Zhou Pengcheng, Yu Wei, Chen Kelin, Li Xuelian, Xia Qianming
Department of Respiratory Medicine, Hospital of Chengdu University of Traditional Chinese Medicine.
Clinical Medical School, Chengdu University of Traditional Chinese Medicine, Chengdu, Sichuan province, P.R. China.
Medicine (Baltimore). 2019 Feb;98(7):e14471. doi: 10.1097/MD.0000000000014471.
Dieulafoy's lesions are characterized by the presence of a dysplastic artery in the submucosa, most frequently associated with gastrointestinal hemorrhage. They are rarely identified in the bronchial submucosa and can cause massive or fatal hemoptysis PATIENT CONCERNS:: The patient was a 62-year-old male farmer with intermittent hemoptysis of approximately 2 years duration and a definite diagnosis could not be established.
A thorax-computed tomography at our hospital revealed that the bronchus of left lower lobe was narrowed with associated local atelectasis, and lung cancer was suspected. A bronchoscopy showed a slit-like stenosis of the left lower lobe, swollen and smooth mucosa, and a significantly wider subsection carina.
A fatal hemorrhage occurred during biopsy and, rescue and resuscitation measures were immediately taken. A double-lumen endotracheal intubation was implanted and single-lung ventilation was started to maintain oxygenation. Hemoptysis completely stopped after bronchial artery embolization.
The patient eventually died of disseminative intravascular coagulation and multiple organ failure. Bronchial arteriography and subsequent autopsy confirmed Dieulafoy's disease of the bronchus.
In cases with recurrent unexplained hemoptysis, where CT chest or thoracic radiography show no abnormalities, pulmonologist should suspect a bronchial Dieulafoy's disease and avoid blindly performing bronchoscopy guided biopsy, which may result in fatal hemoptysis.
Dieulafoy病的特征是黏膜下层存在发育异常的动脉，最常与胃肠道出血相关。它们很少在支气管黏膜下层被发现，可导致大量咯血或致命性咯血。患者情况：该患者是一名62岁的男性农民，间歇性咯血约2年，未能明确诊断。
我院胸部计算机断层扫描显示左下叶支气管狭窄并伴有局部肺不张，怀疑为肺癌。支气管镜检查显示左下叶有裂隙样狭窄，黏膜肿胀且光滑，隆突处明显增宽。
活检过程中发生致命性出血，立即采取了抢救和复苏措施。植入双腔气管插管并开始单肺通气以维持氧合。支气管动脉栓塞术后咯血完全停止。
患者最终死于弥散性血管内凝血和多器官功能衰竭。支气管动脉造影及随后的尸检证实为支气管Dieulafoy病。
对于反复出现不明原因咯血且胸部CT或胸片无异常的病例，肺科医生应怀疑支气管Dieulafoy病，避免盲目进行支气管镜引导下活检，这可能导致致命性咯血。
