作　　者： (曾森强）; (樊慧峰）; (卢根）; (张东伟）; (谢小斐）; (黄莉）; (杨迪元）; (张明杰）;

机构地区： 广州医科大学附属广州市妇女儿童医疗中心呼吸科,510120

出　　处： 《中华实用儿科临床杂志》 2017年第16期1262-1266,共5页

摘　　要： 目的分析儿童支气管桥（BB）畸形的临床和解剖形态特征，探讨其诊断策略、治疗方式及危险因素。方法对2010年5月至2016年10月广州市妇女儿童医疗中心收治的23例BB患儿的临床特征、影像学改变、治疗方式及预后进行回顾性总结和分析。结果1．本组患儿男15例，女8例；发病年龄0～4岁（发病年龄中位数为3．17个月）。2．临床表现主要为咳嗽（23／23例，100．0％）、反复喘息（20／23例，87．0％）、发绀（8／23例，34．8％）、喂养困难／体质量增长缓慢（6／23例，24．6％）等。3．根据Wells法分型Ⅰ型17例，Ⅱ型4例，另2例为前支气管桥。Ⅰ型和Ⅱ型病例中各有2例解剖形态结构不典型。4．合并支气管狭窄21例，以左主支气管起始部合并BB狭窄最为多见（5例），16例行支气管镜检查示支气管软化5例。合并心血管畸形16例（复合畸形11例），其中以左肺动脉吊带（SLPA）最常见（12例）。5．合并SLPA12例中9例行肺动脉吊带矫治术，手术年龄为1个月～8岁，患儿术后4～17个月喘息等呼吸道症状逐步缓解或消失，余2例死亡，1例失访。结论BB通常伴支气管狭窄／软化及心血管等其他器官畸形。临床症状严重度主要取决于是否并呼吸道狭窄／软化及其程度；需要联合多种影像检查才能做出完整的诊断评估。合并单纯SLPA患儿手术效果良好。 Objective To analyze the clinical and anatomic characteristics of bridging bronchus （BB） malfor- mation in children, and to explore its diagnostic strategy, treatment and risk factors. Methods A retrospective study was performed in 23 pediatric patients with BB who were admitted to Guangzhou Women and Children＇s Medical Center form May 2010 to October 2016. All their clinical features ,imaging examination,treatment and prognosis were reviewed and analyzed. Results （ 1 ） Among the 23 patients with BB, 15 were males and 8 were females. The age range of onset was 0 to 4 years old with a median age of 3.17 months. （2）The main clinical manifestations were cough （23/23 cases, 100.0% ）, recurrent wheezing （ 20/23 cases, 87.0% ）, cyanosis （ 8/23 cases, 34.8% ） and feeding difficulty/slow body weight growth （6/23 cases,24.6% ）. （3） There were 17 cases of type I and 4 cases of type I1 according to Wells, and the other 2 cases were anterior BB. There were 2 cases with atypical anatomical morphology in type I and type I1 respectively. （4） Twenty - one cases were associated with bronchial stenosis in which the origin of left main bronchus complicated with BB stenosis was the most common（5 cases）. Bronchoscopy revealed bronchomalacia in 5 of the 16 patients. Sixteen patients associated with cardiac or vascular anomalies, 11 of whom were compound anomalies, and the most common type was sling left pulmonary artery （SLPA） （ 12 cases）. （5） Nine of 12 patients with SLPA re- ceived surgical management, ages ranging from 1 month to 8 years old. The respiratory symptoms remitted gradually or disappeared from 4 to 17 months after surgical management. Two died and one has been lost to follow - up among the remaining 3 patients with SLPA. Conclusions BB is a rare tracheobronchial malformation that is often associated with stenosis or bronchomalacia, and cardiac or vascular anomalies. The severity of the disease mainly depends on whether there is airway stenosis or malacia and its exte

关 键 词： 支气管桥 儿童 心血管畸形 支气管狭窄