Book contents
- Frontmatter
- Contents
- List of contributors
- Acknowledgments
- Preface
- Part I General issues
- Part II Head and neck
- Part III Thorax
- 11 Chest wall deformities
- 12 Congenital diaphragmatic hernia
- 13 Surgical management of airway obstruction
- 14 Pulmonary resection and thoracotomy
- 15 Esophageal atresia
- 16 Antireflux procedures
- 17 Esophageal replacement
- 18 Esophageal achalasia
- 19 Congenital malformations of the breast
- Part IV Abdomen
- Part V Urology
- Part VI Oncology
- Part VII Transplantation
- Part VIII Trauma
- Part IX Miscellaneous
- Index
- Plate section
- References
12 - Congenital diaphragmatic hernia
from Part III - Thorax
Published online by Cambridge University Press: 08 January 2010
- Frontmatter
- Contents
- List of contributors
- Acknowledgments
- Preface
- Part I General issues
- Part II Head and neck
- Part III Thorax
- 11 Chest wall deformities
- 12 Congenital diaphragmatic hernia
- 13 Surgical management of airway obstruction
- 14 Pulmonary resection and thoracotomy
- 15 Esophageal atresia
- 16 Antireflux procedures
- 17 Esophageal replacement
- 18 Esophageal achalasia
- 19 Congenital malformations of the breast
- Part IV Abdomen
- Part V Urology
- Part VI Oncology
- Part VII Transplantation
- Part VIII Trauma
- Part IX Miscellaneous
- Index
- Plate section
- References
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 UrologyLong-Term Outcomes, pp. 150 - 157Publisher: Cambridge University PressPrint publication year: 2006
References
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