Neurodevelopmental and neurofunctional outcomes in children with congenital diaphragmatic hernia
ABSTRACT 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.
- SourceAvailable from: Frans J Walther
Article: Outcome after fetal interventionEarly human development 07/2011; 87(9):581. DOI:10.1016/j.earlhumdev.2011.07.005 · 1.93 Impact Factor
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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 · 1.19 Impact Factor
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ABSTRACT: Overall survival of congenital diaphragmatic hernia (CDH) patients has increased since the widespread implementation of delayed operative repair with extracorporeal membrane oxygenation/gentle ventilation strategies starting in the mid-1990s. With the improvements in survival, CDH survivorship has been marked by increased morbidity with a greater need for monitoring and supportive interventions in infancy and early childhood. The multisystem morbidity and complexity of care for these patients mandate the dedicated resources and focus of medical specialties to ensure that the neurodevelopmental, cardiopulmonary, nutritional, and surgical sequelae of CDH are properly addressed. In this article, we will discuss the experience of CDH centers that offer long-term CDH follow-up as well as the patients' morbidities that require active monitoring until transition to adult care.European Journal of Pediatric Surgery 10/2012; 22(5):384-92. DOI:10.1055/s-0032-1329412 · 0.98 Impact Factor