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. 2020 Jul;55(7):1313-1318. doi: 10.1016/j.jpedsurg.2019.01.058. Epub 2019 Feb 28.
The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection.
After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative.
Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1-12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6-11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%).
This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients.
Level II.
本研究旨在建立一个多机构注册系统，以描述一组接受先天性肺畸形（CLM）切除术的当代患儿的人口统计学、管理和结局特征。
在中央机构审查委员会批准后，建立了一个基于网络的安全数据库，以收集 2009 年至 2015 年期间在一个多机构研究合作机构内进行的经病理证实的 CLM 的回顾性队列数据。
11 家儿童医院贡献了 506 例患者。在 344 例产前诊断的病变中，49.1%测量了先天性肺气道畸形体积比，34.3%进行了胎儿 MRI。134 例（26.7%）患儿出生时有呼吸症状。58 例（11.6%）患儿接受了新生儿切除术，322 例（64.1%）患儿在 1-12 个月行手术，122 例（24.3%）患儿在 12 个月后行手术。切除的中位年龄为 6.7 个月（四分位距，3.6-11.4）。在 230 例无症状患者的选择性肺叶切除术中，102 例（44.3%）成功采用了胸腔镜，但各中心之间存在很大差异。最常见的病变是先天性肺气道畸形（n=234，47.3%）和叶内支气管肺隔离症（n=106，21.4%）。
这项关于手术 CLM 的多中心队列研究强调了术前评估和手术管理方面的显著疾病异质性和大量实践差异。未来的注册研究计划有助于制定循证指南，以优化这些患者的治疗。
II 级。
