Dieulafoy’s disease of the bronchus: rare but potentially fatal: a case report and a review of literature

BACKGROUND: Dieulafoy’s disease of the bronchus can cause massive and even fatal hemoptysis. Even though it is rare, it should be considered by physicians all over the world. This paper reports a case of bronchial Dieulafoy’s disease and summarizes the data of similar cases reported in literature. METHODS: We report a case of bronchial Dieulafoy’s disease (BDD) in Tunisia. We also present a review of literature related to BDD from 1995 to 2022 using the PubMed, Google Scholar, web of science and Chinese National Knowledge Infrastructure Databases. Clinical characteristics, chest imaging, bronchoscopic and angiographic findings were summarized. Treatment courses were identified as well as patients’ outcome. RESULTS: We report the case of a 41-year-old man, so far in good health, presenting with massive hemoptysis. Bronchoscopy showed blood clots and a protruding lesion covered by mucosa with a white pointed cap at the entrance of the right upper lobe. Biopsies were not attempted. Embolization of bronchial artery was first realized and was not successful, with post procedure complications. Surgical intervention stopped the bleeding and pathological examination of the resected specimen confirmed Dieulafoy’s disease of the bronchus. Ninety cases of BDD were reported from 1995 to 2022. The main symptom was hemoptysis. Chest imaging findings were not specific. The diagnosis of BDD was mainly based on the bronchoscopy, branchial angiography and pathological findings or surgical specimens. Bronchoscopy findings were mostly nodular or prominent lesions (52.4%). Twenty-eight patients underwent bronchoscopic biopsies, 20 had massive bleeding and 10 died. Bronchial angiography mainly showed tortuous and dilation of bronchial artery, and the lesions were mainly located in the right bronchus. Selective bronchial artery embolization (SBAE) was performed in 32 patients and 39 patients underwent surgery. CONCLUSION: To our knowledge, this is the first case of bronchial Dieulafoy’s disease to be reported in Tunisia and North Africa. When the diagnosis is suspected, bronchoscopic biopsy should be avoided as it might lead to fatal hemorrhage. Selective bronchial artery embolization can stop the bleeding, but surgery can be required.