Department of Pediatric Surgery, University of Texas McGovern Medical School and Children's Memorial Hermann Hospital, Houston, TX.
Center for Surgical Trials and Evidence-based Practice, University of Texas McGovern Medical School, Houston, TX.
Ann Surg. 2018 May;267(5):977-982. doi: 10.1097/SLA.0000000000002144.
The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate.
Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable.
Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA.
A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P < 0.05) and increased incidence of anomalies (P < 0.0001). Low-volume centers ("Lo", n=44 total, < 10 CDH pts/yr) and high-volume centers ("Hi", n = 21) had median nonrepair rates of 19.8% (range 0%-66.7%) and 16.7% (5.1%-38.5%), respectively. High-volume centers were further dichotomized by rate of nonrepair (HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P < 0.0001). For every 100 CDH patients, HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation.
There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.
本研究的目的为：（i）评估未接受修复的先天性膈疝（CDH）患儿；（ii）确定各机构的未修复率；（iii）根据未修复率比较各机构的结局。
约 20%的 CDH 患儿未接受修复，且手术修复的阈值存在差异。
从多中心前瞻性收集的数据库中提取数据。分析了包括修复（或未修复）和结局在内的标准临床变量。根据未修复率将机构分为低容量组（“Lo”，总共 44 例，每年 < 10 例 CDH 患儿）和高容量组（“Hi”，总共 21 例）。未修复患者的 Apgar 评分较低（P < 0.05），且畸形发生率较高（P < 0.0001）。Lo 组和 Hi 组的未修复率中位数分别为 19.8%（范围 0%-66.7%）和 16.7%（5.1%-38.5%）。进一步将高容量中心按照未修复率分为低-中组（HiLo：5.1-16.7%）和高-中组（HiHi：17.6-38.5%），共 3 组：HiLo、HiHi 和 Lo。低出生体重、低 Apgar 评分、产前诊断和存在先天性畸形是死亡的预测因素。HiLo、HiHi 和 Lo 组的死亡率 O/E 比值分别为 0.81、0.94 和 1.21（P < 0.0001）。与预期相比，HiLo 组每 100 例 CDH 患儿中有 2.73 例（2.4-3.1，95%置信区间）存活。
接受修复和未接受修复的 CDH 患儿之间存在显著差异，且未修复率在各机构间存在显著差异。积极的手术治疗，导致低未修复率，与风险调整死亡率的改善相关。
