Patient characteristics are important determinants of neurodevelopmental outcome during infancy in giant omphalocele.

Abstract

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.

• Authors: Enrico Danzer, Marsha Gerdes, Jo Ann D'Agostino, Judy Bernbaum, Casey Hoffman, Natalie E Rintoul, Lisa M Herkert, Alan W Flake, N Scott Adzick, Holly L Hedrick
• Journal: Early human development (Early Hum Dev) Vol. 91 Issue 3 Pg. 187-93 (Mar 2015) ISSN: 1872-6232 [Electronic] Ireland
• PMID: 25676186 (Publication Type: Journal Article)
• Copyright: Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Topics

• Child Development
• Developmental Disabilities (epidemiology, etiology)
• Female
• Hernia, Umbilical (complications)
• Humans
• Infant
• Infant, Newborn
• Male