Toyoshima Katsuaki, Aoki Hirosato, Katsumata Kaoru, Sato Yoshiaki, Inoue Hirosuke, Ito Miharu, Amari Shoichiro, Maruyama Hidehiko, Arahori Hitomi, Kondo Takuya, Kim Kiyokazu, Yamoto Masaya, Saito Tomoko, Okuyama Hiroomi, Usui Noriaki, Terui Keita, Nagata Kouji
Department of Neonatology, Kanagawa Children's Medical Center, Yokohama, Japan.
Division of Neonatology, Center for Maternal-Neonatal Care, Nagoya University Hospital, Nagoya, Japan.
Pediatr Res. 2025 Jan 23. doi: 10.1038/s41390-025-03870-z.
To retrospectively investigate the developmental outcomes at 3 years of age in patients with congenital diaphragmatic hernia (CDH) using a multicenter collaborative research approach.
We evaluated patients with CDH and no other malformations born between 2010 and 2016 in seven facilities in the Japanese CDH Research Group. The developmental quotient (DQ) at 3 years of age was evaluated using the Kyoto Scale of Psychological Development 2001, the most standardized scale in Japan. Factors associated with a DQ score < 85 were also analyzed.
Of 196 patients, developmental assessments at 3 years of age were performed in 132 patients (67%). Among these, 99 patients (75%) had a DQ score ≥ 85, 25 (19%) had a DQ score between 70 and 84, and 8 (6%) had a DQ score < 70. Multivariate analysis showed that the observed/expected lung area-to-head circumference ratio was an independent predictor of a DQ score < 85, with an adjusted odds ratio of 0.62 (95% confidence interval: 0.40-0.96; p = 0.03).
Generally, isolated CDH is associated with good developmental outcomes for survivors, even after intensive care. However, there is a risk of neurodevelopmental impairment if pulmonary hypoplasia is present.
This research highlights the observed/expected lung area-to-head circumference ratio (o/e LHR) as a crucial indicator to predict neurodevelopmental outcomes in 3-year-old children diagnosed with isolated congenital diaphragmatic hernia (CDH). Our results provide robust evidence from a large multicenter cohort, emphasizing the importance of o/e LHR in early risk stratification and prolonged neurodevelopmental follow-up. These findings highlight the need for comprehensive management and tailored follow-up care in CDH patients, potentially improving clinical protocols and enhancing the quality of life and outcomes for affected children.
采用多中心协作研究方法，回顾性调查先天性膈疝（CDH）患者3岁时的发育结局。
我们评估了日本CDH研究组7个机构中2010年至2016年间出生的无其他畸形的CDH患者。使用日本最标准化的量表《京都心理发展量表2001》评估3岁时的发育商（DQ）。还分析了与DQ评分<85相关的因素。
196例患者中，132例（67%）进行了3岁时的发育评估。其中，99例（75%）的DQ评分≥85，25例（19%）的DQ评分在70至84之间，8例（6%）的DQ评分<70。多因素分析显示，观察到的/预期的肺面积与头围之比是DQ评分<85的独立预测因素，调整后的优势比为0.62（95%置信区间：0.40-0.96；p = 0.03）。
一般来说，即使经过重症监护，孤立性CDH幸存者的发育结局良好。然而，如果存在肺发育不全，则有神经发育受损的风险。
本研究强调观察到的/预期的肺面积与头围之比（o/e LHR）是预测诊断为孤立性先天性膈疝（CDH）的3岁儿童神经发育结局的关键指标。我们的结果提供了来自大型多中心队列的有力证据，强调了o/e LHR在早期风险分层和长期神经发育随访中的重要性。这些发现凸显了对CDH患者进行全面管理和量身定制随访护理的必要性，可能改善临床方案并提高受影响儿童的生活质量和结局。
