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目的探讨婴幼儿期发病的先天性肺腺瘤样畸形(CCAM)患儿的临床特点。方法回顾分析1997~2004年在天津市儿童医院就诊的7例CCAM患儿临床表现、影像学改变及诊断过程。结果7例患儿产前检查和产后随访均不完整,临床表现均以咳嗽、喘憋等急性呼吸窘迫症状为主,听诊均有喘鸣音,3例伴有胸廓畸形;影像学改变包括单侧肺野透过度增加、纵隔摆动、肺内异常肺纹理等,CT表现为多发囊性改变。经手术及术后病理证实为CCAM。入院前均被误诊。结论对以急性呼吸窘迫起病,胸部透视或摄片为单侧气肿表现的患儿,应分析临床资料并行CT检查,以便早期发现CCAM。
Objective To investigate the clinical features of children with congenital lung adenomatous deformity (CCAM) in infancy. Methods The clinical manifestations, imaging changes and diagnosis of 7 CCAM children admitted to Children ’s Hospital of Tianjin from 1997 to 2004 were retrospectively analyzed. Results The prenatal examination and postnatal follow-up of 7 cases were incomplete. The clinical manifestations were mainly acute respiratory distress symptoms such as cough, wheezing and so on. All of them had wheeze in auscultation and thoracic deformity in 3 cases. The imaging changes included single Side of the lung field through the increase, mediastinal swing, abnormal lung lung texture, CT showed multiple cystic changes. Surgical and postoperative pathology confirmed as CCAM. All patients were misdiagnosed before admission. Conclusion In children with onset of acute respiratory distress, chest fluoroscopy or radiographs of unilateral emphysema, clinical data should be analyzed in parallel with CT for the early detection of CCAM.