儿童先天性肺气道畸形73例临床病理分析

Congenital pulmonary airway malformation of the lung in children: a clinicalpathological analysis of 73 cases

目的:探讨先天性肺气道畸形(CPAM)的临床、影像、病理特征及预后。方法:回顾性分析73例儿童CPAM的临床病理特征并复习相关文献。结果:患儿年龄1d^15岁,平均年龄4.3岁,男女发病几率基本相同。依照Stocker组织病理学分型,73例CPAM中Ⅰ型49例,Ⅱ型22例,Ⅲ型2例。均为单侧病变,累及左侧29,右侧44例,合并心脏畸形6例,合并蛛网膜囊肿2例,伴隐形脊柱裂及甲状腺发育不良1例,伴脑发育落后、脑积水1例,伴肾母细胞瘤1例。影像学方面除2例Ⅲ型CPAM显示肺实变样改变外,余病例胸部X线或CT均表现为单个或多个含气大囊;或多发大小相近蜂窝状小囊,囊内未见肺纹理。73例均行患侧肺叶切除术,随访时间2~25个月不等,除2例Ⅲ型患儿死亡外,余患儿恢复良好。结论:CPAM是一种较罕见的发生于肺的先天性囊性腺瘤样畸形,可通过影像检查发现,确诊依据病理组织学检查,通过手术完整切除患侧肺叶,预后较好。

Objective: To explore the clinical, imaging, pathological feature and prognosis of congenital pulmonary airway malformation(CPAM). Methods: Retrospect analysis the clinical pathologic features of 73 cases of CPAM and review related literatures. Results: Patients’ age ranges from 1 day to 15 years, with mean age 4.3 years old. CPAM has no sex predilection. Histologic examination showed that 49 cases were classii ed as Stocker type I, 22 cases as type II, 2 cases as type III. All were unilateral lesion and involvement on the let side of the 29 cases, on the right side of 44 cases. Of the 73 cases studied, the associated malformations included cardiac anomalies(6 cases), arachnoid cyst(2 cases), invisible spinal bii da and thyroid dysplasia(1 case), brain development backwardness and hydrocephalus(1 case), wilm’s tumor(1 case). All of the 73 cases performed X-ray or CT examination: except mass-like change for 2 cases, the others showed single or multiple large air cystic lesions; honeycomb-like small cystic lesions, the cyst lack of lung marking. All cases were underwent lobectomy, Follow-up time ranges from 2~25 months, except 2 cases died of type III, the rest of 71 cases recovered well. Conclusion: CPAM is a relatively rare pulmonary lesion of congenital cystic adenomatoid malformation. Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination. Surgical excision, commonly lobectomy is recommended.