Department of Pulmonary and Critical Care Medicine, Shanghai Fourth People's Hospital, School of Medicine, Tongji University, No. 1279 Sanmen Road, Shanghai, 200434, China.
Department of Thoracic Surgery, Shanghai Fourth People's Hospital, School of Medicine, Tongji University, No. 1279 Sanmen Road, Shanghai, 200434, China.
J Cardiothorac Surg. 2023 Jun 27;18(1):197. doi: 10.1186/s13019-023-02279-1.
Bronchial Dieulafoy's disease (BDD) is a rare disease that causes massive hemoptysis. This paper reports a case of BDD treated surgically. At the same time, we summarize the data of BDD patients reported in domestic and foreign literature to improve the understanding, diagnosis and treatment of this disease.
A case of BDD with hemoptysis during bronchoscopy was reported. In addition, we searched for "bronchial Dieulafoy disease" through Pubmed, Web of Science, CNKI and Wanfang databases, covering the literature related to BDD that was definitely diagnosed or highly suspected from January 1995 to December 2021, and summarized the clinical characteristics, chest imaging, bronchoscopic manifestations, angiographic characteristics, pathological characteristics, treatment and outcome of patients.
The patient was a 68 year old male. Tracheoscopy revealed nodular and mass like changes in the basal segment of the left lower lobe, which appeared massive hemorrhage when touching the surface. The computed tomography angiophy of the bronchial artery confirmed that the branches of the left bronchial artery were tortuous and dilated, and then the left lower lobe of the lung was resected. During the operation, 3 thick tortuous nutrient artery vessels were sent out from the descending aorta, and 1 thick tortuous nutrient artery was sent out from the autonomic arch. All of them were ligated and cut. The pathology after the operation was in accordance with BDD; The patient did not have hemoptysis after discharge and is still under follow-up. The database identified 65 articles from January 1995 to December 2021. After removing repeated reports, meetings, incomplete information and nursing literature, 60 articles were included to report 88 cases of BDD. BDD can occur at all ages, with a male to female ratio of about 1.6:1. It mainly starts with hemoptysis, and can also be seen due to cough, infection, and respiratory failure; Inflammatory changes such as pulmonary patch shadow, exudation shadow and ground glass shadow of pulmonary hemorrhage were more common in chest imaging; The diagnosis of BDD is mainly based on the bronchoscopy, bronchial angiography and pathological findings of surgical or autopsy specimens. Bronchoscopic findings were mostly non pulsating, smooth nodular or mucosal processes. Bronchial angiography mainly showed tortuous dilatation of bronchial artery, and the lesions were mainly located in the right bronchus, more from the bronchial artery; Diagnosis depends on pathology, showing submucosal expansion of bronchus or abnormal artery rupture and bleeding; 54 cases underwent selective bronchial artery embolization, 39 cases underwent pulmonary lobectomy, 66 cases improved, and 10 cases died (all of them were caused by massive hemorrhage during bronchoscopic biopsy).
BDD is rare, but may cause fatal massive hemoptysis. Bronchial angiography is considered to be an effective method to diagnose BDD. Since pathological biopsy may lead to fatal bleeding, the necessity of pathological diagnosis remains controversial. Interventional and surgical treatment plays an important role in patients with cough accompanied by massive hemoptysis.
支气管 Dieulafoy 病（BDD）是一种罕见的疾病，可导致大量咯血。本文报告了一例经手术治疗的 BDD 病例。同时，我们总结了国内外文献中报告的 BDD 患者的数据，以提高对该病的认识、诊断和治疗水平。
报告一例支气管镜下出现咯血的 BDD 病例。此外，我们通过 Pubmed、Web of Science、CNKI 和万方数据库检索“支气管 Dieulafoy 病”，检索时间为 1995 年 1 月至 2021 年 12 月，检索内容为明确诊断或高度疑似的 BDD 相关文献，总结患者的临床特征、胸部影像学表现、支气管镜表现、血管造影特征、病理特征、治疗和转归。
患者为 68 岁男性。支气管镜检查发现左下叶基底段呈结节状和肿块状改变，触诊表面时出现大量出血。支气管动脉 CT 血管造影证实左支气管动脉分支迂曲扩张，随后行左肺下叶切除术。术中从降主动脉发出 3 根厚而迂曲的营养动脉血管，从自主弓发出 1 根厚而迂曲的营养动脉血管，均予以结扎切断。术后病理符合 BDD；患者出院后未再出现咯血，仍在随访中。数据库共识别出 1995 年 1 月至 2021 年 12 月的 65 篇文章，去除重复报告、会议、信息不完整和护理文献后，纳入 60 篇文章，共报告 88 例 BDD。BDD 可发生于各年龄段，男女比例约为 1.6:1。主要以咯血起病，也可因咳嗽、感染、呼吸衰竭等就诊；胸部影像学多表现为肺部斑片状影、渗出影、磨玻璃影等出血性炎症改变；BDD 的诊断主要基于支气管镜、支气管动脉造影和手术或尸检标本的病理发现。支气管镜下表现多为无搏动、光滑的结节状或黏膜样病变。支气管动脉造影主要表现为支气管动脉迂曲扩张，病变主要位于右支气管，多来自支气管动脉；诊断依赖于病理，表现为支气管黏膜下扩张或异常动脉破裂出血；54 例行选择性支气管动脉栓塞术，39 例行肺叶切除术，66 例患者好转，10 例死亡（均因支气管镜活检时发生致命性大出血）。
BDD 罕见，但可引起致命性大咯血。支气管动脉造影被认为是诊断 BDD 的有效方法。由于病理活检可能导致致命性出血，因此病理诊断的必要性仍存在争议。介入和手术治疗在伴有大量咯血的咳嗽患者中发挥着重要作用。
