title: Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases.
abstract: OBJECTIVE
  Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital.
  
  
  METHODS
  Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years (1986-2011).
  
  
  RESULTS
  Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure (OR = 25.7 [95%CI 3.2-221]; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008).
  
  
  CONCLUSIONS
  Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications.
authors.affiliation: Pulmonology Department, Hospital de Pediatría Dr. Juan P. Garrahan, Buenos Aires, Argentina. verogiubergia@hotmail.com
authors.forename: Verónica
authors.initials: V
authors.lastname: Giubergia
authors.forename: Marcelo
authors.initials: M
authors.lastname: Barrenechea
authors.forename: Mónica
authors.initials: M
authors.lastname: Siminovich
authors.forename: Hebe Gonzalez
authors.initials: HG
authors.lastname: Pena
authors.forename: Patricia
authors.initials: P
authors.lastname: Murtagh
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000145:classification / Q000473:pathology / Q000601:surgery; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000473:pathology; D008297:Male; D011013:Pneumonectomy / Q000401:mortality; D011230:Precancerous Conditions / Q000473:pathology; D012189:Retrospective Studies; D012307:Risk Factors; D018709:Statistics, Nonparametric; D016896:Treatment Outcome