tobacco use
            • Inflammatory cellular debris occludes vessel lumen
              • Extensive "corkscrew" collaterals around occlusions
                • Collaterals develop in vasa vasorum of occluded arteries; causes corkscrew appearance
            • Ischemic ulcers in toes & fingers later in disease course; reported in 40-50% of patients
            • Poor prognosis with progression of disease unless patients stop smoking
              • Common to progress to amputation
          • Frostbite: Localized damage to skin & other tissues caused by extreme cold; often involves extremities
            • Pathophysiology of tissue injury in frostbite
              • Initial tissue freezing & crystal formation
                • Improves with tissue rewarming
              • Subsequent tissue inflammation & coagulation
                • Causes microvascular thrombosis/cell death
            • Injury spectrum ranges from irreversible cellular destruction to reversible changes after rewarming
              • Changes include tissue edema, circulatory stasis, progressive thrombosis causing tissue necrosis
              • Course similar to ischemia/reperfusion injury
                • Viable-appearing tissue may later necrose due to microcirculatory collapse after reperfusion
            • Conventional frostbite treatment
              • Patient resuscitation; IV hydration
              • Tissue rewarming in tepid water
              • Heparinization, intraarterial vasodilators
              • Monitoring of viable vs. nonviable tissue
                • Debridement & amputation as necessary
              • Generally poor overall clinical outcomes
            • Transcatheter thrombolysis treatment of frostbite
              • Intraarterial catheter-directed thrombolysis using tissue plasminogen activator (tPA) infusion
                • Reverses microvascular thrombosis before irreversible ischemia & necrosis occur
          • Hypothenar hammer syndrome: Disorder caused by repetitive trauma to distal ulnar arterial segment coursing adjacent to hook of hamate
            • Ulnar artery has superficial course in Guyon canal
              • Repetitive pounding or vibrational activity can cause intimal or diffuse arterial injury
              • Injury may progress to aneurysm, arterial occlusion
                • May result in distal embolization with occlusion of palmar &/or digital arteries
                • Digital arterial occlusions occur in ulnar artery distribution (middle, ring, & small fingers)
              • May be diagnosed at stage in which irreversible consequences have already occurred
                • Often diagnosed too late for recanalization attempt to be viable therapeutic option
                • Digital gangrene requiring amputation possible
          • Raynaud phenomenon: Disorder characterized by episodic digital artery vasospasm/vasoconstriction
            • Primary Raynaud phenomenon (Raynaud disease): Exaggerated smooth muscle cell vasoconstriction in otherwise normal digital artery; usually cold induced
              • No identifiable underlying arterial abnormality
              • Environmental trigger causes primary vasospasm
            • Secondary Raynaud phenomenon (Raynaud syndrome): Vasospastic digital ischemia associated with underlying arterial pathology
              • Most commonly associated with cutaneous & connective tissue disorders
              • Triggered by cold, nicotine, caffeine, & stress
          • Thoracic outlet syndrome: Due to extrinsic compression of neurovascular structures exiting/entering thorax
            • May be predominantly neurogenic, venous, or arterial
              • Symptoms from arterial compression in < 5%
                • Pain, coolness, pallor, diminished pulses
                • Distal thromboembolic symptoms (e.g., ischemia, cyanosis) most common presentation
              • Clinical symptoms involve compression of brachial plexus & related nerves in > 90%
                • Pain, numbness, tingling, hand weakness
          • Vasculitis & other arteriopathies
            • Fibromuscular dysplasia (FMD): Noninflammatory vasculopathy characterized by sequential arterial wall thickening & thinning; media most often involved
              • Although upper extremity FMD very rare, most commonly involves brachial artery when present
              • May have symptoms of upper extremity ischemia
                • Coolness, decreased pulses, pain, distal emboli
            • Giant cell arteritis (GCA): Vasculitis affecting medium- to large-caliber arteries; similar to Takayasu arteritis & may represent single disease spectrum
              • Age at onset often used to classify patients
                • GCA affects older population (> 50 years)
              • Traditionally considered to affect cranial arteries
                • Classically involves temporal arteries; may also involve cervical carotid arteries
              • 20-30% of patients with GCA have involvement of aorta & primary branches
                • May cause long stenoses of mid/distal subclavian arteries, extending into axillary arteries; seen more frequently in GCA than Takayasu
              • Diagnosis confirmed by arterial biopsy
                • Typically, biopsy temporal artery; easily accessible, usually involved when disease present
                • Mononuclear cell infiltration/granulomatous inflammation with multinucleated giant cells
            • Takayasu arteritis: Large-vessel granulomatous inflammatory vasculitis
              • Affects people younger than 40 years
                • Often occurs between 15-30 years
              • Classically characterized by 3 stages
                • Active inflammation; constitutional symptoms
                • Vascular inflammation; vessel pain/tenderness
                • Vascular fibrosis or degeneration causing ischemia or aneurysms
              • Involves aorta &/or primary branches, pulmonary arteries
            • Radiation arteritis: Permanent arterial damage caused by ionizing radiation; endothelial cells extremely vulnerable; later involves media
              • Clinical/imaging manifestations generally follow 3 patterns; related to time interval since irradiation
                • Early lesions (within 5 years): Mural thrombus
                • Intermediate lesions (within 10 years): Mural fibrosis, occlusion, & absence of collaterals
                • Late lesions (mean interval: 26 years): Periarterial fibrosis & "accelerated atherosclerosis"
              • Presumptive diagnosis based on clinical history & angiographic appearance of lesions
                • History of radiation in area of arterial lesion
                • Lesions often occur in atypical locations; adjacent arterial beds largely spared
                • Carotid, subclavian, axillary, & iliac arteries most commonly affected vascular territories
              • Revascularization procedures often difficult
                • Postradiation scarring/fibrosis may significantly impact open surgical revascularization
                • Lesions at thoracic outlet challenging for durable outcomes with endovascular intervention
        • Pertinent Vascular Anatomy

          • Axillary artery: Originates at lateral margin of 1st rib; has 3 segments based on relationship to superficially located pectoralis minor muscle
            • 1st segment: Portion medial to pectoralis minor
              • Superior thoracic artery: Supplies 1st & 2nd intercostal spaces, upper serratus anterior
            • 2nd segment: Portion behind pectoralis minor
              • Thoracoacromial artery: Supplies pectoral & deltoid muscles; extends to clavicle/acromion
              • Lateral thoracic (external mammary) artery: Supplies serratus anterior & pectoralis major muscles, lateral structures of thorax/breast
            • 3rd segment: Portion lateral to pectoralis minor
              • Subscapular artery: Largest branch of axillary artery; supplies subscapularis muscle
              • Anterior circumflex humeral artery: Supplies humeral head, shoulder joint, upper biceps muscles
              • Posterior circumflex humeral artery: Supplies teres minor & deltoid muscles
          • Brachial artery: Continuation of axillary artery beyond lower margin of teres minor muscle; divides into radial & ulnar arteries at antecubital fossa
            • Radial artery: Main artery of lateral forearm
              • Landmark for division between anterior & posterior compartments of forearm
              • Yields superficial palmar branch, which joins superficial palmar arch
              • Terminates in deep palmar arch, which joins with deep branch of ulnar artery
                • Deep palmar arch located more proximally
              • Supplies thumb (princeps pollicis artery), 2nd digit
            • Ulnar artery: Main artery of medial forearm
              • Typically larger than radial artery
              • Yields interosseous artery below radial tuberosity
                • Supplies deep anterior & posterior forearm
              • Distally divides into deep & superficial branches
              • Terminates in superficial palmar arch, which joins with superficial branch of radial artery
                • Superficial palmar arch located more distally
              • Supplies 4th & 5th digits; variable supply to 3rd

        PREPROCEDURE

        • Indications

          • Contraindications

            • Preprocedure Imaging

              • Getting Started

                PROCEDURE

                • Patient Position/Location

                  • Procedure Steps

                    • Alternative Procedures/Therapies

                      POST PROCEDURE

                      • Things to Do

                        • Things to Avoid

                          OUTCOMES

                          • Problems

                            • Complications

                              • Expected Outcomes

                                Selected References

                                1. Nguyen LL et al: Evaluation and management of arterial thoracic outlet syndrome. Thorac Surg Clin. 31(1):45-54, 2021
                                2. Lopez D et al: Use of ultrasound in the diagnosis and management of the vasculitides. Curr Rheumatol Rep. 22(7):31, 2020
                                3. Ciofalo A et al: Giant cell arteritis (GCA): pathogenesis, clinical aspects and treatment approaches. Curr Rheumatol Rev. 15(4):259-68, 2019
                                4. Ghoneim BM et al: Management of Buerger's disease in endovascular era. Int J Angiol. 28(3):173-81, 2019
                                5. Kim ESH et al: Takayasu arteritis: challenges in diagnosis and management. Heart. 104(7):558-65, 2018
                                6. Tacoy G: Management of Takayasu arteritis. Future Cardiol. 14(2):105-8, 2018
                                7. Handford C et al: Frostbite. Emerg Med Clin North Am. 35(2):281-99, 2017
                                8. Nguyen N et al: Presentation, clinical features, and results of intervention in upper extremity fibromuscular dysplasia. J Vasc Surg. 66(2):554-63, 2017
                                9. Millet JD et al: Frostbite: spectrum of imaging findings and guidelines for management. Radiographics. 36(7):2154-69, 2016
                                10. Iannuzzi NP et al: Acute arterial thrombosis of the hand. J Hand Surg Am. 40(10):2099-106, 2015
                                11. Liew NC et al: Pathogenesis and management of Buerger's disease. Int J Low Extrem Wounds. 14(3):231-5, 2015
                                12. Schröttle A et al: Hypothenar hammer syndrome. Vasa. 44(3):179-85, 2015
                                13. Wong VW et al: Interpretation of upper extremity arteriography: vascular anatomy and pathology [corrected]. Hand Clin. 31(1):121-34, 2015
                                14. Borchers AT et al: Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev. 11(6-7):A544-54, 2012
                                15. Grayson PC et al: Distribution of arterial lesions in Takayasu's arteritis and giant cell arteritis. Ann Rheum Dis. 71(8):1329-34, 2012
                                16. Kim YW et al: Surgical bypass vs endovascular treatment for patients with supra-aortic arterial occlusive disease due to Takayasu arteritis. J Vasc Surg. 55(3):693-700, 2012
                                17. Saadoun D et al: Retrospective analysis of surgery versus endovascular intervention in Takayasu arteritis: a multicenter experience. Circulation. 125(6):813-9, 2012
                                18. Modrall JG et al: Early and late presentations of radiation arteritis. Semin Vasc Surg. 16(3):209-14, 2003
                                19. Guerra A et al: Arterial percutaneous angioplasty in upper limbs with vascular access devices for haemodialysis. Nephrol Dial Transplant. 17(5):843-51, 2002
                                20. Yeager RA et al: Relationship of hemodialysis access to finger gangrene in patients with end-stage renal disease. J Vasc Surg. 36(2):245-9; discussion 249, 2002
                                21. Schmidt FE et al: Severe upper limb ischemia. Arch Surg. 115(10):1188-91, 1980
                                Related Anatomy
                                Loading...
                                Related Differential Diagnoses
                                Loading...
                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Procedure

                                    • Outcomes

                                      TERMINOLOGY

                                      • Definitions

                                        • Buerger disease (thromboangiitis obliterans): Nonatherosclerotic inflammatory disease affecting small to medium-sized arteries & veins of extremities
                                          • Strongly associated with heavy tobacco use
                                          • Inflammatory cellular debris occludes vessel lumen
                                            • Extensive "corkscrew" collaterals around occlusions
                                              • Collaterals develop in vasa vasorum of occluded arteries; causes corkscrew appearance
                                          • Ischemic ulcers in toes & fingers later in disease course; reported in 40-50% of patients
                                          • Poor prognosis with progression of disease unless patients stop smoking
                                            • Common to progress to amputation
                                        • Frostbite: Localized damage to skin & other tissues caused by extreme cold; often involves extremities
                                          • Pathophysiology of tissue injury in frostbite
                                            • Initial tissue freezing & crystal formation
                                              • Improves with tissue rewarming
                                            • Subsequent tissue inflammation & coagulation
                                              • Causes microvascular thrombosis/cell death
                                          • Injury spectrum ranges from irreversible cellular destruction to reversible changes after rewarming
                                            • Changes include tissue edema, circulatory stasis, progressive thrombosis causing tissue necrosis
                                            • Course similar to ischemia/reperfusion injury
                                              • Viable-appearing tissue may later necrose due to microcirculatory collapse after reperfusion
                                          • Conventional frostbite treatment
                                            • Patient resuscitation; IV hydration
                                            • Tissue rewarming in tepid water
                                            • Heparinization, intraarterial vasodilators
                                            • Monitoring of viable vs. nonviable tissue
                                              • Debridement & amputation as necessary
                                            • Generally poor overall clinical outcomes
                                          • Transcatheter thrombolysis treatment of frostbite
                                            • Intraarterial catheter-directed thrombolysis using tissue plasminogen activator (tPA) infusion
                                              • Reverses microvascular thrombosis before irreversible ischemia & necrosis occur
                                        • Hypothenar hammer syndrome: Disorder caused by repetitive trauma to distal ulnar arterial segment coursing adjacent to hook of hamate
                                          • Ulnar artery has superficial course in Guyon canal
                                            • Repetitive pounding or vibrational activity can cause intimal or diffuse arterial injury
                                            • Injury may progress to aneurysm, arterial occlusion
                                              • May result in distal embolization with occlusion of palmar &/or digital arteries
                                              • Digital arterial occlusions occur in ulnar artery distribution (middle, ring, & small fingers)
                                            • May be diagnosed at stage in which irreversible consequences have already occurred
                                              • Often diagnosed too late for recanalization attempt to be viable therapeutic option
                                              • Digital gangrene requiring amputation possible
                                        • Raynaud phenomenon: Disorder characterized by episodic digital artery vasospasm/vasoconstriction
                                          • Primary Raynaud phenomenon (Raynaud disease): Exaggerated smooth muscle cell vasoconstriction in otherwise normal digital artery; usually cold induced
                                            • No identifiable underlying arterial abnormality
                                            • Environmental trigger causes primary vasospasm
                                          • Secondary Raynaud phenomenon (Raynaud syndrome): Vasospastic digital ischemia associated with underlying arterial pathology
                                            • Most commonly associated with cutaneous & connective tissue disorders
                                            • Triggered by cold, nicotine, caffeine, & stress
                                        • Thoracic outlet syndrome: Due to extrinsic compression of neurovascular structures exiting/entering thorax
                                          • May be predominantly neurogenic, venous, or arterial
                                            • Symptoms from arterial compression in < 5%
                                              • Pain, coolness, pallor, diminished pulses
                                              • Distal thromboembolic symptoms (e.g., ischemia, cyanosis) most common presentation
                                            • Clinical symptoms involve compression of brachial plexus & related nerves in > 90%
                                              • Pain, numbness, tingling, hand weakness
                                        • Vasculitis & other arteriopathies
                                          • Fibromuscular dysplasia (FMD): Noninflammatory vasculopathy characterized by sequential arterial wall thickening & thinning; media most often involved
                                            • Although upper extremity FMD very rare, most commonly involves brachial artery when present
                                            • May have symptoms of upper extremity ischemia
                                              • Coolness, decreased pulses, pain, distal emboli
                                          • Giant cell arteritis (GCA): Vasculitis affecting medium- to large-caliber arteries; similar to Takayasu arteritis & may represent single disease spectrum
                                            • Age at onset often used to classify patients
                                              • GCA affects older population (> 50 years)
                                            • Traditionally considered to affect cranial arteries
                                              • Classically involves temporal arteries; may also involve cervical carotid arteries
                                            • 20-30% of patients with GCA have involvement of aorta & primary branches
                                              • May cause long stenoses of mid/distal subclavian arteries, extending into axillary arteries; seen more frequently in GCA than Takayasu
                                            • Diagnosis confirmed by arterial biopsy
                                              • Typically, biopsy temporal artery; easily accessible, usually involved when disease present
                                              • Mononuclear cell infiltration/granulomatous inflammation with multinucleated giant cells
                                          • Takayasu arteritis: Large-vessel granulomatous inflammatory vasculitis
                                            • Affects people younger than 40 years
                                              • Often occurs between 15-30 years
                                            • Classically characterized by 3 stages
                                              • Active inflammation; constitutional symptoms
                                              • Vascular inflammation; vessel pain/tenderness
                                              • Vascular fibrosis or degeneration causing ischemia or aneurysms
                                            • Involves aorta &/or primary branches, pulmonary arteries
                                          • Radiation arteritis: Permanent arterial damage caused by ionizing radiation; endothelial cells extremely vulnerable; later involves media
                                            • Clinical/imaging manifestations generally follow 3 patterns; related to time interval since irradiation
                                              • Early lesions (within 5 years): Mural thrombus
                                              • Intermediate lesions (within 10 years): Mural fibrosis, occlusion, & absence of collaterals
                                              • Late lesions (mean interval: 26 years): Periarterial fibrosis & "accelerated atherosclerosis"
                                            • Presumptive diagnosis based on clinical history & angiographic appearance of lesions
                                              • History of radiation in area of arterial lesion
                                              • Lesions often occur in atypical locations; adjacent arterial beds largely spared
                                              • Carotid, subclavian, axillary, & iliac arteries most commonly affected vascular territories
                                            • Revascularization procedures often difficult
                                              • Postradiation scarring/fibrosis may significantly impact open surgical revascularization
                                              • Lesions at thoracic outlet challenging for durable outcomes with endovascular intervention
                                      • Pertinent Vascular Anatomy

                                        • Axillary artery: Originates at lateral margin of 1st rib; has 3 segments based on relationship to superficially located pectoralis minor muscle
                                          • 1st segment: Portion medial to pectoralis minor
                                            • Superior thoracic artery: Supplies 1st & 2nd intercostal spaces, upper serratus anterior
                                          • 2nd segment: Portion behind pectoralis minor
                                            • Thoracoacromial artery: Supplies pectoral & deltoid muscles; extends to clavicle/acromion
                                            • Lateral thoracic (external mammary) artery: Supplies serratus anterior & pectoralis major muscles, lateral structures of thorax/breast
                                          • 3rd segment: Portion lateral to pectoralis minor
                                            • Subscapular artery: Largest branch of axillary artery; supplies subscapularis muscle
                                            • Anterior circumflex humeral artery: Supplies humeral head, shoulder joint, upper biceps muscles
                                            • Posterior circumflex humeral artery: Supplies teres minor & deltoid muscles
                                        • Brachial artery: Continuation of axillary artery beyond lower margin of teres minor muscle; divides into radial & ulnar arteries at antecubital fossa
                                          • Radial artery: Main artery of lateral forearm
                                            • Landmark for division between anterior & posterior compartments of forearm
                                            • Yields superficial palmar branch, which joins superficial palmar arch
                                            • Terminates in deep palmar arch, which joins with deep branch of ulnar artery
                                              • Deep palmar arch located more proximally
                                            • Supplies thumb (princeps pollicis artery), 2nd digit
                                          • Ulnar artery: Main artery of medial forearm
                                            • Typically larger than radial artery
                                            • Yields interosseous artery below radial tuberosity
                                              • Supplies deep anterior & posterior forearm
                                            • Distally divides into deep & superficial branches
                                            • Terminates in superficial palmar arch, which joins with superficial branch of radial artery
                                              • Superficial palmar arch located more distally
                                            • Supplies 4th & 5th digits; variable supply to 3rd

                                      PREPROCEDURE

                                      • Indications

                                        • Contraindications

                                          • Preprocedure Imaging

                                            • Getting Started

                                              PROCEDURE

                                              • Patient Position/Location

                                                • Procedure Steps

                                                  • Alternative Procedures/Therapies

                                                    POST PROCEDURE

                                                    • Things to Do

                                                      • Things to Avoid

                                                        OUTCOMES

                                                        • Problems

                                                          • Complications

                                                            • Expected Outcomes

                                                              Selected References

                                                              1. Nguyen LL et al: Evaluation and management of arterial thoracic outlet syndrome. Thorac Surg Clin. 31(1):45-54, 2021
                                                              2. Lopez D et al: Use of ultrasound in the diagnosis and management of the vasculitides. Curr Rheumatol Rep. 22(7):31, 2020
                                                              3. Ciofalo A et al: Giant cell arteritis (GCA): pathogenesis, clinical aspects and treatment approaches. Curr Rheumatol Rev. 15(4):259-68, 2019
                                                              4. Ghoneim BM et al: Management of Buerger's disease in endovascular era. Int J Angiol. 28(3):173-81, 2019
                                                              5. Kim ESH et al: Takayasu arteritis: challenges in diagnosis and management. Heart. 104(7):558-65, 2018
                                                              6. Tacoy G: Management of Takayasu arteritis. Future Cardiol. 14(2):105-8, 2018
                                                              7. Handford C et al: Frostbite. Emerg Med Clin North Am. 35(2):281-99, 2017
                                                              8. Nguyen N et al: Presentation, clinical features, and results of intervention in upper extremity fibromuscular dysplasia. J Vasc Surg. 66(2):554-63, 2017
                                                              9. Millet JD et al: Frostbite: spectrum of imaging findings and guidelines for management. Radiographics. 36(7):2154-69, 2016
                                                              10. Iannuzzi NP et al: Acute arterial thrombosis of the hand. J Hand Surg Am. 40(10):2099-106, 2015
                                                              11. Liew NC et al: Pathogenesis and management of Buerger's disease. Int J Low Extrem Wounds. 14(3):231-5, 2015
                                                              12. Schröttle A et al: Hypothenar hammer syndrome. Vasa. 44(3):179-85, 2015
                                                              13. Wong VW et al: Interpretation of upper extremity arteriography: vascular anatomy and pathology [corrected]. Hand Clin. 31(1):121-34, 2015
                                                              14. Borchers AT et al: Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev. 11(6-7):A544-54, 2012
                                                              15. Grayson PC et al: Distribution of arterial lesions in Takayasu's arteritis and giant cell arteritis. Ann Rheum Dis. 71(8):1329-34, 2012
                                                              16. Kim YW et al: Surgical bypass vs endovascular treatment for patients with supra-aortic arterial occlusive disease due to Takayasu arteritis. J Vasc Surg. 55(3):693-700, 2012
                                                              17. Saadoun D et al: Retrospective analysis of surgery versus endovascular intervention in Takayasu arteritis: a multicenter experience. Circulation. 125(6):813-9, 2012
                                                              18. Modrall JG et al: Early and late presentations of radiation arteritis. Semin Vasc Surg. 16(3):209-14, 2003
                                                              19. Guerra A et al: Arterial percutaneous angioplasty in upper limbs with vascular access devices for haemodialysis. Nephrol Dial Transplant. 17(5):843-51, 2002
                                                              20. Yeager RA et al: Relationship of hemodialysis access to finger gangrene in patients with end-stage renal disease. J Vasc Surg. 36(2):245-9; discussion 249, 2002
                                                              21. Schmidt FE et al: Severe upper limb ischemia. Arch Surg. 115(10):1188-91, 1980