Congenital lung malformations, which can manifest at any age and pose significant morbidity and mortality risks, are a result of various etiological factors. These malformations can be categorized based on anatomical characteristics, histopathological features, and clinical progression. The most prevalent conditions include congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), hybrid lesions, bronchial atresia, congenital lobar emphysema, and bronchogenic cysts. Diagnostics typically involve a clinical assessment followed by imaging studies and laboratory evaluations. Treatment strategies vary based on the malformation type, symptom severity, and overall patient health. Surgical approaches are tailored to the specific malformation type and location, with objectives including symptom alleviation, complication prevention, creating sufficient intrathoracic space for lung expansion, and removal of abnormal tissue for compensatory lung growth.
Congenital Pulmonary Airway Malformation (CPAM), also known as cystic adenomatoid malformation, is a congenital anomaly during lung embryonic development. It arises due to abnormal lung cell development, potentially leading to the formation of one or multiple cystic sacs. CPAM is one of the most frequently diagnosed lung malformations and can be attributed to genetic predispositions, environmental influences, or other unknown factors. CPAMs are classified into five types based on location, cystic structure, size, and epithelial surface characteristics. They can be broadly categorized into two groups using prenatal ultrasound (US) findings: macrocystic lesions and solid echogenic masses. CPAMs can exert pressure on surrounding structures, leading to observable effects on ultrasound images. Diagnosis typically occurs during prenatal ultrasonography or through imaging tests in the postnatal period. Treatment typically involves surgical intervention, determined based on the size of the CPAM and the presence of symptoms. In recent years, the prenatal management of large microcystic CPAMs with a CVR >1.6 and/or the presence of hydrops before 32 weeks of gestation has evolved. Most patients are now treated with intravenous maternal betamethasone, which inhibits CPAM growth and triggers regression of hydrops.