Nelson Textbook of Pediatrics 22nd Edition | Formerly congenital cystic adenomatoid malformation (CCAM). Hamartomatous lung lesion, incidence 1-4/100,000 births. Stockert classification Types 0-4 (Type 1 most common, 60-70%). Prenatal diagnosis: ultrasound, MRI, CPAM volume ratio (CVR >1.6 predicts hydrops). Clinical: neonatal respiratory distress, recurrent pneumonia (older children). Associated with DICER1 mutations (pleuropulmonary blastoma risk, especially Type 1 and 4). Treatment: surgical resection (recommended by 1 year), observation for small asymptomatic lesions (controversial).
🌐 paeds.online — Pakistan's Pediatric Platform| Type | Features | Prognosis / Malignancy Risk |
|---|---|---|
| Type 0 (acinar dysplasia) | Microscopic, involves all lobes | Fatal at birth, poorest prognosis |
| Type 1 (most common, 60-70%) | Macrocystic (>2 cm), mucus cells | Good, but risk of malignant transformation (pleuropulmonary blastoma, bronchioloalveolar carcinoma) |
| Type 2 (15-20%) | Microcystic (<2 cm), associated with other anomalies (renal, cardiac, TEF) | Poor prognosis (due to associated anomalies)|
| Type 3 (5-10%) | Microcystic/solid, bulky, male predominance | Poor (large, may cause hydrops)|
| Type 4 (10%) | Macrocystic, no mucus cells, associated with pleuropulmonary blastoma | Screen for DICER1 mutations |