Fetal Program, Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, MD.
Division of Pediatric Surgery, Department of Surgery,Washington University School of Medicine, St. Louis, MO.
Ann Surg. 2022 Nov 1;276(5):e622-e630. doi: 10.1097/SLA.0000000000004566. Epub 2020 Nov 17.
The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes.
Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined.
A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression.
Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection ( P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth.
In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.
本研究旨在评估产前诊断为先天性肺畸形（CLM）的儿童的临床结局，并确定与不良结局相关的产前特征。
尽管疾病的临床表现广泛，但对于产前 CLM 亚组中哪些亚组在分娩时更易发生水肿和呼吸窘迫，目前尚未明确。
对 2009 年至 2016 年间 11 家儿童医院的产前诊断为 CLM 的手术数据库进行了回顾性队列研究。使用非参数双变量或多变量逻辑回归进行统计分析。
共分析了 344 名儿童。15 名（5.5%）胎儿在存在水肿的情况下接受了母亲皮质类固醇治疗，且产前手术干预并不常见（1.7%）。75 名（21.8%）患儿在出生时有呼吸症状，34 名（10.0%）需要新生儿肺切除术。169 例（49.1%）患儿记录了先天性肺气道畸形体积比（CVR）测量值，该值与围产期结局显著相关，包括水肿、出生时呼吸窘迫、需要补充氧气、新生儿呼吸机使用和新生儿切除术（P<0.001）。初始 CVR≤1.4 与降低水肿风险显著相关（AUC，0.93；95%置信区间（CI），0.87-1.00）。最大 CVR<0.9（AUC，0.72；95%CI，0.67-0.85）与出生时无呼吸症状相关。
在这项大型多机构研究中，初始 CVR≤1.4 可识别出发生水肿风险极低的胎儿，而最大 CVR<0.9 与出生时无症状疾病相关。这些发现为产前咨询和分娩计划的标准化和质量改进提供了机会。
