Dieulafoy’s disease has been first reported in 1898 by Georges Dieulafoy usually affecting the digestive tract (1). The bronchial location of this disease has been first reported by Sweerts et al. in 1995 (2). It’s an extremely rare affection which may manifest by massive hemoptysis. Over the past decade, cases of the disease have been increasingly outlined. A recent systematic review of the literature published by Qian et al. collected 73 cases from 1995 to 2019 (3). To our knowledge, this case would be the first one published in Tunisia and North Africa.
The cause of the disease is still unknown. Theories vary from congenital vascular malformations to bronchial injury secondary to previous infections (2). Parrot et al. (4) suggested a possible association with inflammatory lesions in tuberculosis or stretching and dilation of the bronchial artery. Advanced age and tobacco smoking have been implicated in the increase of bleeding-related complications (5). However, the disorder may affect people at every age especially middle-aged adults (3), and also non-smokers.
Clinical manifestations are non-specific, but the most common one is recurrent hemoptysis. Massive and even fatal hemoptysis may occur especially while performing bronchoscopy guided biopsy (6). Other symptoms such as a cough, chest pain, infection or respiratory failure can be reported by patients (7).
Establishing the diagnosis of Dieulafoy’s disease can be quite difficult. An exhaustive evaluation was lead with chest X-rays, computed tomography (CT) scans, bronchoscopies, biopsies and bronchial angiographies in historical cases.
In Dieulafoy’s disease of the bronchus, chest X-rays and computed tomography (CT) scans are rarely contributive to the diagnosis. They show mostly manifestations of an intrapulmonary hemorrhage with ground glass opacities (3). The relevance of this exam is its contribution to excluding other lung diseases causing the bleeding. Endobronchial nodes have been identified by chest CT in some cases (8). Multi-slice CT angiography can show a tortuous and dilated bronchial artery (9). Due to lack of specificity and sensitivity, most authors agree that chest X-rays and CT scans are not the best modality by which to diagnose Dieulafoy’s disease.
Bronchoscopy mainly show massive endobronchial hemorrhage, which may be accompanied by blood clot formation. A mucosal protrusion is frequently observed in the site of the bleeding bronchus, which diameter can be only a few millimeters (3, 7). The mucosa covering the protruding surface looks like a “white cap”, without a pulsating sensation. The surrounding mucosa can be normal or congested. In most cases, the abnormal lesions were located in the right bronchus (3).
These bronchoscopic findings in Dieulafoy’s disease of the bronchus are not diagnostic since the abnormal vessel is usually pinpoint mucosal defect surrounded by normal-looking mucosa. Moreover, a small lesion can be undetected due to pooling of blood or clots within bronchial lumen. Among 74 cases reported by Qian et al. (3), biopsies were attempted for 19 patients whom presented a nodular lesion without a typical vascular lesion. It was primarily suspected to be an endobronchial mass or carcinoid tumor. Seventeen patients had bleeding after the biopsy, and six deaths occurred due to massive hemorrhage. Bronchial biopsies in such diseases entail the risk of triggering fatal hemoptysis. Since 2014, with a better understanding of Dieulafoy’s disease, biopsies have been avoided for nodules suspected to be caused by Dieulafoy’s disease (10), which has reduced the risks of massive hemorrhage. In 2010, Guiroli et al. (11) have demonstrated the clinical utility of endobronchial ultrasound EBUS) in the evaluation of bronchial alteration suspicious of Dieulafoy’s lesion. This technique can be helpful to clarify the nature of the nodular lesion and contributes to the diagnosis, avoiding potentially disastrous interventions (12). The major manifestation is a fluid echo-free zone in the submucosal lesion. The Doppler mode can be used to detect blood flow. However, convex probe EBUS cannot reach the upper lobe bronchus nor segmental bronchus. Radial probe EBUS can be used instead but it has no doppler mode and cannot determine blood flow within the lesion.
Bronchial angiography has a twofold benefit. It has a role in the diagnosis as it can show a rich blood supply to the corresponding site of the lesion; a deformed, tortuous and dilated artery with signs of bleeding (3). When detected, these abnormalities can indicate a selective bronchial embolization, which has an important therapeutic value. However, lesions of the arteries may not be visualized, as it was the case for 2 patients in the systematic review of Qian et al. (3) and for our patient as well. The bronchial arteries usually originate from the proximal descending thoracic aorta. Arteries that originate elsewhere in the aorta or from other vasculature are termed ectopic (13). Right bronchial arteries occasionally originate from the aorta but more commonly share their origin with another artery, usually an intercostal artery. Choi et al. (14) evaluated in their study the spectrum of variations in bronchial artery and among ectopic origins, concavity of the aortic arch was the most common.
To make a definite diagnosis, many researchers consider that pathological examination of biopsies, surgical or autopsy specimens is required. However, there are no uniform diagnostic criteria and due to risks involved, the need for pathological diagnosis remains controversial. The pathological exam usually shows an arterial malformation in the bronchial submucosa. The tortuous, dilated and deformed artery forms small nodules coated with bronchial mucosa and protruding from the bronchial lumen (4, 15). Diagnosis is confirmed when a dysplastic artery is identified in the bleeding territory without evidence of other underlying lung disease, vasculitic changes or neoplasm.
Treatment options include conservative internal medication, surgical lung resection, selective bronchial artery embolization (SBAE) and bronchoscopic ablation. Conservative treatment and the use of hemostatic agents is rarely efficient in stopping the hemorrhage. Niu al. (16) have reported that pituitrin and thrombin may occasionally have good therapeutic effect in some cases of infantile bronchial Dieulafoy’s disease. Bronchoscopic ablation has been tried in a minority of cases (3) and is not without perils. Mediastinitis, esophageal injuries, and broncho esophageal fistulas are all potential complications (17). Selective bronchial artery embolization is often performed as a first-line treatment and is efficient in most patients (7). But hemoptysis may reoccur after the procedure. The study of Qian et al. (3) revealed that 52.6% patients who underwent SBAE required secondarily lobectomy. One patient had undergone SBAE seven times due to recurrent hemorrhage (18). Up to date, surgery has been the main definitive treatment with a success rate of nearly 100% in all reports (3). Recurrence of hemoptysis in unlikely after resection of the diseased lung lobe.