Pediatric bronchial Dieulafoy's disease with bronchial artery embolization: two case reports

Abstract

Background: Bronchial Dieulafoy's disease (BDD) is a vascular malformation characterized by the presence of a dysplastic artery in the bronchial submucosa. It is very rare in children but potentially fatal due to life-threatening hemoptysis.

Case description: An 8-year-old boy and a 6-year-old girl were referred to our hospital with recurrent moderate to massive hemoptysis. Intraluminal protrusions with a tendency to bleed were found by bronchoscopy in both patients. Computed tomography angiography (CTA) revealed an abnormal bronchial artery in one patient and a small intraluminal nodule with contrast enhancement in the other. An enlarged bronchial artery and bronchial-to-pulmonary fistulae corresponding to the lesion site were detected by bronchial arteriography in both patients. Based on the radiological findings, the diagnosis of BDD was established. Subsequent bronchial artery embolization (BAE) was successful, and no recurrence of hemoptysis was observed during the 15- to 18-month follow-up.

Conclusions: Our cases highlighted the importance of considering BDD in the context of hemoptysis and endobronchial protrusion in children. Bronchial arteriography plays a critical role in diagnosis, especially in cases where CTA does not reveal vascular malformations. Early identification is essential as biopsy is contraindicated. BAE may be an appropriate treatment to improve the prognosis of children with BDD.

Keywords: Bronchial Dieulafoy’s disease (BDD); bronchial artery embolization (BAE); case report; children; hemoptysis.

Figure 1

Timeline illustrating the diagnosis and treatment process of the patients. *, intraluminal protrusions with tendency for bleeding were found by bronchoscopy in both patients; **, enlarged bronchial artery and bronchial to pulmonary fistula corresponding to the lesion site were detected by bronchial arteriography in both patients. CT, computed tomography; CTA, computed tomography angiography; BDD, bronchial Dieulafoy’s disease; BAE, bronchial artery embolization.

Figure 2

Imaging and bronchoscopic features of case 1. (A) CT demonstrated ground-glass opacities (arrow) in the right lower lobe. (B) CTA revealed a tortuous and enlarged right bronchial artery (arrow) arising from the thoracic aorta. (C) Bronchoscopy revealed two nodules with white caps (arrows) at the anterior basal segment of the right lower lobe. (D) Bronchial arteriography showed an enlarged and tortuous right bronchial artery (arrowhead). The visibility of the right inferior pulmonary artery (arrow) indicated the presence of the bronchial artery to pulmonary artery shunt. (E) The distal right bronchial artery was completely embolized (arrow) and the right inferior pulmonary artery was not visualized. (F) The lesions had all disappeared (arrow) one month after embolization. CT, computed tomography; CTA, computed tomography angiography.

Figure 3

Imaging and bronchoscopic features of case 2. (A) CT demonstrated ground-glass opacities (arrow) in the middle and lower lobe of the right lung. (B) CTA revealed a small protrusion with contrast enhancement (arrow) at the right bronchus. (C) Bronchoscopy revealed a wedged protrusion with a pointed cap (arrow) arising from the lateral wall of the right bronchus intermedius. (D) Bronchial arteriography showed an enlarged and tortuous right bronchial artery (arrowhead). The visibility of the right inferior pulmonary artery (arrow) indicated the presence of the bronchial artery to pulmonary artery shunt. (E) The distal right bronchial artery was completely embolized (arrow) and the right inferior pulmonary artery was not visualized. (F) Only a slight elevation of the mucosa (arrow) was seen at the site of the former lesion seven days after embolization. CT, computed tomography; CTA, computed tomography angiography.