title: Preschool neurological assessment in congenital diaphragmatic hernia survivors: outcome and perinatal factors associated with neurodevelopmental impairment.
abstract: OBJECTIVE
  To evaluate the preschool neurodevelopmental outcome in CDH survivors between 2 and 5 years of age and to identify perinatal and postnatal factors that may be predictive of persistent neurological deficits.
  
  
  METHODS
  The study cohort consists of 60 CDH survivors that were enrolled in our prospective, follow-up program between January 2006 and September 2012. Developmental assessment of study participants between 2 and 3 years of age was performed using the Bayley Scales of Infant Development, 3rd Edition (BSID-III). Cognitive outcomes of CDH children older than 3 years of age were evaluated using The Wechsler Preschool and Primary Scale of Intelligence, 3rd Edition (WPPSI-III). Neurodevelopmental delay was defined by a score of ≤ 85 in any of the evaluated composite scores. Severe impairments were defined as a score of ≤ 69 in at least one domain tested.
  
  
  RESULTS
  Mean follow-up for children assessed by BSID-III (n=42) and WPPSI-III (n=18) was 28 ± 4.5 months and 58 ± 4.0 months, respectively. As a group, mean composite and subdomain BSID-III and WPPSI-III scores were within the expected normal range. For the BSID-III group, 9 (22%) had mild deficits in at least one domain, and 6 (14%) had severe deficits in at least one. Only 3 (7%) patients demonstrated severe neurological delays for all BSID-III composite scales. For the WPPSI-III group, 4 (22%) patients scored within the borderline-delayed range for at least one subscale and only one (6%) child had a WPPSI-III VIQ score in the severe delay range. Abnormal muscle tonicity was found in 35% (hypotonicity 33%, hypertonicity 2%). Need for ECMO, prolonged ventilation, hypotonicity, and other surrogate markers of disease severity (P<0.05) were associated with borderline or delayed neurological outcome.
  
  
  CONCLUSIONS
  The majority of CDH children are functioning in the average range at early preschool and preschool age. Neuromuscular hypotonicity is common in CDH survivors. CDH severity appears to be predictive of adverse neurodevelopmental outcome.
authors: The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
authors: Enrico
authors: E
authors: Danzer
authors: Marsha
authors: M
authors: Gerdes
authors: Jo Ann
authors: JA
authors: D'Agostino
authors: Emily A
authors: EA
authors: Partridge
authors: Casey H
authors: CH
authors: Hoffman-Craven
authors: Judy
authors: J
authors: Bernbaum
authors: Natalie E
authors: NE
authors: Rintoul
authors: Alan W
authors: AW
authors: Flake
authors: N Scott
authors: NS
authors: Adzick
authors: Holly L
authors: HL
authors: Hedrick
mesh_terms: D002675:Child, Preschool; D002658:Developmental Disabilities / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009123:Muscle Hypotonia / Q000209:etiology; D009460:Neurologic Examination; D011446:Prospective Studies; D017741:Survivors; D016896:Treatment Outcome