[Perioperative management of a patient with Sneddon syndrome--a case report].

Abstract

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.