Malformação adenomatóide cística do pulmão diagnosticada ao nascimento

Abstract

to review existent literature about cystic adenomatoid malformation (CAM), especially its perinatal aspects. Sources: bibliography review referent to 1966-2011, at PubMed/Medline and LILACS databases, using the following keywords: cystic adenomatoid malformation, cystic lung disease, congenital malformation prenatal diagnosis, lung development. Summary of the findings: CAM is a rare congenital malformation which incidence is 1:25,000 to 35000 births. Its etiology remains unknown. It usually occurs isolated and has a variable natural history. There are 5 types according to Stockers classification: type I characterized by one or more cysts over 2cm in diameter, type II multiple cysts under 1cm, type III solid mass, type 0 solid lesion and type IV large peripheral cysts. The CAM prenatal diagnosis can be done from 17 to 20 gestational weeks by ultrasound. Fetal complications generally occur in cases that present large pulmonary lesions. It can make invasive fetal procedures necessary. The neonatal presentation varies from asymptomatic newborns to severe respiratory distress and even neonatal death. CAM diagnosis must be confirmed at the postnatal period using image exams, preferably thoracic CT scan. Tomographic finding keeps good correlation to anatomopathological diagnosis. For the symptomatic newborns the recommended management is lobectomy. For the asymptomatic ones there is no consensus about surgical treatment. Conclusions: CAM is a relatively rare anomaly. It can present symptoms that demand immediate diagnoses and treatment. The prenatal diagnosis is important to proper fetal and postnatal management