照井 慶太

OBJECTIVES: Respiratory management is crucial for patients with congenital diaphragmatic hernia (CDH). This prospective study aimed to assess the optimal initial ventilatory mode for CDH by comparing conventional mechanical ventilation (CMV) and high-frequency oscillatory ventilation (HFO). METHODS: Fifteen participating institutes used CMV or HFO as the initial ventilation mode for CDH, we thus divided them into two groups (CMV and HFO). Clinical data were prospectively collected from patients with isolated left CDH born at a gestational age ≥ 34 weeks between 2017 and 2021. The primary outcome was mortality to discharge, with non-inferiority analysis. We also performed analyses stratified by disease severity. RESULTS: Of the 247 patients, 124 received CMV, and 123 received HFO. The mortality rates for CMV and HFO were 11.3% and 15.4%, respectively, which met the definition of non-inferiority. We observed no significant differences in BPD (CMV, 41% vs. HFO, 42%) or the combined outcome of mortality or BPD (48% vs. 51%). Among patients with mild and moderate disease, CMV was associated with less frequent switching of the ventilation mode, shorter mechanical ventilation time, lower use of inhaled nitric oxide (iNO) therapy, and a shorter duration of iNO. In severe cases, HFO was associated with less frequent switching of the ventilation mode and a reduced need for ECMO. CONCLUSIONS: The initial ventilation mode in CDH patients does not influence mortality or BPD outcomes. However, this study suggests that CMV may be more beneficial for mild-to-moderate cases, while HFO may be more advantageous for severe cases.

BACKGROUND: To retrospectively investigate the developmental outcomes at 3 years of age in patients with congenital diaphragmatic hernia (CDH) using a multicenter collaborative research approach. METHODS: 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. RESULTS: 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). CONCLUSION: 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. IMPACT: 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.