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.