Cystic adventitial pathology as an entity in peripheral arterial disease

BACKGROUND: Cystic adventitial disease (CAD) is a rare condition in which mucinous cyst forms within the adventitia of arteries and veins. The management of CAD still remains unclear and a wide range of imaging and treatment options has been described. The purpose of this study is to propose an update of etiology, clinical diagnosis, imaging modalities and treatment options in the setting of CAD described on arterial territory. METHODS: We performed a systematic review including studies reporting case or case series of CAD, searching across the Medline, Embase and Cochrane databases. RESULTS: We identified 513 reports and 677 arterial cysts. Various imaging modalities such as Duplex ultrasonography, magnetic resonance imaging, computed tomographic angiography and conventional angiography were included. A wrde range of treatment options have been performed with the most common being cyst resection followed by saphenous vein graft reconstruction in 259 cases. Follow-up was described in 431 cases with an average of 33 months. There were 51 patients who developed cyst recurrence. Among the 102 cases where joint connection was identified, 40 had either ligation of the joint connection or joint resection, which led to no recurrences. CONCLUSIONS: CAD must be considered as en entity in peripheral arterial disease and considered in the differential diagnosis, in particular for middle-aged male patients who show no evidence of atherosclerotic disease. A better understanding of the pathogenesis of CAD will allow a consensus on treatment strategy and improve outcomes by reducing recurrence rates.