支气管Dieulafoy病1例报告并文献复习

Dieulafoy s disease of bronchus： a case report and review of liferature

目的提高对支气管Dieulafoy病的认识及支气管腔内结节的鉴别。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，并结合文献复习支气管Dieulafoy病的临床特点、诊断要点及相关治疗。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，可能与支气管一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结节样病灶活检后发生致命性大出血。支气管动脉造影为诊断的主要手段，对行支气管镜检查发现管腔内结节行支气管镜腔内超声（endobronchialultrasound，EBUS）或窄带成像（narrow-bandimaging，NBI）检查有助于鉴别管腔内结节的性质。病理检查发现畸形血管是确诊依据。支气管动脉栓塞和外科手术是治疗的主要方法，但支气管动脉栓塞有复发和失败的可能，而肺叶切除术可达到根治的目的。结论原因不明的反复咯血应考虑支气管Dieulafoy病的可能，及时行支气管动脉造影及支气管动脉栓塞术可避免致命性大出血；支气管镜检查下发现管腔内结节样病灶，行EBUS或NBI检查有助于鉴别管腔内结节的性质，避免盲目活检。

Objective To improve the understanding of Dieulafoy＇s disease of bronchus and the identification of the intraluminal nodules by summarizing the clinical and literature reported cases. Methods The clinical data of one patient with Dieulafoyls disease of bronchus were analyzed,the clinical features,diagnosis, and treatment of Dieulafoy＇ s disease of bronchus were discussed combining with literatures. Results Dieulafoyts disease of bronchus is a rare disease pathologically characterized by rupture and hemorrhage of abnormal artery in bronchial suhmucosa. The etiology and pathogenesis are still unknown and studies show that bronchial pulmonary congenital dysplasia, chronic airway inflammation or infury may he associated. The common clinical manifestation is unexplained haemoptysis or fatal haemorrhage when taking a biopsy during bronchoscopy. Bronchial arteriography is the primary diagnostic method. Endobronchial ultrasound （EBUS） or narrow-band imaging （NBI） is helpful to identify the intraluminal nodules found during bronchoscopy. The definite diagnosis is based on abnormal blood vessels by pathological examination. Bronchial artery embolization and surgery are the main treatment, but there is a risk of the recurrence and the possibility of failure when making bronchial artery embolization, while radical lobeetomy can achieve the purpose. Conclusions For patients with uhexplained recurrent hemoptysis,Dieulafoyrs disease of bronchus should be considered. Bronchial arteriography and bronchial artery embolization should be made in time to avoid the fatal hemoptysis. EBUS or NBI is helpful to identify the intraluminal nodules found during bronehoscopy and to avoid blind biopsy.