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12 - Congenital diaphragmatic hernia

from Part III - Thorax

Published online by Cambridge University Press:  08 January 2010

By
Nikki Almendinger ,
Son Lee West  and
Jay Wilson
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Nikki Almendinger
Affiliation:
Department of Surgery, Children's Hospital and Harvard Medical School, Boston, MA, USA
Son Lee West
Affiliation:
Department of Surgery, Children's Hospital and Harvard Medical School, Boston, MA, USA
Jay Wilson
Affiliation:
Department of Surgery, Children's Hospital and Harvard Medical School, Boston, MA, USA
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
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Summary

Introduction

Despite recent advances in critical care, improved ventilatory support strategies, and ready access to extracorporeal life support (ECLS), the management of patients with congenital diaphragmatic hernia (CDH) continues to be a vexing problem for the clinician. Improvements in treatment strategies have been associated with an increase in overall survival probability for CDH patients in recent years; however, there has also been an unanticipated increase in morbidity in these survivors. The objective of this chapter is to briefly review the history and pathophysiology of CDH, as well as current management strategies, but most importantly to summarize current data related to the long-term outcome, morbidity, and recommended follow-up of these complex patients.

Etiology and embryology

The incidence of CDH is between 1 in 2000 and 5000 live births, yielding approximately 1000 affected infants per year in the USA. It has been suggested that the in utero mortality rate is as high as 30%, although this is probably an overestimate. Over 90% of the defects are posteriolateral and 85% of these are left sided. The etiology of CDH is still not known. Exposure to teratogens such as insecticides, phenmetrazine, quinine, nitrofen and vitamin A deficiency has been implicated in the development of CDH. Although CDH has been reported to be associated with trisomies 18, 21 and 22, the genetic contribution to the lesion is likely to be quite complex. Consequently, sporadic occurrence is most common.

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 150 - 157
Publisher: Cambridge University Press
Print publication year: 2006

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References

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