Background Congenital Pulmonary Airway Malformations (CPAM) is a congenital disorder in which there is replacement of a portion of lung by non-functioning cystic section of abnormal tissue. Majority of CPAMs are asymptomatic at birth. The aim of this project was to review the current management of CPAM in a tertiary hospital in Ireland.
Methods Retrospective review of cases of CPAM attending the respiratory outpatients department over 4 years (2013–2017). Data was collected from electronic outpatient letters and hospital radiology system. Data was reviewed against best practice guidelines,1,2,3 and analyzed using descriptive methods.
Results In total 16 cases of CPAM attended the respiratory department between 2013–2017. Diagnosis was either made antenatally, in 10 cases (63%), postnatally in 3 cases and unknown in 3 cases. Evidence suggests a chest x-ray should be performed in the postnatal period, followed by a CT Thorax in the following months. In our cohort, 11 (69%) cases had a CXR in the postnatal period, 3 of which were reported normal. 14/16 cases were asymptomatic, the most common symptom being recurrent infections. All cases had a CT Thorax performed- mean timing was 11 weeks (1–56 weeks).8 cases had a repeat CT Thorax prior to surgery. Surgery was planned in 11(69%) cases. The mean time to surgery was 18 months (2–31 months).2 cases are being followed up medically by the respiratory team with repeated imaging at scheduled intervals and 2 further cases are awaiting surgical review.
Conclusions The management in 9(56%) cases of CPAM in this tertiary hospital met the suggested guidelines in the current literature. It is recommended that children with CPAM are operated on at an early stage, ideally before 2 years of age. This was achieved in just over half of our cohort (55%). Of note, 73% of children who underwent surgery had at least two CT scans prior to surgery.
Recommendations Close follow-up in the postnatal period and early referral to tertiary surgical services for management of their lesion, given the potential future risk for infection and malignancy.2
Education of staff and family regarding the radiation risk of repeated CT imaging in young children.4
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