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

Abstract

Objective To explore the clinical manifestations, imaging findings, pathological classification and treatment of congenital cystic adenomatoid 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 ope-ration 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 unila-teral 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 Ⅱ are the most common pathological classification.The surgical resection should be given early surgical resection and the prognosis is usually good. Key words: Congenital cystic adenomatoid malformation; Child; Clinical manifestations; Imaging findings; Pathological features