Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

Verónica Giubergia, Marcelo Barrenechea, Mónica Siminovich, Hebe Gonzalez Pena, Patricia Murtagh
2012 Jornal de Pediatria  
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
more » ... population includes cases treated and followed up in a pediatric hospital throughout 25 years . 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. J Pediatr (Rio J). 2012;88(2):143-8: Congenital cystic airway malformation, associated malformations, surgical procedures, children. Conclusões: Foram observadas anomalias relacionadas em quase metade da população. Devido à possibilidade de infecção recorrente ou desenvolvimento de neoplasias, deve-se considerar a ressecção cirúrgica quando MACC for diagnosticada. O desfecho cirúrgico é favorável e apresenta complicações tratáveis. J Pediatr (Rio J). 2012;88(2):143-8: Malformação cística congênita das vias aéreas, malformações associadas, procedimentos cirúrgicos, crianças. Não foram declarados conflitos de interesse associados à publicação deste artigo. Como citar este artigo: Giubergia V, Barrenechea M, Siminovich M, Pena HG, Murtagh P. Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases. J Pediatr (Rio J). 2012;88(2):143-8.
doi:10.2223/jped.2177 pmid:22543527 fatcat:w2d7xadfsfbzrpzojy5lnmkesi