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Isolated congenital diaphragmatic hernia and three-year neurodevelopmental outcomes
Katsuaki Toyoshima1, Hirosato Aoki2, Kaoru Katsumata2
1Department of Neonatology, Kanagawa Children's Medical Center, Yokohama, Japan. nqf37179@nifty.com.
Insights
Congenital diaphragmatic hernia (CDH) survivors generally have good developmental outcomes. However, a low observed/expected lung area-to-head circumference ratio indicates a higher risk of neurodevelopmental impairment in children with CDH.
Area of Science:
- Pediatric Surgery
- Neonatology
- Developmental Pediatrics
Background:
- Congenital diaphragmatic hernia (CDH) is a serious birth defect.
- Understanding long-term developmental outcomes is crucial for affected infants.
Purpose of the Study:
- To investigate developmental outcomes at 3 years of age in patients with isolated CDH.
- To identify predictors of neurodevelopmental impairment in this cohort.
Main Methods:
- Retrospective analysis of 132 patients with isolated CDH from a Japanese multicenter registry (2010-2016).
- Developmental Quotient (DQ) assessed using the Kyoto Scale of Psychological Development 2001.
- Multivariate analysis to identify factors associated with DQ < 85.
Main Results:
- 75% of patients had a DQ ≥ 85, while 25% had scores below 85.
- The observed/expected lung area-to-head circumference ratio (o/e LHR) was an independent predictor of DQ < 85 (aOR 0.62; P=.03).
- Pulmonary hypoplasia, indicated by a lower o/e LHR, correlated with poorer neurodevelopmental outcomes.
Conclusions:
- Isolated CDH survivors typically exhibit good developmental outcomes.
- Pulmonary hypoplasia is a significant risk factor for neurodevelopmental impairment.
- The o/e LHR is a valuable tool for early risk stratification and guiding follow-up care in CDH patients.
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.
