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Neurodevelopmental outcomes in children with isolated congenital diaphragmatic hernia: A systematic review and
Lennart Van der Veeken1,2, Simen Vergote1,2, Yada Kunpalin1,3
1Department of Development and Regeneration, Cluster Woman and Child, Group Biomedical Sciences, KU Leuven University of Leuven, Leuven, Belgium.
Insights
Neurodevelopmental delay affects 16% of children with isolated congenital diaphragmatic hernia (CDH), primarily due to motor deficits. Further research on isolated CDH and fetal surgery is recommended for better patient counseling.
Area of Science:
- Pediatric Surgery
- Neonatal Neurology
- Developmental Pediatrics
Background:
- Congenital diaphragmatic hernia (CDH) is known to have neurological effects.
- The specific impact of isolated CDH on neurodevelopment requires further investigation.
Purpose of the Study:
- To systematically review and quantify the risk of neurodevelopmental complications in infants with isolated CDH.
- To identify primary and secondary neurological outcomes in this population.
Main Methods:
- Systematic literature review of studies reporting neurological outcomes in infants with isolated CDH.
- Primary outcome: neurodevelopmental delay. Secondary outcomes: motor skills, intelligence, vision, hearing, language, and behavior.
- Included data from 13 studies encompassing 2624 children with isolated CDH.
Main Results:
- Neurodevelopmental delay was reported in 16% of children with isolated CDH.
- Motor problems were the most common issue (13%), followed by cognitive dysfunction (5%) and hearing deficits (3%).
- Studies including both isolated and non-isolated CDH cases reported higher complication rates.
Conclusions:
- A significant minority of children with isolated CDH experience neurodevelopmental delay.
- Current research predominantly focuses on non-isolated CDH cases.
- More studies are needed on isolated CDH, particularly those involving fetal surgery, to improve patient counseling.
Background:
Congenital diaphragmatic hernia (CDH) reportedly has neurologic consequences in childhood however little is known about the impact in isolated CDH.
Aims:
Herein we aimed to describe the risk of neurodevelopmental complications in children born with isolated CDH.
Materials & Methods:
We systematically reviewed literature for reports on the neurological outcome of infants born with isolated CDH. The primary outcome was neurodevelopmental delay. Secondary outcomes included, motor skills, intelligence, vision, hearing, language and behavior abnormalities.
Results:
Thirteen out of 87 (15%) studies reported on isolated CDH, including 2624 out of 24,146 children. Neurodevelopmental delay was investigated in four studies and found to be present in 16% (3-34%) of children. This was mainly attributed to motor problems in 13% (2-30%), whereas cognitive dysfunction only in 5% (0-20%) and hearing in 3% (1-7%). One study assessed the effect of fetal surgery. When both isolated and non-isolated children were included, these numbers were higher.
Discussion:
This systematic review demonstrates that only a minority of studies focused on isolated CDH, with neurodevelopmental delay present in 16% of children born with CDH.
Conclusion:
To accurately counsel patients, more research should focus on isolated CDH cases and examine children that underwent fetal surgery.

