Endovascular Therapy for Subclavian Artery Rupture in von Recklinghausen Disease

• Published in: Vascular and endovascular surgery Vol. 44; no. 8; pp. 714 - 717
• Main Authors: Santin, Brian J., Guy, Gregory E., Bourekas, Eric C., Go, Michael R.
• Format: Journal Article
• Language: English
• Published: Los Angeles, CA SAGE Publications 01-11-2010; Sage Publications
• Subjects: Adult; Biological and medical sciences; Blood Vessel Prosthesis; Blood Vessel Prosthesis Implantation - instrumentation; Blood. Blood coagulation. Reticuloendothelial system; Endovascular Procedures - instrumentation; Female; Humans; Medical sciences; Neurofibromatosis 1 - complications; Neurofibromatosis 1 - diagnostic imaging; Pharmacology. Drug treatments; Rupture, Spontaneous; Stents; Subclavian Artery - diagnostic imaging; Subclavian Artery - surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tomography, X-Ray Computed; Treatment Outcome; Vascular surgery: aorta, extremities, vena cava. Surgery of the lymphatic vessels; artery rupture; subclavian artery; von Recklinghausen disease; Nervous system diseases; Treatment; Phacomatosis; Surgery; Neurofibromatosis; Rupture; Cardiovascular disease; Endovascular route; Benign neoplasm; Subclavian artery
• ISSN: 1538-5744; 1938-9116
• DOI: 10.1177/1538574410373836

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.