Associated Anomalies and Clinical Outcomes in Infants with Omphalocele: A Single-centre 10-year Review

摘要

Objective: To examine the perinatal characteristics, associated anomalies and clinical outcomes of newbornswith omphalocele admitted to Queen Mary Hospital (QMH), Hong Kong during a 10-year period. Methods:We identified all newborns with omphalocele who were admitted to the neonatal intensive care unit atQMH from 2005 to 2014. Maternal and patient demographic data, associated anomalies and outcomedata were reviewed retrospectively. Results: A total of 19 infants with omphalocele were identified.Median gestational age at birth was 38 weeks with a median birth weight of 3140 g. Fifty-three percent(10/19) were diagnosed with at least 1 other anomaly, with congenital heart defect being the most commonassociated anomaly. Median age at first operation (either primary closure or application of silo) was on day1 of life, with delayed closure in staged operations being carried out at a median age of 8 days. Infants withnon-liver containing omphaloceles were more likely to have primary repair compared to those with livercontainingones (7/8 [88%] vs. 4/11 [36%], p=0.03). Overall survival rate at discharge was 84% (16/19).Two out of 3 cases died of lethal congenital anomaly (alveolar capillary dysplasia) while the other onesuffered from postoperative midgut volvulus. Postoperative complications occurred in 6 patients (6/17[35%]), with ventral hernia being the most common complication. Long-term medical problems includingfailure to thrive (6/12 [50%]), gastroesophageal reflux (5/12 [42%]), developmental delay (3/12 [25%])and recurrent lung infections (1/12 [8%]) were identified. Conclusions: Associated anatomic and geneticanomalies are common in omphalocele. Postnatal workup should include screening of these anomalies.Prognoses for isolated omphaloceles with no major postoperative complications are good in the longterm.