PDF(Pubmed)
Abstract：
Bronchial Dieulafoy\'s disease (BDD), remains poorly understood, with only 88 cases reported globally. Herein, we present the largest case series (n = 7) from a single centre, between 2017 and 2023, retrospectively reviewed, detailing clinical presentations, diagnoses, management and up to 4-year follow-up outcomes. Diagnosis relied on characteristic lesions detected through white light bronchoscopy with or without endobronchial ultrasound (EBUS) or narrow band imaging (NBI), along with computed tomography (CT) scans or bronchial angiography. Identification of aberrant vessels beneath lesions and bronchoscopy details were documented. Treatment modalities and follow-up outcomes until December 2023 were noted. All patients were non-smokers. Review of imaging findings by an experienced radiologist was crucial in suspected cases due to risk of bleeding and often unconclusive results from biopsy. Management of BDD varied, with six patients undergoing bronchial artery embolization (BAE) and one requiring lobectomy; four patients received additional endobronchial therapy, one died due to malignancy, none experienced recurrence of haemoptysis. Identifying patients with large volume haemoptysis disproportionate to parenchymal disease in CT scans is important. A bronchoscopic surveillance is crucial to avoid biopsy; it can be confirmed using EBUS of NBI. While no established guidelines exist, BAE and endobronchial therapy emerge as valuable interventions, with surgical resection reserved for recurrent cases.
摘要：
支气管Dieulafoy病（BDD），仍然知之甚少，全球仅报告了88例病例。在这里，我们从一个中心给出最大的案例系列(n=7)，2017年至2023年，回顾性审查，详细的临床表现，诊断,管理和长达4年的随访结果。诊断依赖于通过有或没有支气管内超声（EBUS）或窄带成像（NBI）的白光支气管镜检查检测到的特征性病变，以及计算机断层扫描（CT）扫描或支气管血管造影。记录病变下异常血管的鉴定和支气管镜检查细节。记录了直到2023年12月的治疗方式和随访结果。所有患者均为非吸烟者。在疑似病例中，有经验的放射科医生的影像学检查结果至关重要，因为有出血的风险，而且活检结果通常不确定。BDD的管理多种多样，有6例患者接受支气管动脉栓塞（BAE），1例需要肺叶切除术；4例患者接受了额外的支气管内治疗，一个人死于恶性肿瘤,没有人出现咯血复发。在CT扫描中识别大量咯血与实质疾病不成比例的患者很重要。支气管镜监测对于避免活检至关重要；可以使用NBI的EBUS进行确认。虽然没有既定的指导方针，BAE和支气管内治疗成为有价值的干预措施，手术切除保留用于复发病例。
