OBJETIVO: 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 (1986-2011). 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 pacientes. Foram encontradas anomalias associadas em 47% das crianças. A mais frequente foi sequestro (71%), mais presente na MACC tipo 2 (p = 0,001). As anomalias mais graves se relacionaram principalmente com o tipo 2 (p = 0,008). Também foram observados um blastoma pleuropulmonar e um carcinoma broncoalveolar. A mortalidade foi de 5% (n = 9). Os fatores de risco para mortalidade foram falência respiratória (OR = 25,7 [IC95% 3,2-221]; p = 0,03), sepse (OR = 9,9 [IC95% 8,2-12]; p = 0,002), necessidade de assistência respiratória (OR = 9,5 [IC95% 2,3-37]; p = 0,04) e diversas comorbidades associadas (OR = 3,3 [IC95% 1,2-22]; p = 0,008). 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.

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.

Objective. The aim of this study was to analyze variables of severity of acute lower respiratory infections in groups with and without personal risk factors. Population, material and methods. We conducted a retrospective cohort study. Medical charts of 461 patients admitted in Garrahan Hospital with infections by respiratory syncytial virus from 1998 to 1999 were examined. These risk factors were: preterm birth ≤ 34 weeks, preterm birth ≤ 6 months at the time of infection, ≤ 45 days of age, chronic pulmonary obstructive postviral disease, bronchopulmonary dysplasia, cystic fibrosis and congenital cardiopathies. Results. The clinical presentations were bronchiolitis 72.2% and pneumonia 13.9%. Mean age was 6.96 months (r 0.33 to 23.8 and mode 1 m). Males: female ratio was 1:0.8. The patients with low personal risk factors were 57.3% and with high personal risk factors 42.7%. Both groups were compared regarding the variables: age low risk 7.27 vs. high risk 6.41 (p 0.073), days of admission 11.01 vs. 15.21 (p 0.001), days of oxygen 6.74 vs. 14.40 (p 0.001), days on assisted ventilation 14 vs. 29 (p 0.001) and number of patients on assisted ventilation 0.46 vs. 2.65 (p 0.001). The global mortality rate was 1.04%. Conclusions. The group of patients with respiratory syncytial virus infection with personal risk factors required more days of hospitalization, days of oxygen and more probability of assisted ventilation. This data should be taken into account when considering prevention and treatment in respiratory syncytial virus infection to improve the prognosis of these patients.

Objetivo. Analizar diferentes variables de gravedad clínica en niños con factores de riesgo y sin ellos para enfermedad respiratoria por virus sincicial respiratorio en nuestro medio. Población, material y métodos. Estudio retrospectivo de cohorte de historias clínicas de pacientes hospitalizados en el Hospital "Prof. Dr. Juan P. Garrahan", con diagnóstico de infección respiratoria aguda baja por virus sincicial respiratorio entre 1998 y 1999. Se consideraron factores de riesgo: recién nacidos pretérmino ≤ 34 semanas, prematuros ≤ 6 meses al momento de la infección, recién nacidos de término ≤ 45 días, enfermedad pulmonar obstructiva crónica, displasia broncopulmonar, fibrosis quística y cardiopatías congénitas. Se dividió a la población en dos grupos: sin riesgo y con riesgo, y se evaluaron distintas variables. Resultados. Se analizaron 461 pacientes. La edad media fue de 6,96 meses (r= 0,33 a 23,8 y modo 1 m). La relación M/F fue de 1:0,8. Las formas clínicas de presentación más frecuentes fueron bronquiolitis 72,2% y neumonía 13,9%. Los pacientes sin riesgo fueron 57,3% y con riesgo, 42,7%. Los pacientes con riesgo tuvieron más días de internación (p= 0,001), de oxigenoterapia (p= 0,001) y mayor requerimiento de asistencia respiratoria mecánica y durante más días (p= 0,001). La mortalidad del grupo fue 1,04%. Conclusión. Los niños con infección por virus sincicial respiratorio con factores de riesgo requirieron mayor tiempo de internación, de oxigenoterapia y tuvieron mayor probabilidad de requerir asistencia respiratoria mecánica. Estos datos determinan en qué pacientes deberían implementarse medidas preventivas y terapéuticas más precoces para mejorar su evolución y pronóstico.