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 .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 ; 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. ResumoObjetivo: A malformação adenomatoide cística congênita (MACC) é a malformação pulmonar ressecada cirurgicamente mais comum em crianças. Este estudo retrospectivo foi realizado para apresentar a experiência de 172 casos de MACC em um hospital pediátrico.Métodos: Séries publicadas com um pequeno número de pacientes relatam detalhes de lesões, evolução e tratamento. Como este estudo lida com características clínicas, evolução e procedimentos cirúrgicos em 172 crianças com diagnóstico de MACC, a população inclui casos tratados e acompanhados em um hospital pediátrico ao longo de 25 anos .Resultados: A idade média ao diagnóstico foi de 48 meses (r = 0,03-213), 52% (n = 90) eram do sexo masculino. Os sintomas de apresentação mais comuns foram dificuldade respiratória em crianças com menos de 6 meses de idade (40%) e pneumonia recorrente nas que tinham mais idade (75%; p = 0.001). Lobectomia foi o procedimento de escolha na maioria dos casos. Todos os tipos histológicos foram encontrados: 1 (70%), 2 (24%), 4 (4%) e 0 e 3 (n = 1). Foi observado um padrão misto em nove...

Co-Existence of Congenital Cystic Adenomatoid Malformation and Pulmonary Sequestration in a Newborn with Spontaneous Pneumothorax: A Case Report and Review of the Literature

“…Approximately 80% of ELS receives its blood supply from the descending thoracic or abdominal aorta but the blood supply may also be from branches of a large vessel (4,15). In a large series of infants, PS was reported to be more frequent in CCAM type 2 (16). Our patient was male and the combination of type 2 CCAM and ELS was in the right side with the blood supply directly from the aorta.…”

“…The mortality rate was found to be 5% in a large series of infants, and most of them (58%) had type 2 CCAM. Respiratory failure, sepsis and respiratory support requirements and severe associated comorbidities have been reported to be the risk factors for mortality (16). Our case had respiratory failure from birth with recurrent pneumothorax and mechanical ventilation requirement all of which may be associated with death.…”

Hydrops fetalis secondary to supradiaphragmatic extrapulmonary sequestration with congenital cystic adenomatoid malformation

“…The age at diagnosis ranges from prenatal period to 73 years. 5,6,9 There is an equal gender distribution with 74% being left sided lesions. Whereas ELS can be readily distinguished from intralobar sequestration by the complete separation of ELS from the normal lung, the presence of CCAM within on ELS requires careful microscopic examination to identify the features mentioned above.…”

Patología Pulmonar Congénita: Evaluación Y Manejo Perinatal

“…Es el segundo tipo más frecuente (20-40%) y tiene una alta asociación con secuestro pulmonar y malformaciones de otros sistemas (cardíacas, hernia diafragmática, atresia de esófago, agenesia renal) (27).…”

“…Frente a esa postura, se encuentra la de aquellos grupos que han optado por una cirugía electiva ante el riesgo de infecciones recurrentes, neumotórax o malignización. La evidencia publicada muestra evolución postoperatoria favorable, bajas tasas de complicaciones y recuperación morfológica y funcional completa (27,62). Nuestro centro basa su conducta en esta última postura, la que se ref leja en el f lujograma de evaluación y manejo de lesiones pulmonares congénitas mostrado en la figura 10.…”