Neonatal Congenital Pulmonary Airway Malformation Causing Respiratory Failure Managed With Early Surgical Resection: A Case Report

Congenital pulmonary airway malformation (CPAM) is a rare cystic lung anomaly in newborns. It can range from asymptomatic to causing life-threatening respiratory distress. However, severe respiratory failure occurring in the immediate neonatal period is uncommon. We report the case of a neonate with CPAM who developed respiratory failure requiring urgent surgical management. The case aims to highlight the importance of early diagnosis and surgical intervention in neonates presenting with severe CPAM to prevent fatal outcomes and support complete recovery. A 29-hour-old male neonate was admitted with cyanosis and severe respiratory distress at birth. His oxygen saturation was only 50% on supplemental oxygen. Chest X-ray and computed tomography (CT) scan revealed a massive multicystic lesion in the left lung consistent with CPAM, with mediastinal shift and compression of the right lung. The baby was intubated and placed on mechanical ventilation, but his respiratory status remained critical. Emergent surgical resection of the left upper lobe was performed. Histopathological examination confirmed a mixed Type I and Type II CPAM. Following early surgical lobectomy, the neonate's respiratory status improved rapidly. He was weaned from the ventilator without difficulty, and mediastinal structures returned to midline. Post-operative course was uneventful, and the infant was discharged in good condition within two weeks. Follow-up at four years showed normal respiratory function, normal pulmonary development on imaging, and no evidence of recurrent pulmonary disease. Early diagnosis and intervention of neonatal CPAM are crucial in the presence of severe respiratory compromise. This case illustrates that urgent surgical resection was effective in correcting respiratory failure caused by a large CPAM, and complete recovery is achievable. Clinicians should consider congenital lung malformations when assessing newborns with unexplained respiratory distress.

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Publication Details

Journal
British Journal of Hospital Medicine
Published
2025-09-15
DOI
https://doi.org/10.12968/hmed.2025.0284
Primary Topic
Congenital Diaphragmatic Hernia Studies
Type
article
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article

Neonatal Congenital Pulmonary Airway Malformation Causing Respiratory Failure Managed With Early Surgical Resection: A Case Report

Yanan Hu, Linlin Fan
British Journal of Hospital Medicine
Congenital Diaphragmatic Hernia Studies
article

Neonatal Congenital Pulmonary Airway Malformation Causing Respiratory Failure Managed With Early Surgical Resection: A Case Report

Yanan Hu, Linlin Fan
article en

Abstract

Congenital pulmonary airway malformation (CPAM) is a rare cystic lung anomaly in newborns. It can range from asymptomatic to causing life-threatening respiratory distress. However, severe respiratory failure occurring in the immediate neonatal period is uncommon. We report the case of a neonate with CPAM who developed respiratory failure requiring urgent surgical management. The case aims to highlight the importance of early diagnosis and surgical intervention in neonates presenting with severe CPAM to prevent fatal outcomes and support complete recovery. A 29-hour-old male neonate was admitted with cyanosis and severe respiratory distress at birth. His oxygen saturation was only 50% on supplemental oxygen. Chest X-ray and computed tomography (CT) scan revealed a massive multicystic lesion in the left lung consistent with CPAM, with mediastinal shift and compression of the right lung. The baby was intubated and placed on mechanical ventilation, but his respiratory status remained critical. Emergent surgical resection of the left upper lobe was performed. Histopathological examination confirmed a mixed Type I and Type II CPAM. Following early surgical lobectomy, the neonate's respiratory status improved rapidly. He was weaned from the ventilator without difficulty, and mediastinal structures returned to midline. Post-operative course was uneventful, and the infant was discharged in good condition within two weeks. Follow-up at four years showed normal respiratory function, normal pulmonary development on imaging, and no evidence of recurrent pulmonary disease. Early diagnosis and intervention of neonatal CPAM are crucial in the presence of severe respiratory compromise. This case illustrates that urgent surgical resection was effective in correcting respiratory failure caused by a large CPAM, and complete recovery is achievable. Clinicians should consider congenital lung malformations when assessing newborns with unexplained respiratory distress.

British Journal of Hospital MedicineVol. 86(9)
Affiliated Hospital of Hebei University (CN), Baoding People's Hospital (CN)
Openalex Percentile: Top 60%
Congenital Diaphragmatic Hernia Studies
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