Patient characteristics are important determinants of neurodevelopmental outcome during infancy in giant omphalocele
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Published on: August 16, 2024
Enrico Danzer1, Marsha Gerdes1, Jo Ann D'Agostino1
1The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA; The Perelman School of Medicine, The University of Pennsylvania, USA.
Insights
More than half of giant omphalocele survivors experience neurodevelopmental impairments. Disease severity, autism, and hypotonicity are linked to poorer outcomes, highlighting the need for early intervention in these complex cases.
Area of Science:
- Pediatric Surgery
- Neonatology
- Developmental Pediatrics
Background:
- Giant omphalocele (GO) is a complex congenital anomaly requiring significant medical intervention.
- Neurodevelopmental (ND) outcomes in GO survivors are not fully understood, necessitating investigation into predictive factors.
Purpose of the Study:
- To identify patient-specific factors that predict neurodevelopmental outcomes in children surviving giant omphalocele.
Main Methods:
- A cohort of 31 giant omphalocele survivors underwent neurodevelopmental assessment using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III).
- Neurodevelopmental delay was defined by BSID-III composite scores ≤84, and severe impairments by scores ≤69.
- Statistical analyses, including ANOVA, chi-square, and logistic regression, were used to correlate ND outcomes with patient-specific factors.
Main Results:
- Mean cognitive, language, and motor scores were in the low average to below-average range.
- 35% of survivors experienced severe neurodevelopmental delays in at least one domain.
- Prolonged ventilation, tracheostomy, pulmonary hypertension, delayed feeding, GERD, abnormal hearing screens, prolonged hospitalization, failure to thrive, autism, hypotonicity, delayed surgical repair, and older age at repair were associated with poorer ND outcomes.
Conclusions:
- Neurodevelopmental impairments are common in giant omphalocele survivors, affecting over half of the cohort.
- Disease severity, indicated by factors like prolonged ventilation and hospitalization, significantly impacts neurodevelopmental dysfunction.
- Autism and hypotonicity are frequent comorbidities associated with delays in cognitive, language, and motor function.
Objective:
To examine patient-specific factors as potential predictors of neurodevelopmental (ND) outcome in children with giant omphalocele (GO).
Materials:
Between 06/2005 and 07/2012, 31 consecutive GO survivors underwent ND assessment using the BSID-III at a median of 24months (range 6-35). ND delay was defined by a score of ≤84 in any composite score. Severe impairments were defined as a score of ≤69 in at least one domain. Correlations between ND outcome and patient-specific factors were analyzed by one-way ANOVA, chi-square, or logistic regression as appropriate.
Results:
The mean cognitive score (86.8±16.8) was in the low average range. Mean language (83.2±21.1) and motor (81.5±16.2) scores were below average. Forty-six-percent scored within the average range for all scales. Mild deficits were found in 19%, and 35% had severe delays in at least one domain. Hypotonicity was present in 55%. Autism was suspected/confirmed in 13%. Predictors of lower ND scores were prolonged ventilator support (P<0.01), high-frequency oscillatory ventilation (P<0.01), tracheostomy placement (P<0.001), O2 supplementation at day of life 30 (P<0.02), pulmonary hypertension (P<0.02), delayed enteral feeding (P=0.01), need for feeding tube (P<0.001), GERD (P=0.05), abnormal BAER hearing screen (P<0.006), prolonged hospitalization (P=0.01), and failure to thrive (P=0.001). Autism was associated with delays in cognitive and language outcomes (P<0.03). Delayed staged closure (P=0.007), older age at final repair (P=0.03), and hypotonicity (P=0.02) were associated with motor dysfunction.
Conclusions:
Neurological impairments were present in more than half of GO survivors. Disease severity was associated with ND dysfunction. Autism and hypotonicity were often co-morbidities with ND delays and poor motor function.
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