Endovascular Therapy for Subclavian Artery Rupture in von Recklinghausen Disease

Abstract

Vascular anomalies are extremely rare in patients with von Recklinghausen disease. This report presents a case of an acute spontaneous subclavian artery rupture in a patient with von Recklinghausen disease. A 44-year-old woman with a history of neurofibromatosis type 1, multiple sclerosis, and aortic valve replacement experienced a ‘‘popping sensation’’ in her neck. An emergent angiogram via a right brachial artery approach revealed active extravasation of contrast from the proximal part of the right subclavian artery between the vertebral and axillary arteries. An 8 mm × 5 cm endoprosthesis stent graft was placed across the area of extravasation via the brachial sheath. Completion angiography revealed brisk flow through the stent graft with resolution of the area of extravasation and no residual stenosis. Arterial rupture, aneurysm formation, stenosis, and dissection can be fatal in patients with neurofibromatosis. Further research is needed to determine screening guidelines and management algorithms for this patient population.