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A D V E R T I S E M E N T
A D V E R T I S E M E N T
Pulmonary hypoplasia, and the resulting pulmonary hypertension, are what cause all the difficulties in these children. It appears that the timing of the entry of the viscera into the chest is critical – if it penetrates the chest early in gestation, the lung is severely inhibited in its growth. If penetration occurs later (after the lung has had chance to develop further) the problem can be much less severe.
A D V E R T I S E M E N T
This drawing on the x-ray depicts the material in the chest, including stomach, large and small intestine, and the spleen. Note how little space remains for the heart and lungs. An NG tube is very helpful in these babies to decrease stomach and bowel distention.
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A D V E R T I S E M E N T
Presently, children are not rushed to the operating room. As pulmonary hypertension is the major concern, and operative intervention can trigger this, aggressive attempts are made to stabilize the child for 5 to 7 days prior to operative correction. Interventions such as high-frequency ventilation and nitric oxide therapy are used.
If the child cannot be stabilized, she can be placed on an ECMO (extra corporeal membrane oxygenation) circuit – essentially an artificial lung – for up to three weeks while the lung matures. In this case, the child stabilized on high-frequency ventilation and nitric oxide and was operated on six days after birth. Pre-operative positioning is shown here.
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This page was last modified on 3/15/1999.
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