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: 2.12). 06/2011; 87(9):625-32. DOI: 10.1016/j.earlhumdev.2011.05.005
Source: PubMed

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.

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    ABSTRACT: Background/Purpose: Neonates with significant congenital diaphragmatic hernia (CDH) require cardiopulmonary support. Management has been characterized by progressive abandonment of hyperventilation. Ability to prognosticate outcomes using measures of ventilation and oxygenation with gentle ventilation remains unclear. We sought to determine whether assessment of gas exchange at the time of NICU admission is predictive of survival in this current era. Methods: Neonates with CDH admitted to a Children's Hospital from 1995 to 2006 were evaluated for demographics, blood gas (ABG) measurements and ventilator settings for the first 48 hours, and discharge outcome. Results: One-hundred-and-nineteen CDH patients were admitted, 88 (74%) survived. Mean admission ABG pCO(2) was higher in infants who died compared to survivors (86 +/- 48 versus 49 +/- 20, p <= 0.001); positive predictive value (PPV) for mortality of pCO(2) >= 80 mmHg was 0.71. Mean first hour preductal oxygen saturation (preductalO(2)Sat) was lower in infants who died compared to survivors (81 +/- 17 versus 97 +/- 5, p < 0.001); PPV for mortality of preductalO(2)Sat < 85% was 0.82. Eleven patients met both pCO(2) and preductalO(2)Sat criteria, and 10 (91%) died, PPV of 0.92. Within hours of admission, pCO(2) and preductalO(2)Sat differences between survivors and nonsurvivors lost significance. Conclusion: Admission pCO(2) and preductalO(2)Sat may be useful in predicting survival in neonatal CDH. The differential in gas exchange between survivors and nonsurvivors loses significance with contemporary neonatal care.
    Journal of Pediatric Surgery 08/2014; 49(8):1197-201. DOI:10.1016/j.jpedsurg.2014.03.011 · 1.31 Impact Factor
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    ABSTRACT: This literature review discusses the growing trend toward multidisciplinary long term follow-up for congenital diaphragmatic hernia.
    Seminars in Fetal and Neonatal Medicine 10/2014; DOI:10.1016/j.siny.2014.09.001 · 3.13 Impact Factor
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    ABSTRACT: Background/Purpose The purpose of this paper was to examine the effect of prenatal counseling in the Advanced Fetal Care Center (AFCC) on the well-being of parents of infants with congenital diaphragmatic hernia (CDH). Methods From 2008 through 2012, 26 mothers and fathers of surgically-repaired CDH patients who received prenatal counseling at our institution, and 15 who received no prenatal counseling, each completed the Short-Form 36 version 1 (SF-36v1) at the appropriate time points: prenatal, two weeks, and six months post-surgery. Results Parents in both groups did not differ by demographic characteristics. Patients who received prenatal counseling had significantly longer ventilatory time and length of stay (LOS) in the ICU and in the hospital compared to those who did not receive prenatal counseling (all P < .01). Mothers and fathers had similar SF-36v1 mental and physical component summary (MCS, PCS) post-surgery scores when compared by counseling status. Prenatal MCS scores for mothers and fathers (47 vs. 41; P = .24) were similar to those at six months post-surgery (47 vs. 47; P = .90). Conclusions When hospital LOS was controlled between groups stratified by AFCC counseling status, MCS scores were comparable prenatally and were sustained at six months post-surgery for both parents. These findings may reflect the support services parents received beginning in the prenatal period.
    Journal of Pediatric Surgery 05/2014; DOI:10.1016/j.jpedsurg.2014.02.050 · 1.31 Impact Factor