INCIDENCE AND FACTORS ASSOCIATED WITH NON-SURVIVAL IN INFANTS WITH OMPHALOCELE

Background:  Little is known about omphalocele patients and survival.

Objective:  To determine the estimated mean survival time for a series of omphalocele patients and identify factors associated with non-survival.

Design/Methods:  After IRB approval, a retrospective review was conducted of omphalocele patients between January 1992 and August 2011. Survivors and non-survivors were compared to identify factors associated with non-survival. Infants with complete follow-up to March 2012 and completed repair were included. The cumulative incidence of survival was analyzed by Kaplan-Meier method. Factors included were: gender, gestational age, birth weight, weight-length z-score, APGARs at 1 and 5 minutes, days of ventilation, abnormal chromosomes, persistent pulmonary hypertension (PPHN), congenital heart defects, brain anomalies, defects containing 75% liver, intestinal surgeries, days to goal feedings and days of total parenteral nutrition (TPN). Factors were compared by chi-square tests for categorical and t-tests for continuous variables. A p-value less than 0.05 was considered significant.

Results:  Forty-eight infants were included. Follow-up ranged from 0.5 to 20 years (median:3.5 years). Fourteen (29.2%) died, all during their initial hospitalization. The mean survival time for non-survivors was 0.27 years (SD 0.33) and mean defect size was 30.5 cm² (range 6-94cm²). Thirty-four survivors (70.8%) underwent repair: 12 (35.3%) infants prior to discharge (mean defect size: 8.9cm² (range: 4-25cm²)) and 22 (64.7%) infants after discharge during an elective hospitalization (mean defect size: 38.3²) (range: 8-120cm²). The estimated mean survival time was 14.3 years (SD 1.3)(95% CI:11.8-16.9) Factors associated with non-survival were: abnormal chromosomes (p=0.013), PPHN (p=0.043), greater ventilator days (p=0.004), low APGARs at 1 (p=0.003),and 5 minutes (p=0.010), and failure to achieve goal feedings (p<0.001). Survivors achieved goal feedings faster than non-survivors (mean: 8.9 days (SD 8.3) vs. 73.5 days (SD 122.6)) (p=0.002) and required less TPN ((mean: 17.5 days) (SD 17.9) vs. 76.3 days (SD 87.2)) (p=0.032).

Conclusions:  The estimated mean survival time for omphalocele patients is 14.3 years. Non-survivors die of comorbidities in the first months of life. Abnormal chromosomes, PPHN, low APGARs, failure to achieve goal feedings and a greater TPN requirement are associated with non-survival. Infants with large defects survive if they have few comorbidities, and achieve goal feedings.