1. Stanford Hospital and Clinics
93 RAYNAUD’S SYNDROME: VASOSPASTIC AND OCCLUSIVE ARTERIAL DISEASE INVOLVING THE DISTAL UPPER EXTREMITY
Vascular Surgery
Stanford Hospital and Clinics

2. 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

4. 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)

5. PREVALENCE
Common – % (US)
Higher in cold climates

6. 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

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

8. MECHAISMS OF PRIMARY VASOSPASM

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

10. 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

11. ABERRANT RIGHT SUBCLAVIAN A.
Table Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome
Table 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

12. 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

13. VARIATIONS OF PALMAR ARCHES
Table Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome
Table 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

14. PRIMARY VS. SECONDARY RAYNAUD’S
Table 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

15. 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

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

17. 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

18. 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

19. FINGER TIP THERMOGRAPHY
Combined with cold immersion

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

21. TREATMENTS

22. 92 UPPER EXTREMITY REVASCULARIZATION

23. 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

24. ETIOLOGY Intrinsic arterial disease Trauma Iatrogenic Non-iatrogenic
Embolic

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

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

27. 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%)

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

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

30. 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

31. 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

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