ABSTRACT

Introduction: Congenital pulmonary airway malformation (CPAM) is a rare developmental lung anomaly characterized by cystic lung lesions, constituting about 25% of congenital pulmonary anomalies. It typically occurs sporadically and is not significantly associated with race, age, or other maternal factors. The prenatal course of CPAM can vary based on factors such as lesion size, mediastinal shift, and associated anomalies. While the overall prognosis is generally favorable in the absence of severe complications like hydrops fetalis, which can adversely affect outcomes, management strategies include corticosteroids, thoracoamniotic shunt (TAS), and, in severe cases, open fetal surgery or the EXIT procedure. Postnatal surgical excision usually offers a curative outcome with an excellent prognosis. Without surgical intervention, there are risks of recurrent infections and, rarely, malignant transformation. Effective prenatal detection significantly impacts clinical decision-making and neonatal outcomes, and the necessity and timing of surgery for asymptomatic infants remain subjects of ongoing debate, underscoring the need for personalized, multidisciplinary care.

Case Report: A female infant, delivered at 38 weeks via Cesarean section, was diagnosed with CPAM following routine prenatal ultrasound. Presence of extensive cystic regions in the right lung confirmed the diagnosis and a CPAM volume ratio (CVR) of 0.37 indicated a low risk of severe complications. The pregnancy was largely uneventful, with maternal chronic hypertension managed by Nifedipine and mild intermittent asthma. Postnatal chest radiography corroborated the CPAM diagnosis, while an abdominal ultrasound investigated intrahepatic calcifications. Maternal screening for cytomegalovirus and toxoplasmosis yielded negative results, and postnatally, the infant also tested negative for both infections. The neonate, though asymptomatic and stable, required close monitoring in the neonatal intensive care unit to prevent potential respiratory compromise.

Conclusion: Advances in prenatal imaging have greatly improved the early detection and management of CPAM, allowing for targeted neonatal care and strategic planning. This case highlights the importance of a multidisciplinary approach involving obstetricians, maternal-fetal medicine specialists, neonatologists, and pediatric thoracic surgeons in the effective management of CPAM. Early identification and diligent postnatal monitoring are essential for ensuring optimal outcomes and minimizing the risk of complications. The coordinated care and strategic decision-making exemplified in this case underscore the potential for enhanced neonatal health and long-term well-being through comprehensive management of congenital anomalies.