Yang Rui-hong, Li Jian-feng, Liu Jun, Sun Kun-kun, Cao Zhao-long, Gao Zhan-cheng
Department of Respiratory and Critical Care Medicine, Peking University People's Hospital, Beijing 100044, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2013 Aug;36(8):577-80.
To improve the understanding of bronchial Dieulafoy disease by summarizing the clinical and literature reported cases.
The clinical data of 3 patients with bronchial Dieulafoy disease diagnosed by pathology from January 1, 2007 to May 31, 2012 in our hospital was collected and summarized. The data of 19 cases from literature case report regarding bronchial Dieulafoy disease both in Chinese and English were also reviewed through databases including Wanfang Data, National Knowledge Infrastructure, National Center for Biotechnology Information and Ovid Technologics from January 1, 2005 to May 31, 2012. The clinical characteristics, diagnosis and treatment of all the 22 cases were summarized and analyzed.
The average age of the 22 cases with bronchial Dieulafoy disease was (47 ± 15) years, and the preponderance was in male adults (16/22). Right lung (16/22) was more commonly involved than the left lung (4/22), and rarely in both lungs (2/22). Eight cases had smoking history, and 10 cases had underlying diseases such as tuberculosis.Sudden onset of massive hemoptysis was a common manifestation. Massive or lethal hemorrhage was often caused by biopsy injury. The abnormality of bronchial Dieulafoy disease was usually demonstrated as nodular lesions within the lumen of the bronchus.However, It was unable to determine their originating of the anomalous arteries in half of the cases(11/22). Most anomalous arteries confirmed by pathology were branched from bronchial artery (9/22), and rarely from pulmonary artery (2/22). The definitive diagnosis was made by pathological examination.Selective bronchial artery embolization and pulmonary lobectomy were the major therapeutic strategies, but bleeding may relapse after bronchial artery embolization, and lobectomy of the lung was a cure approach.
Bronchial Dieulafoy disease should be differentiated in patients with massive and unexplained hemoptysis.It takes a very high risk for biopsy, which rarely needs to be implemented. Bronchial arteriography and selective bronchial artery embolization should be promptly carried out to avoid life-threatening hemoptysis.Lobectomy could be an alternative choice for a cure.
通过总结临床及文献报道病例,提高对支气管迪厄拉富瓦病的认识。
收集并总结2007年1月1日至2012年5月31日在我院经病理诊断的3例支气管迪厄拉富瓦病患者的临床资料。通过万方数据、中国知网、美国国立生物技术信息中心及Ovid技术等数据库,回顾2005年1月1日至2012年5月31日中英文文献报道的19例支气管迪厄拉富瓦病病例资料。对22例患者的临床特征、诊断及治疗进行总结分析。
22例支气管迪厄拉富瓦病患者平均年龄为(47±15)岁,以成年男性为主(16/22)。右肺受累(16/22)多于左肺(4/22),双肺受累少见(2/22)。8例有吸烟史,10例有结核等基础疾病。突发大量咯血是常见表现。活检损伤常导致大量或致命性出血。支气管迪厄拉富瓦病异常通常表现为支气管腔内结节样病变。然而,半数病例(11/22)无法确定异常动脉的起源。病理证实的大多数异常动脉来自支气管动脉(9/22),少数来自肺动脉(2/22)。确诊依靠病理检查。选择性支气管动脉栓塞和肺叶切除术是主要治疗策略,但支气管动脉栓塞后出血可能复发,肺叶切除术是治愈方法。
对于大量不明原因咯血患者应考虑支气管迪厄拉富瓦病。活检风险极高,很少需要进行。应及时行支气管动脉造影及选择性支气管动脉栓塞,以避免危及生命的咯血。肺叶切除术可作为治愈的替代选择。