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Congenital Diaphragmatic Hernia

  • Julia Zimmer
  • Prem PuriEmail author
Living reference work entry
  • 190 Downloads

Abstract

Congenital diaphragmatic hernia is a relatively common congenital malformation with a poorly understood etiology. On account of advances in technical equipment and operative and anesthetic treatment modalities, the surgical repair of the diaphragmatic defect either open or minimally invasive is nowadays often unproblematic. However, associated pulmonary hypoplasia and persistent pulmonary hypertension lead postnatally to severe respiratory distress and contribute to the high morbidity and mortality rates in this potential life-threatening condition. Therefore, optimal perioperative stabilization and management by an interdisciplinary team of pediatric surgeons, neonatologists, and anesthetists is crucial for these neonates. Nearly half of the CDH survivors beyond the neonatal period are able to lead a normal healthy and symptom free life. However, many survivors with complex medical and surgical needs require a multidisciplinary comprehensive care for their pulmonary, neurodevelopmental, and nutritive long-term outcome. Clinical and basic research continues to identify underlying gene and protein alterations and through this providing a potential for new treatment options for CDH.

Keywords

Congenital diaphragmatic hernia Pulmonary hypoplasia Pulmonary hypertension Diaphragmatic repair Animal models Nitrofen 

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.National Children’s Research CentreOur Lady’s Children’s HospitalDublinIreland
  2. 2.Department of Pediatric SurgeryHannover Medical SchoolHannoverGermany
  3. 3.School of Medicine and Medical Science and Conway Institute of Biomedical ResearchUniversity College DublinDublinIreland

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