Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program
ABSTRACT The purpose of the study was to evaluate the neurodevelopmental outcome in infants with congenital diaphragmatic hernia (CDH).
Between June 2004 and September 2007, 41 CDH survivors were prospectively enrolled in an interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development II (prior 2006, n = 9), the Bayley Scales of Infant Development III (after 2006, n = 27), or the Wechsler Preschool and Primary Scale of Intelligence III (children older than 4 years, n = 5). Scores were grouped as average, mildly delayed, and severely delayed by standard deviation intervals (115-85, 71-84, <70), and mixed if average and mildly delayed in either cognitive or language.
Median age at last assessment was 24 months (range, 6-62). Average, mixed, mildly delayed, and severely delayed scores for neurocognitive and language skills were found in 49%, 19%, 17%, and 15%, respectively. Psychomotor scores were normal, mildly delayed, and severely delayed in 46%, 23%, and 31%, respectively. Autism was present in 7%. Abnormal muscle tonicity was found in 51% (49% hypotonic, 2% hypertonic). Multivariate risk factors for borderline or delayed neurodevelopmental, neurocognitive, and/or psychomotor outcome were intrathoracic liver position (P = .02), presence of a right-sided CDH (P = .02), extracorporeal membrane oxygenation need (P < .001), Gore-Tex patch repair (P = .02), O(2) requirement at 30 days of life (P < .01), and hypotonicity (P < .01).
The prospective evaluation in an interdisciplinary follow-up program uncovered striking morbidities in neurodevelopmental status in approximately half of the CDH infants. The most common neurologic sequelae are neuromuscular hypotonicity and psychomotor dysfunction. Patient-specific factors are important determinants of adverse neurologic outcome.
- [Show abstract] [Hide abstract]
ABSTRACT: Notfälle bei Neugeborenen stellen für Geburtshelfer und Neonatologen eine große Herausforderung dar. Beim Mekoniumaspirationssyndrom (MAS; Inzidenz europaweit 0,5–1:1000 Lebendgeburten) kennzeichnen respiratorisches Versagen und häufig eine begleitende pulmonale Hypertension das klinische Bild. Die effektive endotracheale Absaugung unmittelbar nach der Geburt ist hier eine der wichtigsten Maßnahmen. Bei der kongenitalen Zwerchfellhernie (CDH) entwickelt sich in Folge des Defekts und der thorakalen Verlagerung von Bauchorganen eine Lungenhypoplasie. Hier besteht ein hohes Risiko für eine persistierende pulmonale Hypertonie (PPHN). Die Neugeborenen werden sofort intubiert und kontrolliert maschinell beatmet; der chirurgische Eingriff erfolgt nach 24–72Stunden. Durch pränatale Bestimmung der „Lung-head-Ratio“ und des fetalen Lungenvolumens können Mortalität oder Wahrscheinlichkeit für eine ECMO-Behandlung geschätzt und die optimale Therapie geplant werden. Die perinatale Asphyxie (Inzidenz 3–5:1000 Lebendgeburten) ist Hauptursache für neurologische Langzeitschäden und perinatale Todesfälle reifer Neugeborener. Kommt es zu einer hypoxisch-ischämischen Enzephalopathie stellt die Hypothermiebehandlung einen vielversprechenden Therapieansatz dar. Neonatal emergencies still represent a major challenge with important implications for obstetricians and perinatologists. Meconium aspiration syndrome (MAS incidence 0.5–1 per 1,000 live births in Europe) is characterized by an early onset of respiratory failure and often by pulmonary hypertension. Endotracheal inspection and suctioning of meconium immediately after birth are the most important therapeutic steps. In congenital diaphragmatic hernia (CDH), herniation of the abdominal viscera into the thorax leads to severe persistent pulmonary hypoplasia and pulmonary hypertension (PPHN). These newborns must be immediately intubated and receive mechanical ventilation; surgery is performed after 24-72h. Biometric assessment of fetal lung volume and the fetal lung area to head circumference ratio (LHR) are the methods for prenatal prediction of survival and the need for extracorporeal membrane oxygenation (ECMO) and thus allow planning the optimal therapy. Perinatal asphyxia (estimated incidence 3–5 per 1,000 live births) is the most common reason for mortality and long-term neurological morbidity in term newborns. In cases of hypoxic-ischemic encephalopathy (HIE), hypothermia is a promising therapeutic approach. SchlüsselwörterNotfälle–Reifgeborene–Mekoniumaspiration–Kongenitale Zwerchfellhernie–Asphyxie KeywordsEmergencies–Term newborns–Meconium aspiration–Congenital diaphragmatic hernia–AsphyxiaIntensivmedizin + Notfallmedizin 02/2011; 48(1):7-14. DOI:10.1007/s00390-010-0201-6
- [Show abstract] [Hide abstract]
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.Early human development 06/2011; 87(9):625-32. DOI:10.1016/j.earlhumdev.2011.05.005
- [Show abstract] [Hide abstract]
ABSTRACT: Respiratory disorders remain a major problem in postnatal adaptation. In term neonates, an increased incidence of the risk for transient tachypnoea of the neonate has been observed during the past decade, most likely secondary to an increased usage of primary Caesarean section. The disorder is mainly caused by a delayed resorption of foetal lung fluid. Further disorders in term neonates include meconium aspiration syndrome and congenital diaphragmatic hernia leading to impaired gas exchange and pulmonary hypertension. In preterm neonates, respiratory distress syndrome is the main disorder leading to severe acute and long-term impaired gas exchange. Prenatal administration of glucocorticoids and postnatal surfactant therapy remains an established principle in perinatal care for very preterm neonates. The most relevant long-term sequelae, bronchopulmonary dysplasia, is currently being observed in about 15% of preterms with less than 32 weeks of gestation and is associated with severe pulmonary and extrapulmonary consequences. Due to the overall improvement in perinatal care, respiratory disorders still remain a major problem in pulmonary adaptation. However, mortality secondary to neonatal lung failure has been decreased substantially by the improvements in the whole field of perinatal medicine.Zeitschrift für Geburtshilfe und Neonatologie 08/2011; 215(4):145-51. DOI:10.1055/s-0031-1285835