The Science Journal of the American Association for Respiratory Care

2012 OPEN FORUM Abstracts

SYMPTOMATIC CONGENITAL PULMONARY AIRWAY MALFORMATION: A CASE STUDY.

Amy Gibbs1, Gary R. Lowe1, Katia El Taoum2, Dennis Schellhase2; 1Respiratory Care Services, Arkansas ChildrenÂ’s Hospital, Little Rock, AR; 2Dept. of Pediatrics, Pulmonary Medicine Section, University of Arkansas for Medical Sciences, Little Rock, AR

Introduction: We describe the rare presentation of a previously healthy 5 month old infant who developed respiratory distress secondary to a spontaneous tension pneumothorax (PTX) which was caused by a rare congenital lung malformation. Case Summary: The patient presented to an outside hospital with increased work of breathing (WOB), tachypnea and low grade fever. She was diagnosed with acute otitis media and sent home with oral antibiotics. She continued to have increased WOB and was taken back to the hospital where a chest x-ray (CXR) was done revealing an acute right-sided tension PTX and significant mediastinal shift to the left. Needle aspiration was done without resolution. She was transferred to our facility and a chest tube was placed which resolved the distress but a residual air pocket/cyst was noted on repeat CXR. She was placed on 2 LPM O2 to improve WOB. The chest tube came out on day 3 and she developed the tension PTX again. The chest tube was replaced and a CT was done due to lack of improvement of the air pocket. CT revealed a cystic lesion in the right upper lobe consistent with Congenital Pulmonary Airway Malformation (CPAM). An echocardiogram (ECHO) was done to rule out cardiac malformation and revealed a small shunt through the foramen ovale, and a left to right interatrial shunt. She was taken to surgery on hospital day 4 for right upper lobe lobectomy. The pathology report confirmed the diagnosis of CPAM Type 1. She tolerated surgery well and was taken off O2. The chest tube was removed on day 6 without complications and she was discharged the same day. Discussion: CPAM is a rare abnormality of lung development that occurs from abnormal embryogenesis, results in cystic lung changes, and may cause a reduction in alveolar growth. The prevalence is estimated at ~ 1:25,000 to 1:35,000. The mortality rate is estimated at 25-35% of all children who present in the newborn period. There are 5 classifications of CPAM primarily based on cyst size. This case illustrates three important points. First, due to its rarity, CPAM should be considered when an infant presents with a spontaneous tension PTX. Second, an ECHO should be done to rule out coexisting cardiac lesions as they are frequently associated with CPAM. Third, resection of CPAM has two benefits: 1) removes risk of complications such as recurrent infection, PTX, and possibility of malignant transformation; 2) if done in the first 1-2 years of life, allows for compensatory lung growth. Sponsored Research - None