High comorbidity rates in congenital lobar emphysema and the effect on clinical presentation.

Abstract

The presence of comorbidities (CM) in congenital lobar emphysema (CLE) has been previously described with varying rates. However, the clinical implication of CM on the clinical presentation and patient outcome in CLE is unclear. This was a retrospective cohort study between 2000 and 2022 in a single institution. The study included pediatric patients diagnosed with CLE. During the study period, 36 patients were identified with CLE. The presence of respiratory symptoms at diagnosis was documented in 69% (24/35) of the patients with 48% admitted to intensive care units. The presence of CM was documented in 14/36 (39%) of the patients, with cardiovascular anomalies as the most common (22%), followed by genitourinary anomalies (11%) and large airway anomalies (8%). When comparing the CM group (n = 14) vs the non-CM group (N = 22), there was no significant difference regarding gender, term birth, age of diagnosis, and the location of the lesion. The CM group had significantly higher rates of intensive care unit (ICU) admissions (75% vs 33%, p = 0.02), longer duration of admission (median 46days vs 9, p = 0.02), and need for pre-operative non-invasive respiratory support (50% vs 5%, p = 0.002).Conclusion: The presence of CM in CLE was associated with a more severe presentation as reflected with significantly higher rates of ICU admissions, prolonged admissions, and higher need for non-invasive respiratory support. Screening for CM, mostly for cardiac anomalies, is recommended at time of CLE diagnosis. What is Known: • Congenital lobar emphysema (CLE) is a developmental lung anomaly generally associated with other comorbidities; however, their clinical implication on presentation is unclear • The prevalence of comorbidities in CLE was previously reported around 15-20%, with cardiovascular anomalies as the most common What is New: • Comorbidities are very common (39%) in CLE, and their presence is related to a more severe clinical presentation and need for respiratory support • Screening for comorbidities is recommended in CLE, specifically for cardiovascular and genitourinary anomalies.