Recurrent hemoptysis in pediatric bronchial Dieulafoy’s disease with inferior phrenic artery supply: A case report

BACKGROUND: Bronchial Dieulafoy’s disease (BDD) is characterized by the erosion of an anomalous artery in the submucosa of the bronchus. The etiology of pediatric BDD is mainly congenital dysplasia of bronchus and pulmonary arteries, which is different from chronic inflammatory injury of the airway in adult patients. The internal thoracic artery, subclavian artery, and intercostal artery are known to be involved in the blood supply to the BDD lesion in children. CASE SUMMARY: We report a case of BDD in a 4-year-old boy with recurrent hemoptysis for one year. Selective angiography showed a dilated right bronchial artery, and anastomosis of its branches with the right lower pulmonary vascular network. Bronchoscopy showed nodular protrusion of the bronchial mucosa with a local scar. Selective embolization of the bronchial artery was performed to stop bleeding. One month after the first intervention, the symptoms of hemoptysis recurred. A computed tomography angiogram (CTA) showed another tortuous and dilated feeding artery in the right lower lung, which was an abnormal ascending branch of the inferior phrenic artery (IPA). The results of angiography were consistent with the CTA findings. The IPA was found to be another main supplying artery, which was not considered during the first intervention. Finally, the IPA was also treated by microsphere embolization combined with coil interventional closure. During the one-year follow-up, the patient never experienced hemoptysis. CONCLUSION: The supplying arteries of the bleeding lesion in children with BDD may originate from multiple different aortopulmonary collateral arteries, and the IPA should be considered to reduce missed diagnosis. CTA is a noninvasive radiological examination for the screening of suspected vessels, which shows a high coincidence with angiography, and can serve as the first choice for the diagnosis of BDD.