Congenital Pulmonary Airway Malformation in a 1-Month-Old Infant: A Call for Strengthening Antenatal Diagnosis

Abstract

Background Congenital pulmonary airway malformation (CPAM) is a rare condition that poses diagnostic and management challenges because of a low index of suspicion. Most reported cases of CPAM are diagnosed postnatally via chest X-ray and/or chest CT scan or MRI, despite the high diagnostic efficacy of obstetric ultrasound. The management of symptomatic CPAM is surgical excision. Case details This is a case report of a delayed diagnosis of a 1-month-old male infant who presented with signs of respiratory distress since week one of life associated with one episode of fever and a history of being treated for pneumonia with no improvement. On admission to our tertiary unit, the infants’ preliminary differential diagnoses were severe pneumonia, suspected left-sided congenital diaphragmatic hernia or acyanotic congenital heart defect. A chest X-ray revealed a large left-sided cystic lesion with a pressure effect on the left lung and a mediastinal shift to the right side of the chest, which was confirmed by a CT scan of the chest. Left lower lobectomy was performed, resulting in significant improvement with complete resolution of the signs of respiratory distress by postoperative day 13. Conclusion A low index of suspicion among healthcare practitioners can lead to significant morbidity in infants with a delayed diagnosis of CPAM. Postnatal chest X-ray and CT scans play crucial roles in confirming the diagnosis, assessing the extent of the lesion, and guiding the surgical approach in our case, resulting in a favorable outcome. Prenatal diagnosis is still the cornerstone for early detection and early intervention.