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Published on: February 5, 2021
Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an
Enrico Danzer1, Marsha Gerdes, Judy Bernbaum
1The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104-4318, USA.
Insights
Congenital diaphragmatic hernia (CDH) survivors often experience neurodevelopmental issues, including hypotonicity and psychomotor delays. Patient-specific factors significantly impact these adverse outcomes.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Developmental Pediatrics
Background:
- Congenital diaphragmatic hernia (CDH) is a serious condition affecting infant development.
- Long-term neurodevelopmental outcomes in CDH survivors require comprehensive evaluation.
Purpose of the Study:
- To assess the neurodevelopmental status of infants surviving congenital diaphragmatic hernia (CDH).
Main Methods:
- Prospective enrollment of 41 CDH survivors into an interdisciplinary follow-up program.
- Neurodevelopmental assessment using Bayley Scales of Infant Development II/III or Wechsler Preschool and Primary Scale of Intelligence III.
- Categorization of scores into average, delayed, and mixed outcomes based on standard deviations.
Main Results:
- Nearly half of CDH infants showed delayed neurocognitive and language skills (49% average, 19% mixed, 17% mild delay, 15% severe delay).
- Psychomotor scores indicated normal development in 46%, mild delay in 23%, and severe delay in 31%.
- Risk factors for adverse outcomes included intrathoracic liver position, right-sided CDH, ECMO use, Gore-Tex repair, oxygen need, and hypotonicity.
Conclusions:
- Interdisciplinary follow-up reveals significant neurodevelopmental morbidities in approximately 50% of CDH infants.
- Neuromuscular hypotonicity and psychomotor dysfunction are the most frequent neurologic sequelae.
- Individual patient factors are critical predictors of negative neurologic outcomes in CDH survivors.
Purpose:
The purpose of the study was to evaluate the neurodevelopmental outcome in infants with congenital diaphragmatic hernia (CDH).
Methods:
Between June 2004 and September 2007, 41 CDH survivors were prospectively enrolled in an interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development II (prior 2006, n = 9), the Bayley Scales of Infant Development III (after 2006, n = 27), or the Wechsler Preschool and Primary Scale of Intelligence III (children older than 4 years, n = 5). Scores were grouped as average, mildly delayed, and severely delayed by standard deviation intervals (115-85, 71-84, <70), and mixed if average and mildly delayed in either cognitive or language.
Results:
Median age at last assessment was 24 months (range, 6-62). Average, mixed, mildly delayed, and severely delayed scores for neurocognitive and language skills were found in 49%, 19%, 17%, and 15%, respectively. Psychomotor scores were normal, mildly delayed, and severely delayed in 46%, 23%, and 31%, respectively. Autism was present in 7%. Abnormal muscle tonicity was found in 51% (49% hypotonic, 2% hypertonic). Multivariate risk factors for borderline or delayed neurodevelopmental, neurocognitive, and/or psychomotor outcome were intrathoracic liver position (P = .02), presence of a right-sided CDH (P = .02), extracorporeal membrane oxygenation need (P < .001), Gore-Tex patch repair (P = .02), O(2) requirement at 30 days of life (P < .01), and hypotonicity (P < .01).
Conclusions:
The prospective evaluation in an interdisciplinary follow-up program uncovered striking morbidities in neurodevelopmental status in approximately half of the CDH infants. The most common neurologic sequelae are neuromuscular hypotonicity and psychomotor dysfunction. Patient-specific factors are important determinants of adverse neurologic outcome.
