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Published on: February 5, 2021
Neurodevelopmental and neurofunctional outcomes in children with congenital diaphragmatic hernia
Enrico Danzer1, Holly L Hedrick
1The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, PA 1910, USA. danzere@email.chop.edu
Insights
Congenital diaphragmatic hernia (CDH) survivors often experience neurodevelopmental issues, including motor, behavioral, and cognitive challenges. Early identification and interdisciplinary care are crucial for managing these long-term outcomes.
Area of Science:
- Pediatric Surgery
- Developmental Neuroscience
- Neonatology
Background:
- Congenital diaphragmatic hernia (CDH) is a serious birth defect with significant implications for infant survival and long-term health.
- Advances in medical and surgical care have improved survival rates for CDH, shifting focus towards neurodevelopmental outcomes.
- Neurodevelopmental dysfunction is a common and potentially disabling consequence of CDH and its treatment.
Purpose of the Study:
- To critically review current knowledge on neurocognitive, neuromotor, and neurobehavioral development in patients with CDH.
- To examine comprehensive outcome studies, identify predictors of adverse outcomes, and explore pathophysiological mechanisms.
- To inform targeted interventions and establish standard care protocols for CDH patients.
Main Methods:
- Literature review of comprehensive outcome studies in congenital diaphragmatic hernia.
- Analysis of patient and disease-specific predictors for neurodevelopmental outcomes.
- Examination of imaging studies and pathophysiological pathways related to brain abnormalities.
Main Results:
- Neurodevelopmental dysfunction is the most common outcome in CDH patients, manifesting as neuromotor issues in infancy and behavioral/cognitive problems later.
- Intelligence generally falls within the low normal range.
- Structural brain abnormalities are frequently observed on imaging, and specific predictors of adverse outcomes have been identified.
Conclusions:
- Understanding the pathophysiological basis of neurodevelopmental disabilities in CDH is key to developing targeted therapies.
- Continuous, interdisciplinary assessment and follow-up are essential for early identification and management of morbidities.
- Establishing standard care protocols can reduce adverse neurodevelopmental outcomes and improve the quality of life for children with CDH.
Abstract:
The objective of this review was to provide a critical overview of our current understanding on the neurocognitive, neuromotor, and neurobehavioral development in congenital diaphragmatic hernia (CDH) patients, focusing on three interrelated clinical issues: (1) comprehensive outcome studies, (2) characterization of important predictors of adverse outcome, and (3) the pathophysiological mechanism contributing to neurodevelopmental disabilities in infants with CDH. Improved survival for CDH has led to an increasing focus on longer-term outcomes. Neurodevelopmental dysfunction has been recognized as the most common and potentially most disabling outcome of CDH and its treatment. While increased neuromotor dysfunction is a common problem during infancy, behavioral problems, hearing impairment and quality of life related issues are frequently found in older children and adolescence. Intelligence appears to be in the low normal range. Patient and disease specific predictors of adverse neurodevelopmental outcome have been defined. Imaging studies have revealed a high incidence of structural brain abnormalities. An improved understanding of the pathophysiological pathways and the neurodevelopmental consequences will allow earlier and possibly more targeted therapeutic interventions. Continuous assessment and follow-up as provided by an interdisciplinary team of medical, surgical and developmental specialists should become standard of care for all CDH children to identify and treat morbidities before additional disabilities evolve and to reduce adverse outcomes.
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