Literature DB >> 32252932

Subclavian Artery Branch Pseudoaneurysm Rupture With Massive Hemothorax in a Patient With Neurofibromatosis Type 1.

Katherine E Negreira1, John P Lichtenberger2, Blair Allais2, Ammar Alhaddad2, Matthew Bernetich2, Vivek Jain2.   

Abstract

Neurofibromatosis type 1 is a rare disorder that occurs secondary to pathogenic variants in the NF1 tumor suppressor gene on chromosome 17. Characteristic clinical manifestations include multiple hyperpigmented macules, axillary and inguinal freckling, optic gliomas, and numerous skin neurofibromas. Vasculopathies are a rare complication of this disease and can affect vessels ranging from the proximal aorta to small arterioles, with pathology including arterial stenosis, aneurysms, and arteriovenous malformations. Aneurysms in these patients are often asymptomatic, and most patients with this complication appear for treatment after vessel rupture. We describe a 33-year-old man with neurofibromatosis type 1 who presented with chest pain and was ultimately found to have a ruptured left subclavian artery branch pseudoaneurysm leading to a large hemothorax.
Copyright © 2019 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  aneurysm; hemothorax; neurofibromatosis; pseudoaneurysm

Mesh:

Year:  2020        PMID: 32252932     DOI: 10.1016/j.chest.2019.11.001

Source DB:  PubMed          Journal:  Chest        ISSN: 0012-3692            Impact factor:   9.410


  1 in total

1.  Bullous lung disease in association with an isolated giant plexiform neuroma: A case report and brief review of pulmonary involvement in neurofibromatosis type 1.

Authors:  M S Shongwe; M J Mpe
Journal:  Afr J Thorac Crit Care Med       Date:  2021-12-31
  1 in total

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