RESUMEN
OBJECTIVE: To identify the incidence of congenital cystic adenomatoid malformation of the lung (CCAM) at birth; to evaluate prenatal and perinatal history, association with clinical and sociodemographic variables and concordance between CT scan results and anatomopathology studies. METHOD: Descriptive study based on the registry of malformed newborns, deliveries and patients records between August 1990 and November 2010. Ultrasonic, clinical, imaging and anatomopathologic information were studied. Association studies were made using chi-square test. Kappa was used to correlate CT scan to anatomopathology results. RESULTS: The incidence was 1:1980 (25/49 503). The mean gestational age for detection by ultrasonography was 24 ± 3.7 weeks. There were progression of the lesions in 11 cases (44%), stability in 6 (24%) and regression in 8 (32%). Three cases of CCAM followed due to polyhydramnios/hydrops died. There were neither familial cases nor association with sex, weight, age or maternal parity (p > 0.15). Radiographic abnormalities were found in 22/23 studied patients. The correspondence between CT scan and anatomopathologic was 0.77 (Kappa). CONCLUSIONS: The incidence was higher than the one described in the literature, probably, because it is a reference center in fetal medicine. The prenatal lesion involution rate was 32%, an intermediate proportion. There was good concordance between CT scan and anatomopathologic results. The polyhydramnios/hydrops were predictive of worst prognosis.
Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Brasil/epidemiología , Malformación Adenomatoide Quística Congénita del Pulmón/epidemiología , Malformación Adenomatoide Quística Congénita del Pulmón/etiología , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Femenino , Terapias Fetales , Estudios de Seguimiento , Humanos , Incidencia , Recién Nacido , Masculino , Embarazo , Sistema de Registros , Remisión Espontánea , Factores de Riesgo , Tomografía Computarizada por Rayos X , Ultrasonografía PrenatalRESUMEN
Se presenta el caso de un lactante, parto fuera de la maternidad, ingresado en el Servicio de Pediatría por tos, discreta dificultad respiratoria, fiebre y disminución del peso para su edad, que luego del interrogatorio a la madre sobre sus síntomas a partir del nacimiento y las radiografías simples y contrastadas se llega a la conclusión de tratarse de una anomalía congénita del pulmón llamada malformación quística adenomatoidea, entidad muy rara caracterizada por quistes pulmonares únicos o múltiples limitándose a un lóbulo del pulmón y que se presentan en niños desde el período neonatal, se describe el cuadro clínico y radiológico del paciente.(AU)
We present a case report of a breast-fed whild which delivery was out of a lying in hospital admitted to Pediatric service suffering from cough, a discrete respiratory distress, fever, and decreased weight for its age. After the mother´s interrogatpry about its symptoms from delivery, and simple and contrasted X rays, it is concluded it is a lung congenital anomaly colled adenomatoid cystic malformation, a very rare entity characterized by unique on multiple lung cysts, limited to a lung lobule, which is presented in children from neonatal period on. Clinical and radiologic findings of the patient are described.(EU)