支气管Dieulafoy病三例并文献复习

Dieulafoy disease of the bronchus ：3 cases report with literature review

目的探讨支气管Dieulafoy病的临床特点，提高临床医生对本病的认识。方法回顾性分析2007年1月1日至2012年5月31日北京大学人民医院收治的病理确诊的3例支气管Dieulafoy病的临床资料，并通过万方数据知识服务平台、中国知网、National Center for Biotechnology Information及Ovid Technologies数据库检索2005年1月1日至2012年5月31日国内外报道支气管．肺Dieulafoy病的中英文文献。结果共检索到病例报告19篇，本组3例及文献报道的19例患者的平均年龄为（47±15）岁，多发生于成年男性（16／22），右肺（16／22）多于左肺（4／22），双肺罕见（2／22）。8例患者有吸烟史，10例有肺结核等基础病史。临床表现主要为不明原因大咯血；支气管镜检查多表现为支气管腔内小结节样突起，活检常发生大出血，甚至致命；约半数病例无法确定来源（11／22），经病理确定的异常血管多来源于支气管动脉（9／22），少数来源于肺动脉（2／22），外科切除标本病理检查是确诊依据。支气管动脉栓塞和外科手术是治疗的主要方法，但单纯栓塞后咯血常复发，局部肺叶切除术可根治。结论不明原因大咯血应与支气管Dieulafoy病相鉴别，避免盲目活检，及时行支气管动脉造影及支气管动脉栓塞术以避免致命性大咯血，肺叶切除术可根治。

Objectives To improve the understanding of bronchial Dieulafoy disease by summarizing the clinical and literature reported cases. Methods 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 ease 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. Results 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. Conclusions 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. Lobeetomy could be an alternative choice for a cure.