A. C. Akinkuotu1,2, F. Sheikh1,2, O. Olutoye1,2,3, T. Lee1,2, C. J. Fernandes1,3, S. Welty1,3, N. Ayres1,3, D. Cass1,2,3 1Texas Children’s Hospital,Texas Children’s Fetal Center,Houston, TX, USA 2Baylor College Of Medicine,Michael E. DeBakey Department Of Surgery,Houston, TX, USA 3Baylor College Of Medicine,Pediatrics,Houston, TX, USA
Introduction:
Management of giant omphalocele (GO) presents the pediatric surgeon with a conundrum related to the ideal timing of abdominal closure. Because the optimal surgical approach remains unknown, the decision to delay abdominal closure or operate early in the neonatal period is left to surgeon preference. The purpose of this study was to describe the current management and outcomes of infants with omphaloceles at our institution.
Methods:
All patients treated for omphalocele 1/03-2/14 were reviewed. Patients were classified as either isolated omphalocele, or omphalocele with minor or major associated anomalies. Major anomalies were defined as a cardiac defect requiring immediate medical or surgical treatment, Bochdalek-type CDH, alveolar-capillary dysplasia, and chromosomal aneuploidy or duplication. All other anomalies were classified as minor. Prenatal data collected included fetal MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV). Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac.
Results:
Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation and 36 presented postnatally. Of fetal patients, 3 had pregnancy termination, 7 (12%) had in-utero demise, and 3 were delivered elsewhere, treated with comfort measures and suffered immediate neonatal demise. Of 82 live-born infants at our institution, 21 (26%) had chromosomal anomalies and 25 (30%) had major associated anomalies. No live-born baby with an isolated defect (n=19) died, whereas mortality was 15% and 33% for those with minor and major anomalies, respectively (p=0.006). Infants with major anomalies had significantly longer median length of intubation (36 vs.0 vs.0 days; p=0.04) and hospital stay (157vs.8.5vs.18 days; p<0.001) compared to those with minor or no anomalies. Patients with major anomalies also had a significantly higher need for oxygen at 30 days of life (Table). Of 41 infants with GO, (80% 6-month survival), the majority (85%) were managed surgically by delayed closure with a median age at repair of 9 months (range, 3.4-23.6 months). None of the delayed repair patients required a later operative revision while 2 of 6 with early repair did.
Conclusion:
The presence of associated anomalies is the strongest predictor of mortality in fetuses or neonates with omphalocele. For those with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to delineate the optimal treatment approach.
