O2183: Optimizing Timing for Asymptomatic CPAM Resection: A PHIS Database Analysis (Phase 1)

Background: Congenital Pulmonary Airway Malformation (CPAM) is the most common congenital lung malformation. While most patients with CPAM are asymptomatic at birth, some will later experience CPAM-related complications. There is disagreement regarding its management. Citing the risk of leaving CPAM in situ, many advocate for “proactive resection” (PR). Conversely, others favor surveillance with intervention performed after the development of issues (“reactive resection” or RR).

Using a large, national database that allows longitudinal analysis of CPAM patients, we aim to better understand clinical outcomes that inform the decision-making process of CPAM management. This analysis is the first of a series of 5 planned studies to address the question of whether PR is superior to RR. In phase 1 we will establish a comprehensive CPAM cohort and define short-term outcomes.

Methods: A retrospective cohort study was conducted using the Pediatric Health Information System (PHIS) from 2013-2023. Timing of resection was defined as patient's age in months at encounter for resection and categorized into 5 groups based on clinical rationale: “neonatal” (0– < 3 months), “early” (3– < 6 months), “standard” (6– < 12 months), “later” (12– < 24 months), and “delayed” (>24 months). Mortality risk and disease severity at time of resection were classified using All Patient Refined Diagnosis-Related Groups (APR-DRG) algorithm, as defined in PHIS. Group demographics and key outcome variables were compared using descriptive and inferential statistics.

Results: A total of 3,329 CPAM patients were captured. Median age at CPAM resection within “neonatal,” “early,” “standard,” “later,” and “delayed” groups was 1, 4, 7, 15, and 79 months, respectively (p < 0.0001). Open operative approach was most common in the neonatal group (p < 0.0001). The presence of high severity level and mortality risk was greatest in neonatal group (p < 0.0001) and decreased with age, though an increase was observed in the delayed group (p < 0.0001). This trend was also observed for outcome variables: total and post-operative length of stay, blood transfusions, post-operative ventilator, and mortality (p < 0.0001) (table 1). Median adjusted total cost for resection was highest for neonatal group ($24,000) followed by the delayed group ($24,000), with a decrease in the early ($19,000), standard ($18,000), and later ($19,000) groups, respectively (p < 0.0001).

Conclusion: We observe meaningful differences in clinical outcomes after CPAM resection when stratified by age. The youngest (0- < 3 months) and second youngest patients (3 - < 6 months) are most likely to have high severity and mortality risk, and are more likely to require longer hospitalization, blood transfusions, and mechanical ventilation. Their hospital costs and mortality rate are also higher. Interestingly, however, these variables all decrease with advancing age, before a notable spike in the oldest, delayed cohort. The planned next phases of this project will provide additional insight into optimal management of CPAM patients.