The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
J Perinatol. 2013 Nov;33(11):893-8. doi: 10.1038/jp.2013.47. Epub 2013 May 9.
The objective of this study was to longitudinally evaluate the neurodevelopmental (ND) outcome in congenital diaphragmatic hernia (CDH) survivors during the first 3 years of life.
The study cohort consists of 47 CDH survivors that were enrolled in our prospective, follow-up program between July 2004 and September 2010, and underwent serial ND evaluations during the first 3 years of life. ND outcomes were evaluated using the Bayley Scales of Infant Development (BSID)-II or BSID-III. Persistent ND impairment was defined as a score that remained 79 for the cognitive, language and psychomotor domains at the most recent follow-up visit compared with the first assessment.
The median age at first and last evaluation was 8 (range, 5 to 15) and 29 (range, 23 to 36) months, respectively. During the follow-up, ND scores improved to average in 17%, remained average in 60%, remained delayed in 10%, improved from severely delayed to mildly delayed in 2% and deteriorated from average to delayed in 15%. Motor scores improved to average in 26%, remained average in 55%, remained delayed in 8% and improved from severely delayed to mildly delayed in 11%. Intrathoracic liver position (P=0.004), preterm delivery (P=0.03), supplemental O2 requirement at day of life 30 (P=0.007), age at discharge (P=0.03), periventricular leukomalacia (PVL; P=0.004) and initial neuromuscular hypotonicity (P=0.01) were associated with persistent motor delays. No relationship was found between patient's characteristics and the risk of persistent cognitive and language delays.
(1) The majority of children with CDH are functioning in the average range by early preschool age, (2) most children who had early delays showed improvement in their ND outcome, (3) children showing delays in all the three domains were the least likely to show improvement and (4) CDH severity appears to be predictive of persistent psychomotor delays.
本研究旨在对先天性膈疝（CDH）患儿进行为期 3 年的纵向神经发育（ND）评估。
研究队列由 2004 年 7 月至 2010 年 9 月期间入组我们前瞻性随访计划的 47 例 CDH 幸存者组成，并在生命的前 3 年中进行了系列 ND 评估。使用贝利婴幼儿发展量表（BSID）-II 或 BSID-III 评估 ND 结果。持续性 ND 损害定义为与首次评估相比，最近随访时认知、语言和运动领域的评分仍＜79。
中位数首次和末次评估年龄分别为 8（5 至 15）和 29（23 至 36）个月。在随访期间，17%的患儿 ND 评分提高至平均水平，60%的患儿保持平均水平，10%的患儿持续延迟，2%的患儿从严重延迟改善为轻度延迟，15%的患儿从平均水平恶化至延迟。运动评分提高至平均水平的患儿占 26%，保持平均水平的患儿占 55%，持续延迟的患儿占 8%，从严重延迟改善为轻度延迟的患儿占 11%。胸腔内肝脏位置（P=0.004）、早产（P=0.03）、生后 30 天补充氧（P=0.007）、出院年龄（P=0.03）、脑室周围白质软化（PVL；P=0.004）和初始神经肌肉张力低下（P=0.01）与持续性运动障碍相关。患者特征与认知和语言障碍持续风险之间无相关性。
（1）大多数 CDH 患儿在学龄前早期功能处于平均水平，（2）大多数早期出现发育迟缓的患儿 ND 预后有所改善，（3）在所有三个领域均出现发育迟缓的患儿最不可能改善，（4）CDH 严重程度似乎与持续性精神运动障碍相关。
