A Type III Congenital Pulmonary Airway Malformation (CPAM) in the House - Is It a Smooth Sailing Journey?

Background: Congenital pulmonary airway malformation (CPAM) is a developmental lung anomaly often detected prenatally. CPAM type III is a rare subtype of CPAM, accounting for 5-10% of cases. Type III lesions have a high risk of hydrops and neonatal respiratory compromise. The aetiology is unknown. Case Presentation: A fetus was diagnosed in-utero with right lung CPAM on prenatal ultrasound at 29 weeks of gestation and delivered at 36 weeks via induced vaginal delivery due to maternal severe pre-eclampsia and intrauterine growth restriction. The infant was admitted to the neonatal intensive care unit (NICU) with a primary diagnosis of right CPAM. Management: High-resolution CT confirmed large multicystic CPAM occupying the right hemithorax consistent with type III. Right thoracotomy and lobectomy were performed. Histopathology revealed multicystic lung tissue, which is indicative of CPAM type III. The infant was discharged home at three months with long-term oxygen therapy. Conclusion: CPAM is a rare congenital lung anomaly, with type III being an uncommon variant. Surgical resection allows definitive diagnosis and treatment. Histopathological examination of the resected tissue is crucial to classify the CPAM subtype.