Lai Lingyu, Lu Yong, Xi Zhimin, Liu Fang, Qian Liling, Wang Libo, Zhao Quming
Department of General Pediatrics, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.
Respiratory Department, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.
Transl Pediatr. 2023 Jan 31;12(1):79-85. doi: 10.21037/tp-22-294. Epub 2023 Jan 11.
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.
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.
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.
支气管迪厄拉富瓦病（BDD）是一种血管畸形，其特征是支气管黏膜下层存在发育异常的动脉。该病在儿童中非常罕见，但由于危及生命的咯血可能致命。
一名8岁男孩和一名6岁女孩因反复出现中度至大量咯血被转诊至我院。支气管镜检查发现两名患者均有腔内突出且有出血倾向。计算机断层血管造影（CTA）显示一名患者有异常支气管动脉，另一名患者有一个有对比增强的小腔内结节。两名患者的支气管动脉造影均检测到与病变部位对应的支气管动脉增粗和支气管-肺瘘。根据影像学检查结果，确诊为BDD。随后的支气管动脉栓塞术（BAE）成功，在15至18个月的随访期间未观察到咯血复发。
我们的病例强调了在儿童咯血和支气管内突出的情况下考虑BDD的重要性。支气管动脉造影在诊断中起关键作用，尤其是在CTA未显示血管畸形的病例中。由于活检是禁忌的，早期识别至关重要。BAE可能是改善BDD患儿预后的一种合适治疗方法。
