儿童先天性肺囊性疾病69例诊疗分析

The diagnosis and surgical treatment of congenital cystic lung disease in children

目的：总结儿童先天性肺囊性疾病的诊断和治疗经验，探讨肺囊性疾病的手术治疗时机与手术方式。方法回顾性分析2004年7月至2013年7月我们收治的69例先天性肺囊性疾病患儿临床资料，男39例，女30例，年龄2d至15岁，平均年龄（1．2±0．6）岁。其中先天性肺囊肿48例，先天性肺叶气肿15例，先天性肺囊性瘤样畸形6例。肺囊肿中行囊肿摘除术8例，40例行肺叶切除术；先天性肺叶气肿15例，均行肺叶切除术；先天性肺囊性瘤样畸形6例，均行肺叶切除术。结果患儿均治愈出院，无一例死亡。术后近期并发症包括气胸5例，胸腔积液4例，乳糜胸1例，肺炎4例。随访2个月至9年，均恢复良好。结论儿童先天性肺囊性疾病表现多样，易产生压迫症状或急性呼吸窘迫，应早诊断，早治疗，手术切除是治疗的根本方法，对于有急性呼吸窘迫症状者，应急诊手术治疗。

Objetive To sum up the experience of diagnosis and surgical management for congenital cystic lung disease in children, and to explore the timing and operational modus for these diseases. Methods A retrospective analysis was done on the clinical data of 69 cases with congenital cystic lung disease and received surgical treatment in our hospital from July 2004 to July 201 3,including 39 males and 30 females with mean age （1 .2 ±0.6）years old （They ranged in age from two days to 1 5 years.）Among the 69 cases, 48 suffered from lung cyst （40 underwent lobectomy and 8 excision of the cyst）,1 5 congenital lobar emphyse-ma （all underwent lobectomy）and 6 congenital pulmonary adenomatoid malformation （all underwent seques-trectomy）.All patients received a biopsy. Results there were no operation deaths,all the patients discharge from the hospital successfully.Early postoperative complications included 5 cases with pneumothorax and 4 ca-ses with pleural effusion and 1 case with chylothorax and 4 cases with pneumonia.All cases recovered well dur-ing follow-up between 2 months and 9 years. Conclusion Congenital cystic lung disease in children has di-verse clinical manifestations,including acute respiratory distress and compression symptoms which occur very often.Early diagnosis and prompt management are required,and surgical resection,commonly lobectomy,is the main treatment for this lesion.Urgent or emergent operation is needed in symptomatic patients with acute respir-atory distress.