A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1

Neurofibromatosis type 1 is an autosomal dominant disease characterized by multiple dermatological disorders amongst others. Among the less frequent manifestations are vascular abnormalities. Here, we present a case of spontaneous massive hemothorax in a 39-year-old Caucasian woman with neurofibroma...

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Detalles Bibliográficos
Autores principales: Zacarias Föhrding, Luisa, Sellmann, Timur, Angenendt, Sebastian, Kindgen-Milles, Detlef, Topp, Stefan A, Korbmacher, Bernhard, Lichtenberg, Artur, Knoefel, Wolfram T
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223165/
https://www.ncbi.nlm.nih.gov/pubmed/25348553
http://dx.doi.org/10.1186/s13019-014-0172-y
Descripción
Sumario:Neurofibromatosis type 1 is an autosomal dominant disease characterized by multiple dermatological disorders amongst others. Among the less frequent manifestations are vascular abnormalities. Here, we present a case of spontaneous massive hemothorax in a 39-year-old Caucasian woman with neurofibromatosis 1 and a thoracic meningocele with a lethal outcome despite extensive surgical intervention as well as intensive care measures. Spontaneous hemothorax is a rare, but potentially lethal complication of neurofibromatosis type 1, which necessitates quick and decisive intervention; endovascular embolization where possible, otherwise aggressive surgical intervention in unstable patients. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s13019-014-0172-y) contains supplementary material, which is available to authorized users.

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