Bronchial Dieulafoy’s disease: a retrospective analysis of 73 cases

BACKGROUND: Bronchial Dieulafoy’s disease (BDD) is a rare disease that is known to be a cause of hemorrhage. The characteristics of this disease are still unknown. The present study describes the disorder based on a review of the world’s literature, emphasizing the diagnostic and therapeutic views. METHODS: A comprehensive research of BDD of the PubMed, Google Scholar, and Web of Science databases was performed. The following data were collected: patient characteristics; chest imaging, bronchoscopy, vascular angiography, and histopathologic examination findings; and treatment rendered. RESULTS: 73 cases of BDD have been reported from 1995 to 2019. Most of the cases occurred in Asia (52.1%), followed by Europe (31.5%). Chest imaging findings were non-specific. The main bronchoscopy finding was a nodular or protruding lesion (60.9%). 19 patients underwent bronchoscopic biopsies, 17 had bleeding, and 6 died. Four patients were successfully shown to have vascular malformations under mucosal protrusion by endobronchial ultrasound scan (EBUS). Vascular angiography mainly showed tortuous, dilated bronchial arteries. Vascular angiography mainly showed tortuous, dilated bronchial arteries. The arterial supply was mainly provided by bronchial arteries (48 cases) and the pulmonary circulation (4 cases). The lesions were mainly located in the right bronchus (53 cases). Selective bronchial artery embolization (BAE) was attempted in 38 patients and 20 patients underwent lobectomies. Emergency resection was performed in 15 patients, all of whom survived and had no recurrent hemoptysis. CONCLUSIONS: Massive hemoptysis was the common manifestation of BDD. Vascular angiography and EBUS is a very useful examination before biopsy. BAE may be used in stable patients, or patients who cannot tolerate surgery, while surgical resection should be considered in patients who are unstable, patients with uncontrolled hemoptysis, or following BAE failure. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12890-019-0863-1) contains supplementary material, which is available to authorized users.