目的:支气管Dieulafoy病(BDD)是引起大咯血的罕见疾病。本文报告1例BDD手术治疗。同时,总结国内外文献报道的BDD患者资料,诊断和治疗这种疾病。
方法:报告1例BDD在支气管镜检查中出现咯血。此外,我们通过Pubmed搜索“支气管Dieulafoy病”,WebofScience,CNKI和万方数据库,涵盖了1995年1月至2021年12月明确诊断或高度怀疑的BDD相关文献,并总结了临床特征,胸部成像,支气管镜表现,血管造影特征,病理特征,患者的治疗和结果。
结果:患者为68岁男性。气管镜检查显示左下叶基底段有结节状和肿块状改变,接触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曲折扩张,然后切除了左下叶。在操作过程中,3个粗大的曲折营养动脉血管从降主动脉发出,从自主神经弓发出1条弯曲的营养动脉。所有这些都被结扎和切割。术后病理符合BDD;患者出院后无咯血,仍在随访中。该数据库确定了1995年1月至2021年12月的65篇文章。删除重复报告后,会议,不完整的信息和护理文献,纳入60篇文章报告88例BDD。BDD可以发生在所有年龄段,男女比例约为1.6:1。主要从咯血开始,也可以看到由于咳嗽,感染,和呼吸衰竭;炎症变化,如肺片阴影,肺出血的渗出性阴影和磨玻璃影多见于胸部影像学,支气管血管造影和手术或尸检标本的病理结果。支气管镜检查结果大多无脉动,光滑结节或粘膜突起。支气管造影主要表现为支气管动脉曲折扩张,病变主要位于右支气管,更多来自支气管动脉;诊断取决于病理,支气管粘膜下扩张或异常动脉破裂出血;54例选择性支气管动脉栓塞,39例行肺叶切除术,66例好转,死亡10例(均为支气管镜活检术中大出血)。
结论:BDD很少见,但可能会导致致命的大咯血.支气管血管造影被认为是诊断BDD的有效方法。因为病理活检可能导致致命的出血,病理诊断的必要性仍存在争议。在咳嗽伴有大咯血的患者中,介入和手术治疗起着重要作用。
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.