An adult with episodic retrosternal chest pain: an unusual presentation of congenital pulmonary airway malformation – case report

BACKGROUND Congenital pulmonary airway malformation (CPAM) with partial anomalous pulmonary venous connection presenting as episodic retrosternal chest pain on exertion in an adult has not been described.

CASE PRESENTATION A 21-year-old female, non-smoker, presented with a 4-year history of sharp, episodic, retrosternal chest pains brought on with exercise. A contrast-enhanced computed tomography (CT) scan showed a grossly overinflated left lower lobe with partial anomalous pulmonary venous drainage into the left hemi-azygos vein. Lobectomy, the recommended treatment of choice, carried out thoracoscopically, was curative with satisfactory mid-term results. Histology confirmed type-II congenital pulmonary airway malformation.

CONCLUSIONS CPAM can present in young adults with unusual symptoms of chest pain on exertion. When suspected a contrast-enhanced CT scan is the gold standard for establishing the diagnosis. An anatomical lung resection is curative with satisfactory medium term results.