Neurodevelopmental outcomes of congenital diaphragmatic hernia survivors followed in a multidisciplinary clinic at ages 1 and 3.
OBJECTIVE
Infants who survive congenital diaphragmatic hernia (CDH) repair may have ongoing medical and neurodevelopmental morbidity after hospital discharge. We evaluated the relationship between medical and neurodevelopmental outcomes of CDH survivors seen in a multidisciplinary clinic at ages 1 and/or 3.


METHODS
From January 1997 to December 2004, 69 (61%) of 112 CDH survivors were followed in our CDH clinic at ages 1 and/or 3. Medical issues (cardiac, pulmonary, gastrointestinal) were tabulated at hospital discharge and at follow-up. Neurodevelopmental data were obtained from clinic assessments by a neurodevelopmental pediatrician. Descriptive results were summarized for each cohort. Multivariate analyses were performed to identify predictors of motor problems at age 1.


RESULTS
Of the 69 study participants, 64% were male, 75% had left-sided CDH, 17% had cardiac anomalies, and 25% had other congenital malformations. Nearly all required ventilator management (99%) with a median ventilator time of 14 days (range, 1-54 days); 30% required extracorporeal membrane oxygenation. While 87% of patients had medical issues at hospital discharge, 61% and 67% had medical issues at ages 1 and 3, respectively. Pulmonary problems were noted in 34% and 33% of the ages 1 and 3 cohorts, respectively. Motor and language problems were detected in 60% and 18% of the age 1 cohort and 73% and 60% of the age 3 cohort, respectively. Multivariate analysis found ventilator time as the only independent predictor of motor problems at age 1 (odds ratio, 1.12 per day; 95% confidence interval, 1.05-1.20; P < .01).


CONCLUSIONS
Young CDH survivors continue to have ongoing medical problems and a high incidence of motor and language problems. Duration of neonatal ventilatory support was a significant predictor of motor problems at age 1. Prospective studies are needed to confirm these findings.
Department of Medicine, Children's Hospital, Boston, MA 02115, USA.
Sandra
S
Friedman
Catherine
C
Chen
Jocelyn S
JS
Chapman
Stefanie
S
Jeruss
Norma
N
Terrin
Hocine
H
Tighiouart
Susan K
SK
Parsons
Jay M
JM
Wilson
D000046:Academic Medical Centers; D017677:Age Distribution; D000367:Age Factors; D000553:Ambulatory Care / Q000379:methods; D002675:Child, Preschool; D015331:Cohort Studies; D003266:Continuity of Patient Care; D002658:Developmental Disabilities / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000601:surgery*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods; D019957:Motor Skills Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D015999:Multivariate Analysis; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D011336:Probability; D011379:Prognosis; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D012189:Retrospective Studies; D018570:Risk Assessment; D017741:Survivors