A very rare noninflammatory progressive vasculopathy characterized by multiple cerebrovascular accidents and the presence of idiopathic livedo reticularis (livedo racemosa).
Livedo Reticularis and Cerebrovascular Accidents; Sneddon-Champion Syndrome.
A genetic disorder first described by Ian Bruce Sneddon, British dermatologist in 1965.
Incidence estimated to be 4/1,000,000 per annum.
May be a particular form of obliterating vasculitis or of an Antiphospholipid Antibodies Syndrome.
Clinically evocated in patients combining livedo reticularis and neurological signs. Skin biopsies can be contributive (arterial intimal hyperplasia).
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.
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.
Consider interaction between antiepileptic treatment and anesthetic drugs.
et al: The natural course of Sneddon syndrome: Clinical and magnetic resonance imaging findings in a prospective six-year observation study. J Neurol Neurosurg Psychiatry
RPM, van der Veen
et al: Sneddon’s syndrome: Case report and literature review. J Neurol Sci
R: Anaesthesiological considerations in patients with Sneddon’s syndrome. Paediatr Anaesth
et al: Perioperative management of Sneddon syndrome during thyroidectomy. J Clin Anesth
H: Sneddon’s syndrome: A comprehensive review of the literature. Orphanet J Rare Dis