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Sneddon's syndrome is still raising some nosological and etiopathogenic questions. The occurrence of ischemic stroke in young adults especially in the presence of livedo racemosa should suggest the diagnosis and encourage to perform a skin biopsy, which could strengthen the diagnosis. Management begins with prevention of vascular risk factors and treatments based primarily on anti-thrombotic. Large series of studies over several years could provide clarification of the etiopathogenesis of this syndrome and pave the way for the development of diagnostic criteria and new effective therapies in order to prevent progression to irreversible cognitive impairment., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Sneddon's syndrome is a rare combination of generalised livedo reticularis and cerebrovascular accidents. Its clinical presentation varies widely and its aetiology is still not known. 60 to 80% of patients are female. First symptoms of the syndrome are mostly repetitive cerebral strokes, but reduced perfusion of the skin, seen as blue or red-brown mottling, precedes the strokes. The vascular disease is generalised and often accompanied by arteriosclerosis, systemic arterial hypertension, valvular heart disease and the presence of antiphospholipid antibodies. The diagnostic procedures are complicated and have to exclude other autoimmunological diseases. Therapeutic options are anticoagulatory therapy with warfarin, ASS or heparin, reduction of endothelial proliferation with ACE-inhibitors, and improvement of microvascular perfusion with prostaglandine. The increased anaesthesiological risk with these patients is due to the acute risk of thromboembolism and ischaemic cerebral and cardiovascular insults. The anaesthetic management must provide stable perfusion pressures for cerebral and myocardial arteries and avoid increasing risk factors for thromboembolism such as increased blood viscosity or stasis due to improper positioning of the patient. The choice of anaesthetic drugs is dependent on good controllability for haemodynamic stability. The high risk of patients with Sneddon's syndrome justifies a more invasive haemodynamic monitoring and postoperative surveillance on an intensive care unit. This case report describes the anaesthesiological considerations for, and management of, a patient with Sneddon's syndrome who was admitted to hospital for vaginal hysterectomy.

Mesenteric ischaemia is a rare but serious cause of abdominal pain. We present the case of a man with Sneddon's syndrome, who had symptomatic mesenteric ischaemia secondary to a superior mesenteric artery stenosis in conjunction with a hepatic artery stenosis. As far as the authors are aware, this has not previously been described in Sneddon's syndrome, which is a vascular systemic disease characterized by an association between cerebrovascular accidents and a livedo reticularis skin rash. He was treated with balloon angioplasty and stent insertion, with good symptomatic improvement. This has implications for other stenoses in this condition should they become symptomatic.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebrovascular ischemic events. Clinical and MRI aspects are described in 26 patients with Sneddon syndrome. MRI features were classified in 6 groups according to the aspect and topography of the lesions. No correlation was found between the presence of vascular risk factors, valvulopathy or antiphospholipid antibodies and the presence of dementia or MRI lesions. There was a significant correlation between MRI extension of the lesions and clinical disability. Clinical course and MRI findings are presented according to the treatment.

Sneddon syndrome is a systemic non inflammatory arteriopathy characterized by the association of livedo reticularis, thrombotic cerebrovascular disease, coronary artery disease and hypertension. Middle age females are usually affected. It should be considered in the differential diagnosis of acute ischemic attacks in younger patients. It has been related to the antiphospholipid antibody syndrome. Treatment is based on control of hypertension and cardiovascular risk factors along with antiplatelet agents. Two cases meeting the clinical criteria outlined above are reported.