Pezzetta Edgardo, Paroz Alexandre, Ris Hans Beat, Martinet Olivier
Department of Surgery, Service de Chirurgie, Centre Hospitalier Universitaire Vaudois, 1011, Lausanne-CHUV, Switzerland.
Eur J Cardiothorac Surg. 2003 Jun;23(6):1062-4. doi: 10.1016/s1010-7940(03)00145-3.
Spontaneous hemothorax is a rare and life threatening complication of neurofibromatosis. Two types of vascular involvement have been described: (a) stenotic or aneurysmal alterations in large vessels such as the aorta and its branches; and (b) dysplastic features in smaller vessels. Thoracotomy and surgical ligation of the bleeding vessels is primarily indicated in the presence of active bleeding with associated hemodynamic compromise. A more conservative approach with endovascular embolization or non-operative management have also been reported in case of hemodynamic stability. We present a rare case of spontaneous hemothorax treated conservatively in a patient with von Recklinghausen's disease.
自发性血胸是神经纤维瘤病一种罕见且危及生命的并发症。已描述了两种血管受累类型：(a) 大血管如主动脉及其分支的狭窄或动脉瘤样改变；(b) 小血管的发育异常特征。在存在活动性出血并伴有血流动力学不稳定时，主要指征是开胸手术并结扎出血血管。对于血流动力学稳定的情况，也有采用血管内栓塞或非手术治疗等更保守方法的报道。我们报告了一例在患有冯·雷克林豪森病的患者中采用保守治疗的罕见自发性血胸病例。
