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Open Access 01-12-2014 | Case report

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

Authors: Luisa Zacarias Föhrding, Timur Sellmann, Sebastian Angenendt, Detlef Kindgen-Milles, Stefan A Topp, Bernhard Korbmacher, Artur Lichtenberg, Wolfram T Knoefel

Published in: Journal of Cardiothoracic Surgery | Issue 1/2014

Abstract

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.

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Title: A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1
Authors: Luisa Zacarias Föhrding
Timur Sellmann
Sebastian Angenendt
Detlef Kindgen-Milles
Stefan A Topp
Bernhard Korbmacher
Artur Lichtenberg
Wolfram T Knoefel
Publication date: 01-12-2014
Publisher: BioMed Central
Published in: Journal of Cardiothoracic Surgery / Issue 1/2014
Electronic ISSN: 1749-8090
DOI: https://doi.org/10.1186/s13019-014-0172-y

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

Abstract

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