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Understanding and Treating Congenital Diaphragmatic Hernia

use by pediatric surgeons and pathologists at several institutions. We anticipate that widespread use of a standard approach to CDH classification will improve the quality of information in the medical literature, and ultimately will be used to develop genotype-phenotype correlations, they state.

In the womb, fetal lung growth and maturation are strongly affected by the extent to which developing lungs are stretched by liquid that fills future airspaces. In the article by Paul A. Khan, Marc Cloutier, and Bruno Piedboeuf of the Centre de Recherche du Centre hospitalier de LUniversite Laval data are presented from examination of sheep, rabbit, rat and mice models of tracheal occlusion (TO). TO, a surgery that is performed in utero helps stimulate fetal lung growth and remedy pulmonary hypoplasia by closing the trachea, thus preventing fluid from leaving the lungs and causing them to be stretched. TO not only promotes development of an increased gas exchange surface area, but also markedly remodels pulmonary vasculature and stimulates endothelial cell proliferation, the authors state. In all of the models they note that TO eliminates the thickening of fine blood vessels that afflicts CDH patients. With future technical refinement and improved post-surgical care, TO may well become a more reliable and effective means of clinical intervention, they conclude.

Advances in understanding diaphragm development from the evaluation of genetic mouse models were discussed in an article by Ackerman and John J. Greer of the University of Alberta. They note that there are genes that are now known to play a role in diaphragm development, and that many of these genes are also important for cardiac and lung development. They describe the role of the transcription factor Fog2: Fog2 is the first gene recognized to be necessary for both primary lung development and primary diaphragm development providing evidence for the hypothesis that diaphragmatic defec
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