Background: Most congenital cystic adenomatoid malformations (CCAM) are found in utero or during the immediate neonatal period. Some malformations regress in utero, while others persist and remain unnoticed until later in childhood. The optimal clinical management of patients with CCAM is controversial. The aim of this study is to suggest a safe strategy for treatment of CCAM based upon the age of the patient at diagnosis, by analyzing the clinical features of CCAM and considering the possibility of regression in early infancy.
Method: This is an observational retrospective study of 19 patients with CCAM. The clinical features, histopathological classification, status of lesion regression, diagnostic method, treatment, and outcome were collected. Patient data were analyzed highlighting age at disease presentation.
Results: Five out of the seven neonates with neonatally-diagnosed CCAM presented with respiratory distress. Eight of the twelve patients in the late-onset group had respiratory tract infections. Regression of the lesion during the early postnatal period was documented in 4 neonatally-diagnosed CCAMs, while none of the patients in the late-onset group showed signs of radiographic changes after a mean follow up of 4 years. Skeletal malformation was the most common associated anomaly in our series.
Conclusion: Conservative treatment is suggested for neonatally-diagnosed CCAM because of possible postnatal remission. Surgery may be required in older patients because of possible recurrent infections, infrequent mass regression, radiation exposure, and inconveniencies during follow-up visits. A thorough survey of possible associated skeletal anomalies in patients with CCAM is also recommended as early correction can improve life quality.