Stanford Hospital and Clinics 93 RAYNAUD’S SYNDROME: VASOSPASTIC AND OCCLUSIVE ARTERIAL DISEASE INVOLVING THE DISTAL UPPER EXTREMITY Vascular Surgery Stanford Hospital and Clinics 02-13-2006

DEFINITION Raynaud’s Syndrome – episodic pallor or cyanosis of the fingers due to vasoconstriction of small arteries or arterioles in the fingers occurring in in response to cold or emotional stress Raynaud’s disease – primary vasospastic disorder without identifiable underlying cause Raynaud’s phenomenon – vasospasm secondary to an underlying condition or disease

CLINICAL PRESENTATION Induced by cold exposure Sudden onset of waxy pallor of digits Cyanosis follows the pallor Resolving with hyperemia and rubor of the skin Female > male (4:1 to 1.6:1)

PREVALENCE Common – 3.5-4.6% (US) Higher in cold climates

DIAGNOSIS OF PRIMARY RAYNAUD’S SYNDROM Vasospastic attacks precipitated by exposure to cold or emotional stimuli Symmetrical or bilateral involvement of the extremities Absence of gangrene Symptom present for a minimum of 2 years Absence of any other underlying disease

BLOOD FLOW REGULATION OF FINGERS “Hunting response” – responding to cold temperature, arterial vasoconstriction and dilatation alternates. Frequency about every 30 seconds to 2 minutes

MECHAISMS OF PRIMARY VASOSPASM

SECONDARY VASOSPASTIC DISORDER Existing fixed vascular obstruction Decrease the threshold for cold-induced vasospasm Conditions causing vessel lumen narrowing - Scleroderma Increasing viscosity - Myeloma

ANATOMY OF UPPER EXTREMITY AND POTENTIAL ETIOLOGY Direct compression - Aberrant right subclavian artery, Thoracic outlet syndrome Embolization – Thoracic outlet syndrome, atherosclerosis Deep and superficial palmar arches

ABERRANT RIGHT SUBCLAVIAN A. Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome ABERRANT RIGHT SUBCLAVIAN A. TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies

Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome NORMAL PALMAR ARCHES TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies

VARIATIONS OF PALMAR ARCHES Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome VARIATIONS OF PALMAR ARCHES TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies

PRIMARY VS. SECONDARY RAYNAUD’S Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome PRIMARY VS. SECONDARY RAYNAUD’S TYPE GENDER OTHER FEATURES Primary Usually female Age < 45 years   Vasospasm of multiple or all digits Normal vascular examination No skin abnormalities Normal laboratory studies Secondary Male or female Any age Single digit involved Abnormal pulse examination Vascular laboratory abnormalities Positive autoantibodies

GENERAL CATEGORY SPECIFIC DISORDERS Connective tissue disease Scleroderma, CREST   Systemic lupus erythematosus Rheumatoid arthritis Mixed connective tissue disease Overlap connective tissue disease Dermatomyositis and polymyositis Vasculitis (small, medium-sized vessel) Occlusive arterial disease Atherosclerosis Thromboangiitis obliterans (Buerger's disease) Giant cell arteritis Arterial emboli (cardiac and peripheral) Thoracic outlet syndrome Occupational arterial disease Hypothenar hammer syndrome Vibration induced Drug-induced vasospasm β-Adrenergic blocking drugs Vasopressors Ergot Cocaine Amphetamines Vinblastine/bleomycin Myeloproliferative and hematologic disease Polycythemia rubra vera Thrombocytosis Cold agglutinins Cryoglobulinemia Paraproteinemia Malignancy Multiple myeloma Leukemia Adenocarcinoma Astrocytoma Infection Hepatitis B and C antigenemia Parvovirus Purpura fulminans

PHYSICAL EXAMINATION Investigate causes for secondary Raynaud’s Exam heart Upper extremity vascular exams

SEGMENTAL PRESSURE MEASUREMENT To eval large vessel occlusive diease Measure systolic pressures at brachial, upper elbow, and wrist Abnormal – difference > 10 mm Hg Wrist-brachial ratio - > 0.8

FINGER SYSTOLIC BLOOD PRESSRES Normal finger-brachial index – 0.8 to 1.27 Occlusive disease – diff. > 15 mm Hg, or, finger SBP<70 mm Hg Measure while changing finger temperature

FINGER TIP THERMOGRAPHY Combined with cold immersion

OTHER TESTS Cold recovery time – NL <10 mins Laser Doppler Flux Duplex ultrasound Contrast Angiography – gold standard

TREATMENTS

92 UPPER EXTREMITY REVASCULARIZATION

OVERVIEW AND PRESENTATION Symptomatic UE ischemia is rare – 5% Most are primary Raynaud’s syndrome – medical management Acute ischemia – 5 “P”s Chronic ischemia – equivalent of claudication (dominant hand more) Tissue loss are rare – rich collaterals Axillary A. ligation – 10% limb loss Brachial A. ligation – 3-5% lead to gangrene

ETIOLOGY Intrinsic arterial disease Trauma Iatrogenic Non-iatrogenic Embolic

INTRINSIC ARTERIAL DISEASE Atherosclerosis Rare to upper extremity Occasionally seen in axillary, brachial, radial and ulnar A. FMD Hypothenar hammer syndrome – distal ulnar A

TRAUMA Iatrogenic Brachial A. – most common (0.9-4% after cath) Axillary A. – 0.8% thrombotic complications Radial A. – 5-40% (hand ischemia 0.3-0.5%) Non-iatrogenic Blunt – intimal disruption, early/late presentation Traction – intimal disruption (mild), arterial disruption (severe) Penetrating – direct/blast injury

EMBOLI Account for 25% total embolic event External source – cardiac, aortic arch, subclavian A pathology Intrinsic source – intimal flaps, stenosis, injection Most common source – cardiac (A-Fib) Most common location – Brachial A. (60%)

EVALUATION Acute ischemia – PE Segmental pressure Duplex ultrasound CTA MRA Angiogram

TREATMENT Acute injury – urgent operation Chronic – depends on clinical presentation

AXILLARY ARTERY Proximal portion – transverse incision at deltopectoral groove Distal portion – axillary or upper arm incision End to end anastomosis Saphenous vein is the graft of choice Chronic occlusion – carotid-to-brachial bypass, or axillary-to-brachial bypass

BRACHIAL ARTERY Embolectomy – incision below the antecubital fossa Incision right on the projected injury site Long segment occlusion – Saphenous vein graft Direct end-to end anastomosis

RADIAL AND ULNAR ARTERIES Rarely necessary Acute traumatic injury – urgent repair Embolectomy – antecubital fossa