title: 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).
  
  
  METHODS
  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: The 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. Electronic address: danzere@email.chop.edu.
authors: Enrico
authors: E
authors: Danzer
authors: The 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.
authors: Marsha
authors: M
authors: Gerdes
authors: The 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.
authors: Jo Ann
authors: JA
authors: D'Agostino
authors: The 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.
authors: Judy
authors: J
authors: Bernbaum
authors: The 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.
authors: Casey
authors: C
authors: Hoffman
authors: The 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.
authors: Natalie E
authors: NE
authors: Rintoul
authors: The 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.
authors: Lisa M
authors: LM
authors: Herkert
authors: The 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.
authors: Alan W
authors: AW
authors: Flake
authors: The 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.
authors: N Scott
authors: NS
authors: Adzick
authors: The 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.
authors: Holly L
authors: HL
authors: Hedrick
mesh_terms: D002657:Child Development; D002658:Developmental Disabilities / Q000453:epidemiology / Q000209:etiology*; D005260:Female; D006554:Hernia, Umbilical / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male
keywords: Autism; Bayley Scales of Infant Development; Giant omphalocele; Neurodevelopmental outcome; Pulmonary hypoplasia