RESUMO
Acute abdominal aortic occlusion (AAAO) is a rare, life threatening condition, which usually occurs in elderly patients, causing challenging management issues. In patients who have no cardiac or vascular disease this catastrophic event is very rare and is due to hypercoagulable disorders. This study reviews the literature on AAAO in hypercoagulable states in the light of our experience on a case of an acute thrombosis of nonaneurysmal, nonatherosclerotic abdominal aorta in a female patient with protein S deficiency and Sjögren's syndrome and her younger brother, which was found to have atherosclerotic involvement of distal aorta and elevation in homocysteine levels. Because of a misleading clinical presentation, the diagnosis was delayed and conservative treatment failed. Both were successfully treated with emergency aorto-bifemoral grafting. Other cases of arterial thrombosis and hypercoagulable disorders were found in first-degree relatives. Our experience and the review of the literature suggest that the interaction between host and environment factors can lead to acute thrombosis of the non-pathologic abdominal aorta; not only classic hypercoagulability disorders, but also immunologic, metabolic, toxicological cofactors can be involved. Delay in diagnosis is frequent and may not influence the prognosis, but does not allow conservative therapy. Prognosis depends mainly on pathologic cofactors that require detection and appropriate treatment in order to prevent complications and recurrences.
