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Chapter 48 - Congenital Diaphragmatic Hernia: In Utero Treatment Today and Tomorrow

from Congenital Diaphragmatic Hernia

Published online by Cambridge University Press:  21 October 2019

Mark D. Kilby
Affiliation:
University of Birmingham
Anthony Johnson
Affiliation:
University of Texas Medical School at Houston
Dick Oepkes
Affiliation:
Leids Universitair Medisch Centrum
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Summary

Congenital diaphragmatic hernia (CDH) is a life-threatening surgically correctable congenital birth defect (3/10 000 live-born babies) [1]. Survival chances are dependent on the presence of associated malformations and severity of lung hypoplasia. Mortality remains up to 30% [2]. In the previous chapter we have outlined how fetuses with the suspicion of CDH should be assessed, using genetic testing and modern imaging techniques. Individualized prognosis of isolated CDH can be made prenatally by measurement of lung size, the presence of the liver in the thorax, and the side of the defect [3]. Patients with predicted poor prognosis are ideal candidates for an intervention that may improve the outcome. Such intervention is not aiming at repairing the diaphragmatic defect, as it can be easily closed after birth: it rather should reverse pulmonary hypoplasia (i.e. stimulate lung growth) before birth. Historically this was attempted by anatomical repair of the defect in utero, yet results were suboptimal [4].

Type
Chapter
Information
Fetal Therapy
Scientific Basis and Critical Appraisal of Clinical Benefits
, pp. 503 - 511
Publisher: Cambridge University Press
Print publication year: 2020

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References

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