Diagnosis and intervention of congenital cystic adenomatoid malformation / 国际儿科学杂志

ABSTRACT

Congenital cystic adenomatoid malformation(CCAM) is a rare cystic lung disease with an incidence rate ranging from 1/35 000 to 1/25 000 births.The mechanism is still unclear and is regarded a nonhereditary anomaly caused by arrest of lung.In some countries, over 99% of CCAMs were diagnosed by fetal sonography at gestational age of 18~20 weeks.Chest X-ray and CT are common methods to detect the CCAMs after birth.Newborns with CCAM may present with respiratory distress, but most patients are asymptomatic in life.Although many methods for diagnosis and treatment were suggested, no unified guideline is provided yet.Herein, we reviewed the advances in the mechanism, diagnosis and treatment of CCAM to highlight this rare event.