The objective of this review was to provide a critical overview of our current understanding on the neurocognitive, neuromotor, and neurobehavioral development in congenital diaphragmatic hernia (CDH) patients, focusing on three interrelated clinical issues: (1) comprehensive outcome studies, (2) characterization of important predictors of adverse outcome, and (3) the pathophysiological mechanism contributing to neurodevelopmental disabilities in infants with CDH. Improved survival for CDH has led to an increasing focus on longer-term outcomes. Neurodevelopmental dysfunction has been recognized as the most common and potentially most disabling outcome of CDH and its treatment. While increased neuromotor dysfunction is a common problem during infancy, behavioral problems, hearing impairment and quality of life related issues are frequently found in older children and adolescence. Intelligence appears to be in the low normal range. Patient and disease specific predictors of adverse neurodevelopmental outcome have been defined. Imaging studies have revealed a high incidence of structural brain abnormalities. An improved understanding of the pathophysiological pathways and the neurodevelopmental consequences will allow earlier and possibly more targeted therapeutic interventions. Continuous assessment and follow-up as provided by an interdisciplinary team of medical, surgical and developmental specialists should become standard of care for all CDH children to identify and treat morbidities before additional disabilities evolve and to reduce adverse outcomes.
"Ultrasound-guided insertion of a thoraco-amniotic shunt is increasingly advocated as treatment in hydropic fetuses with primary hydrothoraces and/or space-occupying congenital lung lesions, such as congenital cystic adenomatoid malformations (CCAM)  , and vesicoamniotic shunting is available as intervention in congenital lower urinary tract obstruction (LUTO) . Lethal lung hypoplasia in congenital diaphragmatic hernia (CDH) can be reversed by fetal endoscopic tracheal occlusion (FETO) using a balloon technique  . "
[Show abstract][Hide abstract] ABSTRACT: To ascertain developmental differences between term infants after major noncardiac surgery and cardiac surgery compared with healthy control infants in New South Wales, Australia.
This prospective population-based cohort study enrolled infants between August 1, 2006, and December 31, 2008, who required major noncardiac surgery within the first 90 days of life. Developmental outcomes were compared in these children, cohorts of term infants requiring cardiac surgery, and healthy controls. Infants were assessed at 1 year of age using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III).
Of the 784 infants enrolled, 688 (90.2%) of infants alive at 1 year were assessed. Of these, 539 infants were term and were included in the present analysis. Compared with controls, the infants who underwent cardiac surgery had significantly lower (P < .001) mean scores in all 5 BSID-III subscales, and the infants who underwent noncardiac surgery had significantly lower (P < .05) mean scores in 4 of the 5 BSID-III subscales. The greatest difference was in the incidence of gross motor delay in both the cardiac surgery group (OR, 0.25; 95% CI, 0.16-0.41) and the noncardiac surgery group (OR, 0.41; 95% CI, 0.26-0.63).
This unique population-based prospective study compared the developmental outcomes of infants who underwent major noncardiac surgery and cardiac surgery. Major surgery in infants was found to be significantly associated with developmental delay at 1 year of age compared with control infants. These data have important implications for interventions and clinical review in the first year of life.
The Journal of pediatrics 05/2012; 161(4):748-752.e1. DOI:10.1016/j.jpeds.2012.03.044 · 3.79 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Early detection of neurodevelopmental delay and appropriate intervention has been associated with improved academic and social outcomes. Identifying those who are at high risk and might benefit is not straightforward. Approximately 2% of infants are admitted to a neonatal intensive care unit after birth and these babies are known to be at high risk of developmental impairment. While it is well recognised that the extreme preterm infant is at high risk of developmental impairment, there is increasing evidence of a risk in late preterm infants as well as those undergoing major cardiac and non-cardiac surgery. Not all infants are enrolled in multidisciplinary follow-up clinics with easy access to early intervention. These clinics are expensive to run with both limited and conflicting data on their long-term value. This review will concentrate on identifying which infants are at risk, reviewing the aetiology of the risk factors and the efficacy of follow-up clinics.
Journal of Paediatrics and Child Health 09/2012; 48(9):789-93. DOI:10.1111/j.1440-1754.2012.02540.x · 1.15 Impact Factor
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