An adult with episodic retrosternal chest pain: an unusual presentation of congenital pulmonary airway malformation \u2013 case report

Ira Goldsmith,Sobaran Sharma,Joseph George,Umair Aslam

doi:10.1186/s13019-021-01470-6

Abstract

BackgroundCongenital 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 presentationA 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.ConclusionsCPAM 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.

Highlights

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
CPAM are composed of variable-size cysts, with at least one dominant cyst (> 2 cm in diameter); type-II CPAM are composed of smaller, uniform cysts less than 1 cm in diameter; and type-III CPAM are a solid mass composed of microcysts [1, 4]
Patients with CPAM may be asymptomatic or present with symptoms, which are mainly respiratory, for example, in neonates and infants CPAM are a cause of respiratory distress, and children may present with life-threatening pulmonary infections [2, 3]

Summary

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

Background

Findings

Discussion and conclusions