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Chapter 19.1 - Congenital diaphragmatic hernia

Pathophysiology

from Section 2 - Fetal disease

Published online by Cambridge University Press:  05 February 2013

By
Nicola A. Lewis  and
Philip L. Glick
Edited by
Mark D. Kilby ,
Anthony Johnson  and
Dick Oepkes
Mark D. Kilby
Affiliation:
Department of Fetal Medicine, University of Birmingham
Anthony Johnson
Affiliation:
Baylor College of Medicine, Texas
Dick Oepkes
Affiliation:
Department of Obstetrics, Leiden University Medical Center
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Summary

Introduction

Congenital diaphragmatic hernia (CDH) occurs in 1 in 2000 to 1 in 3800 births [1, 2]. Prenatal diagnosis carries a 2–4 times greater mortality perhaps related to larger defects and more severe pulmonary hypoplasia or associated anomalies in these infants. For fetuses identified prenatally, 25–70% are born alive of which approximately 55% survive to discharge [1–3]. Seventy percent of postnatal deaths occur within the first 24 hours [4].

Major non-pulmonary malformations occur in 15–72% of prenatally detected cases. This is associated with a fourfold increase in mortality rate compared to infants with isolated CDH; 95% of stillbirths and 60% of infants dying within the first 24 hours have non-pulmonary major malformations [3].

The diaphragmatic defect

The most common diaphragmatic defect is the posterolateral defect (Bochdalek hernia ). Anterior defects (Morgagni hernias) or total agenesis of the hemi-diaphragm are also seen. Diaphragmatic defects occur on the let in 80–90%, on the right in 5–15%, and bilateral or anterior Morgagni hernias in 2–5% of infants [3].

Keywords

visceral herniationpulmonary hypoplasiapulmonary hypertensionsurfactant deficiencylung injurycongenital diaphragmatic hernialung immaturity
Type
Chapter
Information
Fetal Therapy
Scientific Basis and Critical Appraisal of Clinical Benefits
 , pp. 370 - 375
Publisher: Cambridge University Press
Print publication year: 2012

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References

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