Cholesterol Embolisation Syndrome

Learning objectives

Incidence and prevalence of Cholesterol Embolization Syndrome Clinical presentation with thunderclap headaches Diagnostics and Management

Introduction

• Embolisation of the contents of an atherosclerotic plaque (primarily cholesterol crystals) from a proximal large artery
• Causes distal small to medium arteries causing mechanical plugging and an inflammatory response.
• Embolisation distal to head/neck vessel scan cause acute limb, renal, gut is ischaemia but is not covered here

Aetiology

• Causes stroke and neurological damage if lesions are proximal to head and neck vessels
• Generally characterized by a multitude of small emboli/microemboli occurring over time.
• Usually would involve ascending aorta
• Usually causes diffuse and small infarcts
• Non-specific acute inflammatory response
• Plaque damage during procedures e.g. coronary angiography but this seems rare

Pathological criteria

The following 6 key elements are required for the development of cholesterol embolisation syndrome:

Presence of a plaque in a proximal, large-caliber artery (such as the internal carotid artery, the iliac arteries, or the aorta) Plaque rupture (spontaneous, traumatic, or iatrogenic) Embolization of plaque debris (containing cholesterol crystals, platelets, fibrin, and calcified detritus) Lodging of the emboli in small to medium arteries with a diameter of 100 to 200 micrometres, leading to mechanical occlusion Foreign-body inflammatory response to cholesterol emboli End-organ damage due to a combined effect of mechanical plugging and inflammation

Clinical

• Acute ischaemic stroke may be caused often diffuse and small
• Constitutional symptoms such as fever and malaise
• These episodes may be recurrent
• Visual symptoms : cholesterol emboli may be seen by fundoscopy
• Livedo reticularis, blue toe syndrome, skin ulcers, digital gangrene
• Acute renal failure and hypertension

Differential

• Artery to arterial thromboembolism in which thrombus that forms over an atheromatous plaque in large artery which embolises. This is much commoner.
• Cardioembolic stroke

Investigations

• Raised WCC (hypereosinophilia) and elevated ESR and CRP due perhaps to IL-5 release
• U&E : may show an AKI
• Transoesophageal echocardiography (TEE) is the most commonly used imaging technique for the detection and measurement of the aortic atherosclerotic plaque
• CT/MRI Aorta can also detect aortic plaque
• CT Brain - may show infarction(s) but MRI more sensitive showing distribution
• Carotid duplex : may show plaque
• ECG and 24 hr tape and Echo: exclude AF and cardioembolic causes
• Skin or Muscle biopsy: may be done to confirm diagnosis

Management

• No specific treatment beyond acute stroke care. Uncertainty over the use of thrombolysis.
• It is a manifestation of atherosclerosis so management of smoking, hypertension, and serum cholesterol strongly advised
• Statin therapy may decrease the risk of cholesterol embolisation syndrome

References and further reading

• Cholesterol Embolisation Syndrome. Circulation. 2010;122:631-641

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