Involvement of peripheral arteries in Von Recklinghausen neurofibromatosis

• Published in: Journal des maladies vasculaires Vol. 36; no. 3; p. 189
• Main Authors: Elmesnaoui, A, Benlahbib, M, Lekehal, B, Bouayad, M, Sefiani, Y, Ammar, F, Bensaid, Y
• Format: Journal Article
• Language: French
• Published: France 01.06.2011
• Subjects: Adolescent; Adult; Female; Humans; Male; Neurofibromatosis 1 - complications; Peripheral Vascular Diseases - etiology
• ISSN: 2214-8116
• DOI: 10.1016/j.jmv.2011.02.002

Arterial lesions are relatively rare in neurofibromatosis type I but can have potentially serious consequences for the patient. We report two clinical cases of peripheral arterial involvement in neurofibromatosis type 1. The first case was a 25-year-old female with Von Recklinghausen disease who developed a swollen tender mass around her left arm. The arterial CT scan revealed a false aneurysm of the brachial artery. The surgical procedure involved repair of the false aneurysm, and ligation of the brachial artery. Intra- and postoperative bleeding was severe, leading to hemodynamic instability and impaired hemostasis. The patient died on day 2, postoperatively. The second case involved a 17-year-old male with Von Recklinghausen disease who presented stage II arteritis of the right lower limb. Arteriography revealed a long stenosis of the right superficial femoral artery extending to the popliteal artery and an important collateral circulation in the leg arteries. Medical treatment with regular clinical supervision was recommended in this patient.