Xie Bao-song, Chen Yu-sheng, Lin Mei-fu, Huang Qing-hua, Lin Zhang-shu
Department of Respiratory Medicine, Fujian Provincial Hospital, Fuzhou 350001, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2006 Dec;29(12):801-3.
To describe the characteristics of Dieulafoy's disease of the bronchus.
One patient with Dieulafoy's disease of bronchus was described and relevant literatures were reviewed.
Bronchial Dieulafoy's disease is extremely rare, characterized by the erosion of an anomalous artery in the submucosa of bronchus. The etiology of Dieulafoy's disease of bronchus is still unknown, but congenital vascular malformation and airway chronic inflammation or bronchial injury have been postulated. The sudden onset of massive hemoptysis and fatal hemorrhage caused by biopsy injury are common manifestations. Bronchial and pulmonary angiography is a very useful examination before operation, and histopathological diagnosis can be made by the examination of the surgical specimen and the necroscopic specimen. Selective bronchial artery embolization could be proposed as a method for stopping the bleeding, but it may fail and bleeding may recur. At present, lobectomy of the lung is a fundamental approach.
Bronchial Dieulafoy's disease should be included in the differential diagnosis of cryptogenic hemoptysis, and if a cone-shaped endobronchial protrusion is found by bronchoscopic examination, care should be taken for potential danger of a biopsy.
描述支气管迪厄拉富瓦病的特征。
描述1例支气管迪厄拉富瓦病患者并复习相关文献。
支气管迪厄拉富瓦病极为罕见,其特征为支气管黏膜下异常动脉的侵蚀。支气管迪厄拉富瓦病的病因尚不清楚,但推测与先天性血管畸形以及气道慢性炎症或支气管损伤有关。大量咯血突然发作以及活检损伤导致的致命性出血是常见表现。支气管和肺动脉造影是术前非常有用的检查,通过手术标本和尸检标本检查可作出组织病理学诊断。可提出选择性支气管动脉栓塞作为止血方法,但可能失败且出血可能复发。目前,肺叶切除术是根本的治疗方法。
支气管迪厄拉富瓦病应纳入不明原因咯血的鉴别诊断中,若支气管镜检查发现锥形支气管内突出物,活检时应注意潜在危险。