A rare case of congenital pulmonary airway malformation in a 14-year-old male presenting with spontaneous pneumothorax.

INTRODUCTION

and importance: Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental dysplatic lesion of the fetal tracheobronchial tree. It accounts for approximately 25 % of all congenital lung malformations. It is usually unilateral and involves one lobe with no significant gender or racial predilection. The vast majority of reported CPAM cases were discovered prenatally or within the first 2 years of life; however, it is rarely found in older children and adults.

CASE PRESENTATION

The purpose of this paper is to present a case of a 14-year-old male with a chest tube inserted 5 days before, as a management to left-sided spontaneous pneumothorax. His vital signs and laboratory tests were all within normal. Chest X-ray showed irregular opacity in the left lung field.

CLINICAL DISCUSSION

CT revealed multiple cystic-like lesions in the upper lobe of the left lung. The clinical impression was pointing towards a congenital lung lesion. The patient then underwent surgery. There were several pleural adhesions. The adhesions were released, and the upper left lobe was resected. Histopathological findings were compatible with type II CPAM. Four days postoperatively, chest X-ray was within the normal.

CONCLUSION

We report this case to highlight the importance of considering CPAM and other congenital malformations as a differential diagnosis in the adult population, especially in patients with sudden onset of pulmonary symptoms along with multiple cystic-like lesions on CT, as well as to draw attention towards spontaneous pneumothorax as a possible first presentation for CPAM.