Yang Huizhen, Liu Yuyan, Lou Jianghua, Chen Xianliang, Zhang Quncheng, Zhang Xiaoju, Herth Felix J F
Department of Respiratory and Critical Care Medicine, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, China,
Department of Comprehensive Intervention, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, China.
Respiration. 2025;104(8):597-602. doi: 10.1159/000545261. Epub 2025 Mar 21.
Bronchial Dieulafoy's disease (BDD) comprises rare vascular malformations. This study analyzes a series of BDD patients diagnosed through combined bronchial computed tomography arteriography (CTA) and bronchoscopy, addressing critical gaps in diagnostic standardization and therapeutic decision-making.
This was a retrospective review of patients who underwent CTA and bronchoscopy for mild to massive and unexplained recurrent hemoptysis in two centers during a 6-year period.
Thirty-six patients were diagnosed with BDD by bronchial CTA and bronchoscopy. Abnormal vessels were observed by CTA in all 36 patients; twisted vessels protruding into the bronchial lumen were found in 14 patients. Mucosal eminence lesions in the ipsilateral lobar/segmental bronchus were detected by white light bronchoscopy in all patients. Bronchial artery embolization (BAE) was performed in 10 patients, and endobronchial intervention was attempted in 18 patients as the initial treatment. Eight patients received application of antibiotics and hemostatic drugs, only. Recurrent hemoptysis occurred in 1 patient.
Vascular disease was the main cause of large to massive hemoptysis. CTA is a noninvasive method that could be used for first-line screening for bronchial vascular malformations. Bronchoscopic procedures could confirm the diagnosis of BDD. BAE is often the first choice, clinically, owing to the invasiveness of thoracic surgery and the patient's status. Bronchoscopic interventional therapy is an effective complement to BAE.
支气管迪厄拉富瓦病（BDD）是一种罕见的血管畸形。本研究分析了一系列通过支气管计算机断层扫描血管造影（CTA）和支气管镜检查诊断的BDD患者，填补了诊断标准化和治疗决策方面的关键空白。
这是一项对在6年期间于两个中心因轻度至大量且原因不明的反复咯血而接受CTA和支气管镜检查的患者的回顾性研究。
36例患者通过支气管CTA和支气管镜检查被诊断为BDD。所有36例患者CTA均观察到异常血管；14例患者发现扭曲血管突入支气管腔。所有患者白光支气管镜检查均发现同侧叶/段支气管黏膜隆起病变。10例患者接受了支气管动脉栓塞术（BAE），18例患者作为初始治疗尝试了支气管内介入治疗。8例患者仅接受了抗生素和止血药物治疗。1例患者出现反复咯血。
血管疾病是大量至 massive咯血的主要原因。CTA是一种可用于支气管血管畸形一线筛查的非侵入性方法。支气管镜检查可确诊BDD。由于胸外科手术的侵入性和患者状况，BAE在临床上通常是首选。支气管镜介入治疗是BAE的有效补充。
