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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


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