Upper airway obstruction and hemorrhagic shock due to ruptured aneurysms in a patient with neurofibromatosis type 1

Published:September 29, 2020DOI:


      • Neurofibromatosis type 1 is rarely complicated with an artery aneurysm formation and rupture.
      • Rupture of artery aneurysms in the neck region can be life-threatening because of upper airway obstruction and hemorrhagic shock.
      • Endovascular treatment has been shown to be safe and effective in NF-1 patients with artery aneurysm formation and rupture.


      To read this article in full you will need to make a payment
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The American Journal of Emergency Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Oderich G.S.
        • Sullivan T.M.
        • Bower T.C.
        • Gloviczki P.
        • Miller D.V.
        • Babovic-Vuksanovic D.
        • et al.
        Vascular abnormalities in patients with neurofibromatosis syndrome type I: clinical spectrum, management, and results.
        J Vasc Surg. 2007; 46: 475-484
        • Seow Vei-Ken
        • Chong Chee-Fah
        • Wang Tzong-Luen
        • You Chi-Fang
        • Han He-Yi
        • Chen Chien-Chih
        Ruptured left subclavian artery aneurysm presenting as upper airway obstruction in von Recklinghausen’s disease.
        Resuscitation. 2007; 74: 563-566
        • Peyron Pierre-Antoine
        • Pollanen Michael S.
        Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1.
        Forensic Sci Med Pathol. 2017; 13: 436-440
        • Souftas Vasileios
        • Tsivgoulis Georgios
        • Kirmanidis Michalis
        • Vadikolias Konstantinos
        • Piperidou Charitomeni
        • Prassopoulos Panos
        Spontaneous subclavian artery rupture in neurofibromatosis type I.
        Neurol Sci. 2011; 32: 979-980
        • Roger W.
        Byard. Forensic considerations in cases of neurofibromatosis--an overview.
        J Forensic Sci. 2007; 52: 1164-1170
        • Bargiela David
        • Verkerk Misha M.
        • Wee Ian
        • Welman Kiera
        • Ng Eugene
        • Choong Andrew M.T.L.
        The endovascular management of neurofibromatosis-associated aneurysms: A systematic review.
        Eur J Radiol. 2018; 100: 66-75
        • Pulivarthi S.
        • Simmons B.
        • Shearen J.
        • Gurram M.K.
        Spontaneous hemothorax associated with neurofibromatosis type I: a review of the literature.
        J Neurosci Rural Pract. 2014; 5: 269-271
        • Jang J.Y.
        • Lim Y.S.
        • Woo J.H.
        • Jang J.H.
        Spontaneous rupture of intercostal artery after severe cough.
        Am J Emerg Med. 2015; 33 (e1–3): 131