Congenital diaphragmatic hernia: review of the literature in reflection of unresolved dilemmas

Background:  Congenital diaphragmatic hernia (CDH) is a rare but clinically and scientifically challenging condition. The introduction of ultrasound has enabled early prenatal detection and consequently, hope of early therapeutic intervention. Aim:  We undertook the task to review the recent develop...

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Bibliographic Details
Published in:Acta Paediatrica 2009-12, Vol.98 (12), p.1874-1881
Main Authors: Grisaru-Granovsky, S, Rabinowitz, R, Ioscovich, A, Elstein, D, Schimmel, MS
Format: Article
Language:English
Subjects:
Antenatal diagnosis
Biological and medical sciences
Congenital diaphragmatic hernia
Female
Fetal Diseases - diagnosis
Fetal Diseases - therapy
Fetal Therapies - methods
Foetal surgery
General aspects
Hernia, Diaphragmatic - diagnosis
Hernia, Diaphragmatic - therapy
Hernias, Diaphragmatic, Congenital
Humans
Hypoplastic lung
Infant, Newborn
Medical sciences
Pregnancy
Prenatal Diagnosis
Prognosis
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Summary:Background:  Congenital diaphragmatic hernia (CDH) is a rare but clinically and scientifically challenging condition. The introduction of ultrasound has enabled early prenatal detection and consequently, hope of early therapeutic intervention. Aim:  We undertook the task to review the recent developments in understanding the pathology of CDH as well as the history and current management strategies to aid perinatologists in consultations with parents of CDH‐affected foetuses. Study design:  A Medline search was undertaken of all reports and reviews published between 1980 and 2008 using MeSH search terms ‘diaphragmatic hernia’, ‘congenital’ and ‘newborn’. Results:  The true incidence of CDH is still difficult to estimate because of the high incidence of hidden mortality of CDH. Complete case ascertainment also poses difficulties in assessment of the impact of new therapeutic modalities on overall survival. Recent improvements in prenatal detection are a milestone in affording time for re‐assessments and parental counselling. The true benefit of antenatal therapy is circumscribed and should be offered only in selected cases of isolated severe CDH as defined by existing guidelines. Postnatal intensive respiratory supportive therapy and innovative surgical techniques within specialized tertiary centres has had a major impact on survival of babies with CDH. Conclusion:  The high survival of ‘selected cases’ that are live births and benefit from optimal care will be difficult to improve by antenatal interventions. The multidisciplinary approach to basic research and randomized clinical trials will further define the best approach to the foetus and neonate with CDH.
ISSN:0803-5253
1651-2227
DOI:10.1111/j.1651-2227.2009.01436.x