Author(s): Tsakayannis DE, Zurakowski D, Lillehei CW
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Abstract For infants with omphalocele, the size of the defect and the presence of associated anomalies are well known prognostic factors. However, the prognostic importance of the respiratory status at birth has not been well defined. The authors reviewed the records of 30 infants with omphalocele (treated during a 10-year period) to determine whether respiratory insufficiency at birth affected survival. Eighteen infants did not require ventilatory support before repair of the omphalocele. The mean gestational age and birth weight were 38.2 +/- 2.6 weeks and 3.4 +/- 0.6 kg, respectively. Cardiac or other major associated anomalies were present in six infants (33\%). Seven (39\%) had a "giant" omphalocele (ie, liver-containing and/or omphalocele sac > 5 cm in diameter). The average length of ventilatory support was 3.2 days. All infants in this group were managed by primary (14) or staged closure of the omphalocele (4) immediately after birth. One infant died, but the remainder survived without any significant complications. Twelve infants had severe respiratory distress at birth and required positive pressure ventilation (mean peak inspiratory pressure, 31.4 +/- 1.2 cm H2O; mean FiO2, 0.8 +/- 0.1). The mean gestational age and birth weight were 32.7 +/- 3.5 weeks and 1.9 +/- 0.8 kg, respectively. Cardiac or other major associated anomalies were present in nine infants (75\%), and eight (67\%) had a giant omphalocele. The average length of ventilatory support was 57.7 days, which was significantly longer than for the previous group (P < 0.001). Two infants died of respiratory failure within 48 hours of birth, before the initiation of any treatment for the omphalocele. Six were managed with surgical repair of the omphalocele, primary or staged, immediately after birth. In four, topical treatment was used to allow improvement in the respiratory status. Only one of the six infants initially managed by surgical repair survived, whereas three of the four infants managed nonsurgically recovered. Stepwise logistic regression analysis showed that the presence of respiratory distress at birth was the only significant predictor of mortality, independent of gender, gestational age, birth weight, presence of other anomalies, or size of the omphalocele (odds ratio = 25.48; likelihood ratio test = 13.86; P < .001). In conclusion, respiratory failure at birth in infants with omphalocele is a significant predictor of mortality. Initial conservative management of the omphalocele until there is improvement in the respiratory status may result in a better outcome.
This article was published in J Pediatr Surg
and referenced in Clinics in Mother and Child Health