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先天性膈疝幸存者的神经发育结局:单机构经验

Neurodevelopmental outcomes in CDH survivors: A single institution's experience.

作者信息

Church Joseph T, Mon Rodrigo, Wright Tiffany, Coughlin Megan A, Ladino-Torres Maria, Tapley Christopher, Bowen Heather, Matusko Niki, Mychaliska George B

机构信息

Section of Pediatric Surgery, Department of Surgery, Michigan Medicine, Ann Arbor, MI.

Section of Pediatric Surgery, Department of Surgery, Michigan Medicine, Ann Arbor, MI.

出版信息

J Pediatr Surg. 2018 Jun;53(6):1087-1091. doi: 10.1016/j.jpedsurg.2018.02.064. Epub 2018 Mar 5.

DOI:10.1016/j.jpedsurg.2018.02.064
PMID:29580786
Abstract

PURPOSE

Survivors of congenital diaphragmatic hernia (CDH) face high morbidity. We studied the neurodevelopmental outcomes of CDH survivors at a single institution.

METHODS

CDH survivors born July 2006-March 2016 at a free-standing children's hospital were reviewed. Neurodevelopment was assessed using the Peabody Developmental Motor Scales (PDMS-2) broken into gross, fine, and total motor quotients. Data collected included prenatal variables (liver herniation, defect laterality, observed:expected total fetal lung volume (o:eTFLV) on MRI), birth demographics (sex, race, estimated gestational age (EGA), birth weight (BtWt), 5 min APGAR, associated anomalies), and therapies/hospital course (HFOV/HFJV, ECMO, timing of repair, pulmonary hypertension (PHTN) severity, length of stay, ventilator days). Variables were analyzed using mixed linear modeling.

RESULTS

Sixty-eight children were included. Most patients had left-sided CDH (55/68, 81%) without liver herniation (42/68, 62%). ECMO utilization was 25/68 (37%). The mean [95% confidence interval] gross motor quotient for the entire cohort was 87 [84-91], fine motor quotient was 92 [88-96], and total motor quotient was 88 [84-93], representing below average, average, and below average functioning, respectively. o:eTFLV predicted fine motor quotient among prenatal variables. Associated anomalies and ECMO use predicted all quotients in the final model.

CONCLUSIONS

Associated anomalies and ECMO use predict neurodevelopmental delay in CDH survivors.

TYPE OF STUDY

Retrospective observational study; Prognostic.

LEVEL OF EVIDENCE

II.

摘要

目的

先天性膈疝(CDH)幸存者面临着较高的发病率。我们在单一机构研究了CDH幸存者的神经发育结局。

方法

回顾性分析2006年7月至2016年3月在一家独立儿童医院出生的CDH幸存者。使用皮博迪发育运动量表(PDMS-2)评估神经发育情况,该量表分为粗大运动商、精细运动商和总运动商。收集的数据包括产前变量(肝脏疝出、缺损侧别、MRI上观察到的预期总胎儿肺体积(o:eTFLV))、出生人口统计学特征(性别、种族、估计孕周(EGA)、出生体重(BtWt)、5分钟阿氏评分、相关畸形)以及治疗/住院过程(高频振荡通气/高频喷射通气(HFOV/HFJV)、体外膜肺氧合(ECMO)、修复时机、肺动脉高压(PHTN)严重程度、住院时间、呼吸机使用天数)。使用混合线性模型分析变量。

结果

纳入68名儿童。大多数患者为左侧CDH(55/68,81%)且无肝脏疝出(42/68,62%)。ECMO使用率为25/68(37%)。整个队列的平均[95%置信区间]粗大运动商为87[84 - 91],精细运动商为92[88 - 96],总运动商为88[84 - 93],分别代表低于平均水平、平均水平和低于平均水平的功能。在产前变量中,o:eTFLV可预测精细运动商。相关畸形和ECMO的使用在最终模型中可预测所有商数。

结论

相关畸形和ECMO的使用可预测CDH幸存者的神经发育延迟。

研究类型

回顾性观察研究;预后研究。

证据水平

II级。

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