93 RAYNAUD’S SYNDROME: VASOSPASTIC AND OCCLUSIVE ARTERIAL DISEASE INVOLVING THE DISTAL UPPER EXTREMITY

Vascular SurgeryStanford Hospital and Clinics

02-13-2006

• 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

DEFINITION

• 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)

CLINICAL PRESENTATION

• Common – 3.5-4.6% (US)

• Higher in cold climates

PREVALENCE

• 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

DIAGNOSIS OF PRIMARY RAYNAUD’S SYNDROM

• “Hunting response” – responding to cold temperature, arterial

vasoconstriction and dilatation alternates. Frequency about every

30 seconds to 2 minutes

BLOOD FLOW REGULATION OF FINGERS

MECHAISMS OF PRIMARY VASOSPASM

• Existing fixed vascular obstruction

• Decrease the threshold for cold-induced vasospasm

• Conditions causing vessel lumen narrowing - Scleroderma

• Increasing viscosity - Myeloma

SECONDARY VASOSPASTIC DISORDER

• Direct compression - Aberrant right subclavian artery, Thoracic outlet

syndrome

• Embolization – Thoracic outlet syndrome, atherosclerosis

• Deep and superficial palmar arches

ANATOMY OF UPPER EXTREMITY AND POTENTIAL ETIOLOGY

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

NORMAL 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

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

PRIMARY VS. SECONDARY RAYNAUD’S

Table 93-1. Clinical Features to Distinguish Primary from Secondary Raynaud's Syndrome

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

• Investigate causes for secondary Raynaud’s

• Exam heart

• Upper extremity vascular exams

PHYSICAL EXAMINATION

• 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

SEGMENTAL PRESSURE MEASUREMENT

• 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 SYSTOLIC BLOOD PRESSRES

• Combined with cold immersion

FINGER TIP THERMOGRAPHY

• Cold recovery time – NL <10 mins

• Laser Doppler Flux

• Duplex ultrasound

• Contrast Angiography – gold standard

OTHER TESTS

TREATMENTS

92 UPPER EXTREMITY REVASCULARIZATION

• 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

OVERVIEW AND PRESENTATION

• Intrinsic arterial disease

• Trauma

Iatrogenic

Non-iatrogenic

• Embolic

ETIOLOGY

• Atherosclerosis

Rare to upper extremity

Occasionally seen in axillary, brachial, radial and ulnar A.

• FMD

• Hypothenar hammer syndrome – distal ulnar A

INTRINSIC ARTERIAL DISEASE

• 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

TRAUMA

• 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%)

EMBOLI

• Acute ischemia – PE

• Segmental pressure

• Duplex ultrasound

• CTA

• MRA

• Angiogram

EVALUATION

• Acute injury – urgent operation

• Chronic – depends on clinical presentation

TREATMENT

• 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

AXILLARY 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

BRACHIAL ARTERY

• Rarely necessary

• Acute traumatic injury – urgent repair

• Embolectomy – antecubital fossa

RADIAL AND ULNAR ARTERIES