Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program.
OBJECTIVE
The purpose of the study was to evaluate the neurodevelopmental outcome in infants with congenital diaphragmatic hernia (CDH).


METHODS
Between June 2004 and September 2007, 41 CDH survivors were prospectively enrolled in an interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development II (prior 2006, n = 9), the Bayley Scales of Infant Development III (after 2006, n = 27), or the Wechsler Preschool and Primary Scale of Intelligence III (children older than 4 years, n = 5). Scores were grouped as average, mildly delayed, and severely delayed by standard deviation intervals (115-85, 71-84, <70), and mixed if average and mildly delayed in either cognitive or language.


RESULTS
Median age at last assessment was 24 months (range, 6-62). Average, mixed, mildly delayed, and severely delayed scores for neurocognitive and language skills were found in 49%, 19%, 17%, and 15%, respectively. Psychomotor scores were normal, mildly delayed, and severely delayed in 46%, 23%, and 31%, respectively. Autism was present in 7%. Abnormal muscle tonicity was found in 51% (49% hypotonic, 2% hypertonic). Multivariate risk factors for borderline or delayed neurodevelopmental, neurocognitive, and/or psychomotor outcome were intrathoracic liver position (P = .02), presence of a right-sided CDH (P = .02), extracorporeal membrane oxygenation need (P < .001), Gore-Tex patch repair (P = .02), O(2) requirement at 30 days of life (P < .01), and hypotonicity (P < .01).


CONCLUSIONS
The prospective evaluation in an interdisciplinary follow-up program uncovered striking morbidities in neurodevelopmental status in approximately half of the CDH infants. The most common neurologic sequelae are neuromuscular hypotonicity and psychomotor dysfunction. Patient-specific factors are important determinants of adverse neurologic outcome.
Danzer
Enrico
E
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104-4318, USA.
Gerdes
Marsha
M
Bernbaum
Judy
J
D'Agostino
Joann
J
Bebbington
Michael W
MW
Siegle
Jennifer
J
Hoffman
Casey
C
Rintoul
Natalie E
NE
Flake
Alan W
AW
Adzick
N Scott
NS
Hedrick
Holly L
HL
D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006548:Hernia, Diaphragmatic / Q000150:complications*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D008297:Male; D001523:Mental Disorders / Q000150:complications*; D009422:Nervous System Diseases / Q000150:complications*; D011446:Prospective Studies