Sneddon Syndrome

At a glance

A very rare noninflammatory progressive vasculopathy characterized by multiple cerebrovascular accidents and the presence of idiopathic livedo reticularis (livedo racemosa).

Synonyms

Livedo Reticularis and Cerebrovascular Accidents; Sneddon-Champion Syndrome.

History

A genetic disorder first described by Ian Bruce Sneddon, British dermatologist in 1965.

Incidence

Incidence estimated to be 4/1,000,000 per annum.

Genetic inheritance

Autosomal dominant.

Pathophysiology

May be a particular form of obliterating vasculitis or of an Antiphospholipid Antibodies Syndrome.

Diagnosis

Clinically evocated in patients combining livedo reticularis and neurological signs. Skin biopsies can be contributive (arterial intimal hyperplasia).

Clinical aspects

Onset possible in childhood. A delay from months to years is possible between skin and neurological lesions. Features can involve skin (livedo reticularis with no infiltration, and affecting the limbs, trunk, and sometimes the face), central nervous system (cerebrovascular accidents, epilepsy, vertigo, and sometimes Pseudobulbar Syndrome, chorea, episodes of amnesia, or transient amaurosis), and cardiovascular system (occlusive noninflammatory arteriopathy; arteriography can show multiple occlusions in medium-sized arteries; CT scan can show defect corresponding to infarct). Laboratory investigations can find antiphospholipid antibodies, anticardiolipin antibody, and lupus anticoagulant.

Precautions before anesthesia

Evaluate neurological function (clinical, history, EEG, CT/MRI scan). The cardiovascular function must be systematically evaluated to eliminate the presence of systemic arteries obliteration that has been described.

Anesthetic considerations

Blood pressure control is necessary and may require invasive monitoring. Regional anesthesia is not contraindicated but benefit (particularly of central blockade) has to be clearly established. Careful balance between bleeding and risk of thrombosis. Consider thromboelastography.

Pharmacological implications

Consider interaction between antiepileptic treatment and anesthetic drugs.

References