A very rare progressive disorder affecting blood
vessels and characterized by multiple cerebrovascular accidents and the
presence of idiopathic livedo reticularis.
Livedo Reticularis and Cerebrovascular Accidents;
A genetic disorder first described by Ian Bruce Sneddon,
British dermatologist in 1965.
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, lupus anticoagulant.
Evaluate neurological function
(clinical, history, EEG, CT/MRI scan).
function must be systematically evaluated to eliminate the presence of systemic
arteries obliteration that have 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
Consider interaction between
antiepileptic treatment and anesthetic drugs.
Boesch SM, Plorer AL, Auer AJ, 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 74(4):542, 2003.
Bruyn RPM, van der Veen JPW, Donker AJM, et al: Sneddon's syndrome: Case
report and literature review. J Neurol Sci
Heesen M, Rossaint R: Anaesthesiological considerations in patients with
Sneddon's syndrome. Paediatr Anaesth 10(6):678, 2000.