先天性肺囊性疾病52例

Congenital cystic lung lesions in 52 children

目的探讨儿童先天性肺囊性疾病的诊断、手术治疗方法及预后。方法回顾性分析2003年1月至2012年6月在复旦大学附属儿科医院住院手术治疗的先天性肺囊性疾病儿童52例。对患儿临床表现、病变部位、影像学、病理学、漏诊误诊、手术治疗方法和短期肺功能进行统计分析。结果52例先天性肺囊性疾病患儿中，男33例，女19例；年龄1d-159个月，中位数为3．67个月；新生儿5例。病种：支气管源性肺囊肿42例，肺隔离症6例，大叶性肺气肿3例，先天性囊性腺瘤样畸形1例。临床表现以肺部感染为主，其中61．5％的患儿首次肺部感染后确诊，21．1％的患儿在反复呼吸道感染后确诊，约17．3％的患儿无症状。病变部位以右下肺最多见，约占36．5％。影像学以多发气囊肿最多见，约占42．3％。病理结果均提示支气管源性。漏诊率为17．3％，误诊率为24．9％，其中支气管源性肺囊肿误诊率为19．2％。治疗以肺叶切除术为主，无死亡病例。结论4,JI,先天性肺囊性疾病的术前诊断主要依靠影像学检查，临床表现有助于早期识别，术前易漏诊误诊，术后病理有助于确诊，手术疗效肯定，预后好。

Objective To investigate the diagnosis, operation and outcomes of congenital cystic lung lesions in children. Methods Fifty-two children with congenital cystic lung lesions were collected. The clinical data s were ana- lyzed including manifestations, lesion characteristics, imaging, histopathology, diagnosis, surgical treatment methods and short-term pulmonary functions. Results There were 33 male and 19 female,aged from 1 day to 159 months,their median age was 3.67 months. Five cases were neonates. There were 42 cases of bronchogenic cyst, 6 cases of pulmonary sequestration ,3 cases of congenital lobar emphysema and 1 case of congenital cystic adenomatoid malformation. The cases commonly manifested by lung infections. Of 52 cases,61.5% were diagnosed after the first onset of lung infection, 21.1% for repeated lung infections and 17.3% with no symptom. Right lower lung was the most common lesion site and the cases accounted about for 36.5%. Multiple gas cysts were the most common imaging findings accounting about for 42.3%. The pathologic examination of all cases showed bronchogenic cysts. The missed diagnosis rate was 17.3%. The misdiagnosis rate was 24.9% , which of bronchogenic cyst cases was 19.2%. All cases received the lobectomy without death. Conclusions The preoperative diagnosis of congenital cystic lung lesions is mostly based on imaging findings. Clinical manifestations are useful for identifying early the lesions. The preoperative diagnosis may be easily missed or incorrectly done. Pathological findings contribute to make a definite diagnosis. The surgery effect is satisfactory and the outcome is good.