小儿先天性肺腺瘤样畸形的诊断及外科治疗

目的 总结小儿先天性肺腺瘤样畸形(CPAM)诊治经验.方法 回顾2006年11月至2009年11月手术的13例CPAM临床资料,男6例,女7例,手术年龄50d至13岁.多数患儿有临床症状,经胸部X线和CT检查确诊.3例小于2个月者急诊手术,其余患儿择期手术.术式有单肺叶切除10例,肺叶+不规则肺段切除2例,囊肿剥离术1例.病理示Ⅰ型8例,Ⅱ型和Ⅲ型各2例,Ⅳ型1例.结果 无手术死亡.2例术后早期出现支气管胸膜瘘,1例经保守治疗,1例在胸腔镜下瘘口缝合后治愈.术后平均住院9.7 d,平均随访12.3个月,2例肺叶+不规则肺段切除者活动量轻度受限,其余患儿恢复良好.结论 胎儿超声是CPAM产前诊断的主要手段,胸部CT检查对产后诊断有高度特异性.

The diagnosis and surgical treatment of congenital pulmonary adenomatoid malformation

Objective To present the experience of diagnosis and treatment of congenital pulmonary adenomatoid malformation (CPAM) in children. Methods Between November 2006 and November 2009,13 patients aged from 50 days to 13 years at surgery, including 6 males and 7 females, were diagnosed with CPAM and underwent pulmonary resection at this center. Most patients presented respiratory distress and respiratory infections before surgery. Diagnosis was made based on chest radiography and computerized tomography (CT). The patients were performed elective surgeries except 3 patients (less than 2 months old) underwent emergency surgeries. Of the 13 patients, 10 were performed single lobectomies, 2 underwent lobectomies plus segmental resections, and 1 had a marsupialisation of the lung lobe cysts. Pathologic diagnoses were Stocker type 1 CPAM of 8 patients, Stocker type 2 CPAM of 2,type 3 CPAM of 2 and type 4 CPAM of 1. Results No death was noted. Two patients had bronchopleural fistula after surgery, of which 1 was healed after conservative treatments and the other underwent thoracoscopic repair. Mean hospital stay after operation was 9. 7 days. Mean follow-up period was 12. 3 months. The 2 patients underwent lobectomies plus segmental resections had mild activity limitation. Conclusions Antenatal ultrasounography is helpful to diagnose the fetal CPAM. CT is a specific way to diagnose thc postnatal CPAM. Early surgical interventions are beneficial in preventing the development of complications.