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目的探讨先天性气管支气管畸形的临床特点,以提高对该病的认识,减少误诊漏诊。方法2004年2月至2008年6月在温州育英儿童医院呼吸科诊断为先天性气管支气管畸形的患儿46例,其中男29例,女17例。分析其临床、影像学、纤维支气管镜等特点。结果46例患儿年龄3d至14岁,其中<3个月8例;3个月至1岁20例,>1～3岁15例,>3～14岁3例。均诊断为先天性气管支气管畸形,其中气管支气管软化10例,支气管起源异常15例,支气管缺如5例,气管支气管狭窄21例。复合畸形10例。发病年龄3d至14岁,均表现为反复咳嗽、反复或持续喘息,喘息多在吃奶、哭闹或运动后加重。X线胸片和CT平扫可见弥漫性或局限性透明度增强或肺不张,10例气管支气管软化通过纤维支气管镜确诊,螺旋CT三维重建诊断气管支气管狭窄21例,诊断支气管起源异常14例。结论(1)对有下列临床特点者应该考虑到气管支气管畸形的可能:持续咳嗽经常规检查不能明确病因;反复或持续喘息,经抗炎或支气管扩张剂治疗无效;反复或持续喉鸣;X线胸片同一部位反复或持续肺炎或肺不张;X线胸片提示局限性肺气肿、肺不张、纵隔移位而原因不明。(2)纤支镜对先天性气管支气管软化的诊断具有重要价值。(3)螺旋CT三维重建对诊断气管支气管起源异常和狭窄的诊断价值和纤支镜相当,尤其适用于年幼儿和危重儿。
Objective To explore the clinical features of congenital tracheobronchial malformations to improve the understanding of the disease and reduce the misdiagnosis and missed diagnosis. Methods From February 2004 to June 2008, 46 children with congenital tracheal bronchial deformity were diagnosed in the Respiratory Department of Yuyin Children’s Hospital of Wenzhou, including 29 males and 17 females. Analysis of its clinical features, imaging, bronchoscopy and other characteristics. Results 46 cases of children aged 3d to 14 years, of which <3 months in 8 cases; 3 months to 1 year old in 20 cases,> 1 to 3 years old in 15 cases,> 3 to 14 years old in 3 cases. All were diagnosed as congenital tracheobronchial malformations, including 10 cases of tracheobronchial softening, 15 cases of abnormal bronchial origin, bronchial absence in 5 cases, bronchial stenosis in 21 cases. Compound deformity in 10 cases. The age of onset of 3d to 14 years old, are manifested as repeated cough, repeated or sustained wheezing, wheezing and nurse more, worse or worse after exercise. X-ray and CT scan showed diffuse or limited transparency enhancement or atelectasis, 10 cases of tracheobronchial softening confirmed by fiberoptic bronchoscopy, spiral CT three-dimensional reconstruction of tracheobronchial stenosis in 21 cases, the diagnosis of bronchial origin in 14 cases. Conclusions (1) The possibility of tracheobronchial deformity should be taken into account in patients with the following clinical features: persistent cough can not be clearly diagnosed by routine examination; repeated or persistent wheezing, invalid by anti-inflammatory or bronchodilator therapy; repeated or persistent throat; X Linear chest radiographs of the same site repeated or persistent pneumonia or atelectasis; X-ray showed limited emphysema, atelectasis, mediastinal shift and the reasons for unknown. (2) Bronchoscopy is valuable for the diagnosis of congenital tracheobronchial softening. (3) Three-dimensional reconstruction of spiral CT diagnosis of tracheobronchial origin and stenosis of the diagnostic value and bronchoscopy, especially for young children and critically ill children.