title: A case of lethal spontaneous massive hemothorax in a patient with neurofibromatosis 1.
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.
authors.forename: Luisa Zacarias
authors.initials: LZ
authors.lastname: Föhrding
authors.forename: Timur
authors.initials: T
authors.lastname: Sellmann
authors.forename: Sebastian
authors.initials: S
authors.lastname: Angenendt
authors.forename: Detlef
authors.initials: D
authors.lastname: Kindgen-Milles
authors.forename: Stefan A
authors.initials: SA
authors.lastname: Topp
authors.forename: Bernhard
authors.initials: B
authors.lastname: Korbmacher
authors.forename: Artur
authors.initials: A
authors.lastname: Lichtenberg
authors.forename: Wolfram T
authors.initials: WT
authors.lastname: Knoefel
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006491:Hemothorax / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D008588:Meningocele / Q000150:complications*; D009456:Neurofibromatosis 1 / Q000150:complications*; D011859:Radiography