Neurodevelopmental outcomes at 5years of age in congenital diaphragmatic hernia.
OBJECTIVE
To evaluate neurodevelopmental sequelae in congenital diaphragmatic hernia (CDH) children at 5years of age.


METHODS
The study cohort of 35 CDH patients was enrolled in our follow-up program between 06/2004 and 09/2014. The neurodevelopmental outcomes assessed at a median of 5years (range, 4-6) included cognition (Wechsler Preschool and Primary Scale of Intelligence [WPPSI], n=35), Visual-Motor-Integration (n=35), academic achievement (Woodcock-Johnson Tests of Achievement, n=25), and behavior problems (Child Behavior Check List [CBCL], n=26). Scores were grouped as average, borderline, or extremely low by SD intervals.


RESULTS
Although mean Full (93.9±19.4), Verbal (93.4±18.4), and Performance (95.2±20.9) IQ were within the expected range, significantly more CDH children had borderline (17%) and extremely low (17%) scores in at least one domain compared to normative cohorts (P<0.02). The Visual-Motor-Integration score was below population average (P<0.001). Academic achievement scores were similar to expected means for those children who were able to complete testing. CBCL scores for the emotionally reactive (23%) and pervasive developmental problems scales (27%) were more likely to be abnormal compared to normal population scores (P=0.02 and P=0.0003, respectively). Autism was diagnosed in 11%, which is significantly higher than the general population (P<0.01). Univariate analysis suggests that prolonged NICU stay, prolonged intubation, tracheostomy placement, pulmonary hypertension, autism, hearing impairment, and developmental delays identified during infancy are associated with worse cognitive outcomes (P<0.05).


CONCLUSIONS
The majority of CDH children have neurodevelopmental outcomes within the average range at 5years of age. However, rates of borderline and extremely low IQ scores are significantly higher than in the general population. CDH survivors are also at increased risk for developing symptoms of emotionally reactive and pervasive developmental problems. Risk of autism is significantly elevated. Disease severity and early neurological dysfunction appear to be predictive of longer-term impairments.
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. Electronic address: danzere@email.chop.edu.
Enrico
E
Danzer
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Casey
C
Hoffman
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Jo Ann
JA
D'Agostino
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Marsha
M
Gerdes
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Judy
J
Bernbaum
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Ryan M
RM
Antiel
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Natalie E
NE
Rintoul
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Lisa M
LM
Herkert
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Alan W
AW
Flake
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
N Scott
NS
Adzick
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Holly L
HL
Hedrick
D000067290:Behavior Rating Scale; D002648:Child; D002657:Child Development; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications*; D006801:Humans; D008297:Male; D065886:Neurodevelopmental Disorders / Q000209:etiology*; D009460:Neurologic Examination; D012189:Retrospective Studies; D012306:Risk; D012720:Severity of Illness Index; D013997:Time Factors
Autism; Behavioral problems; Congenital diaphragmatic hernia; Neurodevelopmental outcome; Pulmonary hypertension; Pulmonary hypoplasia; Socioeconomic status