支气管Dieulafoy病一例报告并文献复习

目的提高对支气管Dieulafoy病的认识。方法对1例临床确诊为支气管Dieulafoy病患者的临床资料进行分析，并结合文献复习。结果支气管Dieulafoy病是一种罕见病，以支气管黏膜下畸形的动脉破裂出血为病理特征，其病因及发病机制尚不清楚，可能与支气管肺动脉先天发育异常、气道慢性炎症或损伤有关。临床表现主要为突发大咯血或支气管腔内病灶活检后发生致命性大出血。支气管肺动脉造影是术前诊断的主要手段，手术或尸检标本的组织学检查可以确诊。支气管动脉栓塞可以作为一种治疗方法，但有复发和失败的可能，肺叶切除术可以达到根治目的。结论遇到原因不明的大咯血应注意排除支气管Dieulafoy病的可能；支气管镜检查若发现管腔内突起样病灶，不要盲目活检。

Dieulafoy's disease of the bronchus: a case report and review of the literature

Objective To describe the characteristics of Dieulafoy's disease of the bronchus.Methods One patient with Dieulafoy's disease of bronchus was described and relevant literatures were reviewed.Results 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.Conclusions 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.