Congenital pulmonary airway malformation (CPAM) presenting as spontaneous pneumothorax in young boy

Abstract

Congenital pulmonary airway malformation (CPAM) is most common congenital lung anomaly of lower respiratory tract accounting for approximately 25% of all congenital pulmonary malformations. It is usually unilateral and involves single lobe of lung. It is usually diagnosed prenatally; rarely found in children and adults. We report a rare case of 14-year-old male presented with sudden onset breathlessness secondary to right sided pneumothorax associated with right lower lobe cystic lesion; successfully managed with multidisciplinary approach involving tube thoracostomy and non-anatomical wedge resection of right lower lobe cystic lesion (using VATS). Adults diagnosed with CPAM usually present with breathlessness, fever, recurrent pulmonary infection, pneumothorax, and haemoptysis. For definitive treatment of symptomatic CPAM cases, surgical resection at the time of diagnosis is recommended in view of possible risk of malignant transformation and recurrent respiratory tract infections. Considering the mild but definitive risk of malignancy, it is advocated to closely monitor the individuals with CPAM even after the surgical resection.