Kunisaki Shaun M, Saito Jacqueline M, Fallat Mary E, St Peter Shawn D, Kim Aimee G, Johnson Kevin N, Mon Rodrigo A, Adams Cheryl, Aladegbami Bola, Bence Christina, Burns R Cartland, Corkum Kristine S, Deans Katherine J, Downard Cynthia D, Fraser Jason D, Gadepalli Samir K, Helmrath Michael A, Kabre Rashmi, Lal Dave R, Landman Matthew P, Leys Charles M, Linden Allison F, Lopez Joseph J, Mak Grace Z, Minneci Peter C, Rademacher Brooks L, Shaaban Aimen, Walker Sarah K, Wright Tiffany N, Hirschl Ronald B
Section of Pediatric Surgery, Department of Surgery, University of Michigan and Michigan Medicine, C.S. Mott Children's and Von Voigtlander Women's Hospital, Ann Arbor, MI, USA.
Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO, USA.
J Pediatr Surg. 2019 Jun;54(6):1138-1142. doi: 10.1016/j.jpedsurg.2019.02.043. Epub 2019 Mar 1.
The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium.
After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015).
Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p < 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p < 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0).
Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion.
Level III.
本研究旨在评估大型多中心研究联盟中先天性肺叶气肿（CLE）患者的临床表现及手术效果。
经中央信赖机构审查委员会批准后，对11家参与研究的儿童医院（2009 - 2015年）所有接受手术治疗的肺畸形患者进行回顾性队列研究。
在506例肺畸形患者中，确诊为CLE的患儿有53例（10.5%）。与非CLE病例中的331例（73.1%）相比，13例（24.5%）CLE患儿在产前检测到肺部肿块（p < 0.0001）。32例（60.4%）CLE患儿出生时出现呼吸道症状，而非CLE患儿为102例（22.7%）（p < 0.0001）。CLE最常见的部位是左上叶（n = 24，45.3%）、右中叶（n = 16，30.2%）和右上叶（n = 10，18.9%）。18例（34.0%）在新生儿期接受了切除术，30例（56.6%）在1至12个月龄时接受了手术，5例（9.4%）在12个月后接受了切除术。6例（11.3%）接受了胸腔镜切除术。中位住院时间为5.0天（四分位间距，4.0 - 13.0）。
在肺畸形中，CLE具有一些独特特征，包括产前检测率低、好发于上叶/中叶以及胸腔镜使用率低。尽管出生时呼吸窘迫很常见，但CLE在临床上往往呈现延迟且更隐匿的表现形式。
三级。
