先天性肺囊性腺瘤样畸形15例临床、影像及病理特点

Clinical manifestations, imaging f＇mdings and pathological features of 15 cases with congenital cystic adenomatoid malformation in the lung

目的探讨先天性肺囊性腺瘤样畸形（CCAM）的临床表现、影像学、病理特点及治疗情况。方法回顾性分析温州医科大学附属育英儿童医院2006年8月至2014年8月经手术和病理证实的15例CCAM患儿的临床特点、影像学、病理特点、诊断、治疗方法及预后。结果15例患儿中男11例，女4例。临床表现：肺部感染12例，反复胸痛1例，胸骨下段凹陷畸形1例，无症状1例。胸部CT检查表现为单侧肺部多发气囊肿9例（其中1例并漏斗胸和隔离肺），左肺下叶巨大液气囊肿1例，肺部密度增高影4例，1例反复胸痛患儿CT检查表现为右侧膈肌膨隆合并隔离肺。全部病例行手术切除，1例行囊肿切除术，1例行右隔离肺切除术及膈肌折叠术，1例并漏斗胸患儿行病变肺叶切除、隔离肺切除及微创漏斗胸矫正术（Nuss手术），其余12例行病变肺叶切除术。15例患儿术后恢复良好，无并发症发生。病理分型Ⅰ型11例，Ⅱ型3例，Ⅲ型1例，其中并肺隔离症2例。结论CCAM是一种少见的疾病，临床上常因肺部感染被发现，影像学主要表现为多发气囊肿，胸部CT检查是术前诊断的重要依据，病理分型以Ⅰ型和Ⅱ型多见，治疗以早期手术切除为主，预后良好。

Objective To explore the clinical manifestations, imaging findings, pathological classification and treatment of congenital cystic adcnomatoid malformation （CCAM） of the lung. Methods The clinical features, imaging findings, pathology information, diagnosis, treatment method and its prognosis of children with CCAM confirmed by operation and pathology were retrospectively analyzed in Yuying Children＇s Hospital Affiliated to Wenzhou Medical University from August 2006 to August 2014. Results Eleven patients were boys and 4 patients were girls. One case had asymptomatic clinical features, 12 cases had pulmonary infection, 1 case had recurrent chest pain, and 1 case had depressed deformity in sternum inferior segment. Chest CT scanning indicated that 9 cases had multiple gas cysts at unilaterm side of lung, among which 1 case was of funnel chest and pulmonary sequestration, 1 case of huge cyst containing air and fluid at inferior lobe of left lung, and 4 cases of high density lung shadow; CT examination indicated that 1 case had recurrent chest pain and eventration of diaphragm of the right side combined with pulmonary sequestration. All cases were treated by surgical resection, of whom 1 case was given cystectomy and sequestrectomy, diaphragmatic plication respectively, 1 case complicated with funnel chest disease underwent lesion pulmonary lobectomy, sequestrectomy and minimally invasive corrective surgery in pectus excavatum （Nuss surgery）, and the remaining 12 cases received lesion pulmonary lobectomy. All of 15 cases recovered well without complications. Pathological classification type of CCAM included 11 cases of type I ,3 cases of type II and 1 case of type III, among which 2 cases had pulmonary sequestration. Conclusions CCAM is a rare disease which can be discovered along with pulmonary infection. Multiple gas cysts are the most common imaging findings and the preoperative diagnosis of CCAM is mostly based on chest CT examination. Type I and type H are the most common pathological classification. The surgical resection should be given early surgical resection and the prognosis is usually good.