70例婴幼儿先天性肺囊腺瘤样畸形的围术期分析

The analysis for surgical treatment of congenital cystic adenomatoid malformations

目的：总结婴幼儿先天性肺囊腺瘤样畸形的诊断和手术治疗方法。方法回顾性分析自2008年8月至2013年7月于本院就诊的70例先天性肺囊腺瘤样畸形患儿临床资料，比较出生后X线检查、CT扫描检查及手术治疗结果，并进行随访。结果70例患儿中，男39例，女31例。入院时最小年龄为30 min，最大年龄为13岁。手术时期：新生儿期40例，1个月至1岁26例，1～13岁4例，平均手术年龄（4.3±0.6）个月。分型：Ⅰ型18例，Ⅱ型35例，Ⅲ型6例，混合型6例，存在大叶性肺气肿5例。4例合并其他畸形，其中2例合并纵隔囊肿；1例混合型合并骶尾部畸胎瘤；1例左侧Ⅲ型合并左侧膈疝。病变位置：1例为双侧，其余均为单侧。右侧21例，左侧18例。术前X线检查均无特异性。70例均采用开胸手术治疗，其中肺叶切除术59例，肺段切除术6例，肺叶＋肺段切除术3例；2例行右肺两肺叶切除术。术后并发症包括气胸、咳嗽、伤口感染、胸腔粘连、膈肌膨升，经治疗后痊愈。随访2个月至5年，均存活，无一例死亡，且无术后复发现象。结论出生后增强CT检查对于先天性肺囊腺瘤样畸形的确诊及手术方案的制定极其重要，建议确诊后早期手术切除。

Objetive To review and analysis diagnosis and surgical treatment of Congenital Cystic Ade-nomatoid Malformations （CCAM）. Methods Reviewed the CCAM cases operated in our institution between August 2008 and July 201 3 Data on pre-and postnatal investigations,clinical presentation,X-ray contrast with CT scan,neonatal surgical procedure and outcome of surgery,and fellowed-up. Results 70 cases of fetal chest cystic or mixed mass were diagnosed antenatally by ultrasound,been confirmed by CT scaned postnatal.CCAM has been classified by Stocker,type-Ⅰin 1 8 cases,type-Ⅱin 35 cases,and type-Ⅲin 6 cases and mis-cellaneous in 6 cases,congenital lobar emphysema in 5 cases. Associated malformations included medistinl cyst in 2 cases;sacrococcygeal teratoma in 1 case ;diaphragmatic hernia in 1 case. The average surgery age was 4.3 ＆#177;0.6 months. CCAM location：except for 1 case for double side,the rest are unilateral.：39 cases in the right. Conclusion In this review,it showed that the enhanced CT scan after the birth is extremely important for the diagnosis of Congenital Cystic Adenomatoid Malformations and the scheme for the operation setting.After diagnosis the early surgical resection has been suggested.