Neurodevelopmental and neurofunctional outcomes in children with congenital diaphragmatic hernia

The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, PA 1910, USA.
Early human development (Impact Factor: 1.79). 06/2011; 87(9):625-32. DOI: 10.1016/j.earlhumdev.2011.05.005
Source: PubMed


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.

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    • "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) [4] [5], and vesicoamniotic shunting is available as intervention in congenital lower urinary tract obstruction (LUTO) [6]. Lethal lung hypoplasia in congenital diaphragmatic hernia (CDH) can be reversed by fetal endoscopic tracheal occlusion (FETO) using a balloon technique [7] [8]. "

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