Department of Pediatrics, Division of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Department of Surgery, Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Eur J Pediatr. 2024 Oct;183(10):4573-4577. doi: 10.1007/s00431-024-05684-3. Epub 2024 Jul 13.
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 46 days 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.
先天性肺大疱（CLE）合并并存疾病（CM）的存在已有报道，但 CM 对 CLE 临床表现和患者结局的临床意义尚不清楚。这是一项 2000 年至 2022 年在单家机构进行的回顾性队列研究。该研究纳入了诊断为 CLE 的儿科患者。在研究期间，共发现 36 例 CLE 患者。有 69%（24/35）的患者在诊断时存在呼吸系统症状，其中 48%的患者入住重症监护病房。在 36 例患者中，有 14 例（39%）存在 CM，其中心血管异常最为常见（22%），其次是泌尿生殖系统异常（11%）和大气道异常（8%）。比较 CM 组（n=14）和非 CM 组（n=22），两组间在性别、足月产、诊断年龄和病变部位方面均无显著差异。CM 组的 ICU 入住率（75% vs 33%，p=0.02）、住院时间中位数（46 天 vs 9 天，p=0.02）和术前无创呼吸支持的需求（50% vs 5%，p=0.002）均显著更高。结论：CLE 合并 CM 与更严重的临床表现相关，表现为 ICU 入住率、住院时间延长和无创呼吸支持需求增加。建议在 CLE 诊断时筛查 CM，尤其是心脏异常。已知情况：• 先天性肺大疱（CLE）是一种发育性肺异常，通常与其他并存疾病相关；然而，其对临床表现的临床意义尚不清楚。• CLE 并存疾病的患病率先前报道为 15-20%，心血管异常最为常见。新情况：• CLE 中并存疾病非常常见（39%），其存在与更严重的临床表现和呼吸支持需求相关。• 建议在 CLE 中筛查并存疾病，特别是心血管和泌尿生殖系统异常。
