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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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
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author Zacarias Föhrding, Luisa
Sellmann, Timur
Angenendt, Sebastian
Kindgen-Milles, Detlef
Topp, Stefan A
Korbmacher, Bernhard
Lichtenberg, Artur
Knoefel, Wolfram T
author_facet Zacarias Föhrding, Luisa
Sellmann, Timur
Angenendt, Sebastian
Kindgen-Milles, Detlef
Topp, Stefan A
Korbmacher, Bernhard
Lichtenberg, Artur
Knoefel, Wolfram T
author_sort Zacarias Föhrding, Luisa
collection PubMed
description 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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spelling pubmed-42231652014-11-08 A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1 Zacarias Föhrding, Luisa Sellmann, Timur Angenendt, Sebastian Kindgen-Milles, Detlef Topp, Stefan A Korbmacher, Bernhard Lichtenberg, Artur Knoefel, Wolfram T J Cardiothorac Surg Case Report 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. BioMed Central 2014-10-29 /pmc/articles/PMC4223165/ /pubmed/25348553 http://dx.doi.org/10.1186/s13019-014-0172-y Text en © Zacarias Föhrding et al.; licensee BioMed Central Ltd. 2014 This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Zacarias Föhrding, Luisa
Sellmann, Timur
Angenendt, Sebastian
Kindgen-Milles, Detlef
Topp, Stefan A
Korbmacher, Bernhard
Lichtenberg, Artur
Knoefel, Wolfram T
A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
title A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
title_full A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
title_fullStr A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
title_full_unstemmed A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
title_short A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
title_sort case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
topic Case Report
url 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
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