Chiew Wah Loh1, Kah Peng Eg1,2, Jessie de Bruyne2, Shih Ying H’ng2, Yan Yi Neo2, Soo Ling Chuah2, Anand A/L Sanmugam2, Wei Sheng Tan2, Anna Marie Nathan1,2
1Universiti of Malaya, Kuala Lumpur, Malaysia
2Universiti of Malaya Medical Centre, Kuala Lumpur, Malaysia
Introduction
Congenital pulmonary airway malformation (CPAM) is a developmental anomaly of the lower respiratory tract with variable clinical presentation. Surgical resection remains the definitive treatment, although the optimal timing is still debated. This case report presents two cases who were managed surgically at different ages, highlighting contrasting clinical outcomes.
Case Report
Case 1 is a late preterm neonate (36 weeks gestation) antenatally diagnosed with left-sided CPAM. She underwent two fetal thoracocenteses. At birth, she presented with severe respiratory distress, requiring intubation. She had unilateral left tension pneumothorax and pulmonary hypertension. An emergency lobectomy was performed on day 4 of life. Postoperatively, she had persistent mild respiratory distress necessitating home CPAP therapy. She is currently thriving with good weight gain and no episodes of recurrent pneumonia.
Case 2 is a term neonate with a prenatal diagnosis of right-sided CPAM. He was intubated at birth for severe respiratory distress. However, due to nosocomial pneumonia, his lobectomy was delayed to 5 months old, eventhough he required home CPAP. Surgical intervention was prompted by worsening respiratory distress due to mediastinal shift and left lung compression. Post-lobectomy, he continued to have persistent respiratory distress requiring home NIV therapy and recurrent pneumonia. Follow-up revealed chronic suppurative lung disease, poor weight gain and persistent right lung hypoplasia.
Conclusion
These two cases underscore the potential benefits of early surgical intervention in CPAMs with persistent respiratory distress at birth, which may be associated with better postoperative recovery and long-term outcomes. Conversely, delayed surgical management may contribute to increased respiratory morbidity and poorer prognosis.