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

作者：Foehrding Luisa Zacarias; Sellmann Timur; Angenendt Sebastian; Kindgen Milles Detlef; Topp Stefan A; Korbmacher Bernhard; Lichtenberg Artur; Knoefel Wolfram T^*

来源：Journal of Cardiothoracic Surgery, 2014, 9(1): 172.

DOI：10.1186/s13019-014-0172-y

• Neurofibromatosis type 1
• Spontaneous hemothorax
• Meningocele
• Endovascular embolization

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.

• 出版日期2014-10-29