Management of Upper Extremity Arterial and

Venous Occlusive DiseasesCLAUDIE SHEAHAN MD

PROFESSOR OF CLINICAL SURGERYLSU SCHOOL OF MEDICINE

Upper extremity arterial disease

• Rare – less than 5% of PVD• Dutch study –

– Prevalence 2.3% diabetics – 0% non-diabetics

Clinical Presentation

• Episodic - Spastic• Claudication• Tissue loss

History and Physical

• Symptoms c/w connective tissue disorders– Arthralgias– Dysphagia– Sclerodactyly– Xeropthalmia– Xerostomia

History and Physical

Also look for signs of• Malignancy• Hypothyroidism• Frostbite• Trauma• Vibrating tools• Drug use

Examination

• Color change• Digital pitting• Digital ulcer • Gangrene

Exam

• Palpation of pulses• Auscultation for bruits• Adsons maneuvers-should be

done sitting• Allens test

Allen’s test

Collateral flow across the palmar arch

Cold provocation testing

Low specificity and reproducibility

Cardiac echo

Look for embolization

potential

X-Rays

• Cervical rib• Soft tissue atrophy• Distal phalangeal tuft

reabsorption

Biopsy

• Skin for vasculitis• SSc atrophy of epidermis and adnexal fat• Arteritis- wall necrosis

Vascular lab• Multiple level segmental

pressures • Multiple level pulse volume

plethysmography (PVR)• Digital pressures /PPG• ABI• DBI (digital brachial index) ≥0.75

Vascular lab

• Demonstrate symmetry – Between arms– Lower extremities

• Gradient 30-50 mmHg with subclavian artery occlusion

• Digital plethysmography for differentiating vasospasm vs occlusive lesion

Angiography

• Aortic arch: atherosclerosis, ulceration, aneurysms

• TOS maneuvers • Retrograde flow in vertebral

“Subclavian Steal”• Vasodilators, hand warming,

magnified view

Angiography in collagen vascular diseases

• Absence of calcium• Total occlusion vs string • Multiple segmental lesions • Less developed collaterals

Upper extremity arterial disease

Occlusive vs. Spastic

Raynaud's Syndrome

•Vasospastic•Primary•Raynaud’s Disease

•Idiopathic

•Obstructive•Secondary•Raynaud’s Phenomena

Primary Raynaud’s

• Exaggerated response to cold, emotional stimuli

• 3 stages: White, Blue, Red• 15-20min episodes• Pain usually absent • Thermal threshold varies 17-26°C

Primary Raynaud’s

• Most common• Benign• Does not lead to severe ischemia

Secondary Raynaud’s

• More likely to progress to severe occlusive lesions within the digital arteries

• Rest pain and ulceration• Associated with Systemic Sclerosis SLE, RA, mixed collagen disease

Epidemiology

• Mostly women• Median age onset 14• Prevalence varies with climate• Study from Portland 30%

incidence• Higher risk in first degree

relative

Pathophysiology

“Local asphyxia of the extremities" was a result of "increased irritability of the central parts of the cord presiding over the vascular innervation"

-Maurice Raynaud 1862

Pathophysiology

“Local fault”

-Sir Thomas Lewis 1930

Bloodwork

• CBC• ESR/CRP• ANA• Biochemical profile• Thyroid function• RF• CXR/thoracic outlet

Nailfold Capilloroscopy

• Early manifestation of scleroderma

• Dilated capillaries, areas of avascularity

Nailfold Capilloroscopy

• Nailfold capillaries lie parallel rather than ꓕ

• Abnormalities not present in vasospastic disease

• Independent predictor for SScdevelopment

1° vs 2° Raynaud's

• Digital ischemia may precede systemic manifestations of these secondary diseases, makes diagnosis more difficult

• % of patients who go on to develop secondary Raynaud’s

Management of Primary Raynaud’s

• Cold avoidance, Gloves• Stop smoking• Stop beta blockers• Stop ergot preparations

(migraine therapy)• Biofeedback doesn’t work

Medical management of Raynaud's

First line therapy – calcium channel blockers

–Procardia XL (nifedipine) 30-60mg qd to bid–Norvasc (amlodipine) 5-10mg qd

Medical management of Raynaud's

Second line therapy with proven efficacy• Angiotensin II RCP blockade (losartan) • ACE Inhibitors (enalopril)• (Prozac) Fluoxetine• Alpha blockers (prazosin)

Alpha blockers reserpine

Medical management of Raynaud's

• Topical vasodilators (GTN)• IV prostanoid (Iloprost)• Endothelin RCP antagonist (Bosentan) • +/- antiplatelets

PDE5 Inhibitors

• Increase availability of NO• Second choice for secondary Raynaud's• Sildenafil (Viagra) 20-25 mg TID

Raynaud's syndrome disordersless than 1% of all Raynaud's

• Immunological and connective tissue disorders– Scleroderma– Mixed CT disease– RA– SLE– Dermatomyositis– Polymyositis– Hep B antigen induced vasculitis– Sjogrens syndrome– Drug induced vasculitis– Hypersensitivity angiitis– Undifferentiated CT disease– Antiphospholipid syndrome

• Obstructive arterial diseases– Atherosclerosis– buergers syndrome– TOS

• Environmental conditions– Vibration injury– Direct arterial trauma– Cold injury

• Drug induced Raynaud's syndrome without vasculitis– Ergot– B blockers– Cytotoxic drugs– OCPS

• Miscellaneous– Vinyl chloride disease– CRF– Cold agglutinins– Cryoglobulinemia– Neoplasia– Neurologic disorders– Endocrine disorders

Small Vessel Occlusion

Mechanism of obstruction is variable–Autoimmune vasculitis - widespread digital and

palmar artery occlusions

Small Vessel Occlusion

Vibrating tools- fibrotic form of

palmar and digital artery obstruction

- presumably shear stress

Small Vessel Occlusion

Pathophysiology–Hypercoagulable states–Embolic–Atherosclerotic–Renal failure

Small vessel occlusion

Gangrene • Usually occlusive disease-either digital or palmar arch. • BUT a proximal embolizing or obstructing lesion should be

looked for• Process of digital gangrene is different from necrosis-it is not

suppurative but a process of desication and mummification

Medical management

• No abx ointment-will dessicate

• Occasional suppurativeprocess requires iv abx, possible debridement

• Auto-amputation

Surgical therapy

• Cervical thoracic sympathectomy results are too short

• Perineural neurectomy• Fat grafting• Botox• Occasionally PTA

Surgical therapy

• Amputation rarely indicated• Spinal cord stimulation

Scleroderma

• Raynaud's phenomena present in 95% pts

• 40-50% pts will have a digital ulcer in their lifetime

Hypothenar Hammer Syndrome

• Male• Sudden onset• Unilateral

Hypothenar Hammer Syndrome

• Visible/palpable mass by hypothenar eminence due to ulnar artery aneurysm

• Aneurysm may embolize• May also present with thrombosis

Hypothenar Hammer Syndrome

Vasculitis of Malignancy

• Hairy cell leukemia– Systemic necrotizing vasculitis

• “Paraneoplastic” vasculitides• Vasculitis with solid tumors

Drug Abuse Vasculitis

• IVDA of meth or cocaine is associated with pan arteritis

• Accidental arterial injection • Treatment

– Heparin, debridement

Antiphospholipid Syndrome

• Acquired autoimmune disorder• Recurrent arterial and venous thrombosis – anywhere• Thrombotic events treated with anticoagulation

Ergotism

• Most cases due to ergot alkaloid treatments

• Can cause severe spasm of digital vessels - gangrene

Thromboangiitis Obliterans (TO)

• Formerly known as Buerger’s• Segmental medium and small vessel occlusion • Arms and legs• 40-50% pts have history of superficial migratory

thrombophlebitis, Raynaud’s or both

Thromboangiitis Obliterans (TO)

• USA• 10% isolated UE involvement• 30-40% UE and LE involvement• 50% LE

Thromboangiitis Obliterans (TO)

Arterial wall inflammatory infiltrate

Etiology unknown – Tobacco is permissive, probably

causative

Diagnosis

Essential Criteria• Onset of sx <45

• Tobacco use

• No disease proximal to popliteal artery

• No disease proximal to distal brachial artery

• Objective documentation distal occlusive disease

Supportive Criteria• Superficial thrombophlebitis

• Raynaud’s

• UE involvement

Differential Diagnosis

Must exclude:• Proximal embolic source• Trauma and local lesion• Autoimmune disease• Hypercoagulable state• Atherosclerosis• Atherosclerotic risk factors (DM, HTN, Hyperlipidemia)

Angiography

• Symmetric

• Radial or ulnar occlusion

• Segmental rather than diffuse

• Extensive digital and palmar artery occlusive disease

Treatment

• Stop smoking• Palliative• Remissions related to resumption of smoking• Limb salvage not as good as with atherosclerosis• Normal life expectancy, no coronary involvement

Large Vessel Arteritis

• Giant Cell• Radiation induced

Giant Cell Arteritis

Systemic GCA (temporal arteritis) vs. Takayasu’s arteritis

Giant Cell Arteritis

Systemic GCA (temporal arteritis)• Usually carotid artery branches

• But any branches

• Most often complaint is severe pain along course of temporal artery

• Women older than 50

Systemic GCA

Diagnosis• Biopsy

• Duplex may be helpful

Treatment• Prompt steroid therapy

• MTX

• Cyclosporin, Azathioprine

• TNF inhibitors

• Pyrimidine synthesis inhibitors

Takayasu’s Arteritis

• Younger patients, female more often

• Smooth tapered stenosis of subclavian, axillary, brachial

• Can cause stenosis of aorta and its branches, main difference is pulmonary artery involvement

• Thoracic aortic aneurysm 17x risk

• AAA 2.4x risk

Takayasu’s Arteritis

Pediatric

• Not as strong sex predilection

• Heart failure is common

• 75% survival at 20 yrs, heart failure driven

• HTN

• Also, myocarditis, coronary artery involvement, valvular involvement, pulmonary involvement

Takayasu’s Arteritis

• Two stages of illness– Acute – Chronic

• Same medications used as temporal arteritis

• Results not as consistent as with TA

Surgical Intervention

• Best done in quiescent (chronic) state

• Symptomatic stenosis

• Aneurysmal lesions

Surgical Intervention

• Successful surgery requires bypass to normal artery

• Continue steroid therapy

• PTA higher restenosis rate than expected, 90%

Surveillance

• Follow ESR, CRP

• PET-CT scan

Upper Extremity Acute Arterial Insufficiency

50% - embolization• mostly cardiac• also aneurysm brachiocephalic/subclavian

25% - primary arterial thrombosis25% - iatrogenic

• ABGs• A lines• AVF creation

Upper Extremity Acute Arterial Insufficiency

• Embolization is associated with 25% mortality–Recurrence and embolization to other vascular beds

• Renal, Mesenteric, Cerebral

Revascularization

• Thrombectomy• Endovascular• Combined

Repetitive Trauma

• Quadrilateral space syndrome-axillary artery injury

• Humeral head syndrome- post humeral circumflex artery injury

• Both lead to embolization• Common in throwing athletes

Large Vessel Occlusive Disease

• Atherosclerosis- most common

• Same risk factors as lower extremity

• Diabetes, renal failure lesions may involve large and small vessels

Large Vessel Occlusive Disease

AneurysmSubclavian and axillary – most common

Iatrogenic venous disease

• Trauma• IV access• PICC lines• Dialysis catheters

COVID 19 – Acute Limb Ischemia

• Thrombotic etiology predominates

• Can occur in pts receiving thromboprophylaxis

COVID 19- Acute Limb Ischemia

• Risk factors – Older age– Obesity– Cardiovascular comorbidities

• More typically affects patients with severe COVID-19

• Occurs five to seven days after respiratory decompensation

COVID 19 – Acute Limb Ischemia

• Thrombosis of large or medium-sized arteries have all been reported.

• Small vessel thrombosis leading to digital gangrene is often associated with the administration of vasopressor agents.

COVID 19 – Acute Limb Ischemia

• About 20 percent of patients who present with COVID-19 related ALI have few or no respiratory symptoms.

• Furthermore, ALI can occur during the recovery phase following infection of any severity.