THORACIC DEFORMITY AND RESPIRATORY FAILURE IN INFANTS WITH GIANT CMPHALOCELE

Abstract

Although respiratory failure in infants with abdominal wall defects has been attributed to increased intra-abdominal pressure after closure, we have observed prolonged respiratory insufficiency in several such infants despite operations designed to minimize intra-abdominal pressure. We therefore reviewed the charts of 108 infants from 1975-82 who had abdominal wall defects: gastroschisis - 54; small amphalocele - 29; liver-containing or giant amphalocele (GO) - 21; and cloacal extrophy-4. Nine of 21 infants with GO (43%) had prolonged respiratory insufficiency and 5 eventually died. Only 2 of 87 infants with other abdominal wall defects had prolonged respiratory insufficiency and neither died of respiratory failure. Infants with GO required longer durations of both oxygenation (p<.01, ANOVA, = 122 days, range 0-1030) and ventilation (p<.001, ANOVA, = 66 days, range 0-387) than infants in the other groups. Clinical observation suggested that infants with GO have small, narrow chests resembling thoracic dystrophy. Therefore, chest radiographs from all groups were examined for several parameters of thoracic size. After correction for birth weight, the chests of babies with GO were significantly narrower than the chests of the other babies (p<.001, ANOVA). Prolonged respiratory insufficiency in infants with giant amphaloceles appears to be explained by pulmonary hypoplasia or by the narrow chest deformity which limits lung expansion.