A case of bronchial Dieulafoy disease and literature review
Authors: Shi Xiaoqian , Wang Mingdong , Wang Yifei , Zhang Wei , Zhao Xuewei , Li Bing 
Published: December 2022

Abstract
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 CTA 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).

Conclusion

BDD is rare, but may cause fatal massive hemoptysis. Bronchography 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. When the cause of hemoptysis is not clear, it should be considered in the differential diagnosis. Interventional and surgical treatment plays an important role in patients with cough accompanied by massive hemoptysis.