Issue 12/2025
Perenovska, P.
Clinic of Pediatrics – University Hospital “Aleksandrovska”, Medical University – Sofia
Congenital cystic adenomatous malformation (CCAM) is a rare congenital anomaly of the lung, arising as a result of impaired embryogenesis between the 5th and 8th weeks of gestation. It is characterized by proliferation of the terminal bronchioles and formation of cysts of various sizes, with one lobe affected in 95% of cases. The malformation can lead to compression of the mediastinal structures, pulmonary hypoplasia and non-immune hydrops. It is classified into three types: type I – large cysts with a favorable prognosis; type II – small cysts, often accompanied by additional anomalies; and type III – massive solid lesions with unfavorable outcome.
Diagnosis can be made prenatally by ultrasound, usually between 16–22 weeks of age, and postnatally by radiography and computed tomography, which is highly sensitive for distinguishing from other malformations. Clinical presentation ranges from severe respiratory distress in newborns to recurrent infections in older children, with about 10% remaining asymptomatic.
The therapeutic approach depends on the severity and age: in large lesions and respiratory distress, urgent surgical resection is necessary, while in mild cases, early elective surgery between 3–6 months is considered. Nonsurgical treatment is limited and carries a risk of progression and complications. Early diagnosis and an interdisciplinary approach are key for optimal outcome.
Keyword: Congenital cystic adenomatous lung malformation