Rare presentation of pneumothorax in a young woman with underlying congenital pulmonary airway malformation

Congenital pulmonary airway malformation (CPAM) is the most common among a rare group of congenital anomalies of the lower respiratory tract. It has variable presentation depending on its subtype and the patient’s age. It may lead to respiratory distress in neonates. It can be a particularly challenging diagnosis in children born asymptomatic but present with complications later in life such as haemoptysis, recurrent chest infections, breathlessness and pneumothorax. Prenatal ultrasound, chest X-ray, CT scan and MRI are helpful in making a radiological diagnosis. A woman in her late teens presented with shortness of breath and right pleuritic chest pain. CT’s chest showed a number of bullae of varying sizes on the right, including one huge bulla compressing all three lobes. She underwent lung volume reduction surgery via video-assisted thoracoscopy, and the histology specimen confirmed the diagnosis of CPAM type 1.