Omphalocele: Contemporary Outcomes From A Multicenter Registry

Sunday, October 27, 2013: 9:20 AM
Windermere Ballroom W (Hyatt Regency Orlando, formerly the Peabody)
Saleem Islam, MD, MPH, Department of Surgery, Section of Pediatric Surgery, University of Florida, Gainesville, FL, Cynthia D. Downard, M.D., M.M.Sc., Pediatric Surgery, University of Louisville, Louisville, KY, Shawn D St Peter, MD, Surgery, The Children's Mercy Hospital, Kansas City, MO, Faisal G. Qureshi, Pediatric Surgery, Children's National Medical Center, Washington, DC, Steven W. Bruch, Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, Paul D. Danielson, MD, All Children's Hospital, St. Petersburg, FL and Elizabeth Renaud, MD, Montefiore Medical Center, New York, NY

Purpose: Omphaloceles can be challenging cases to manage. It has been difficult to generate data to guide management or define outcomes due to scarcity of cases. Most data that exists is from single center studies with historical bias. We report initial results from a large, contemporary, multicenter registry of abdominal wall defects.

Methods: After IRB approvals from each center, data from eight institutions were collected using a web-based registry. All cases were from 2000-2013. Prenatal, maternal, perinatal, surgical, and outcome information was collected for each case and data was analyzed.

Results: 228 omphalocele cases were identified. A majority (74%) were delivered by cesarean section, with a mean gestational age of 36.6 weeks. Overall survival to discharge was 84.7%. Any associated anomaly was noted in 63% of cases, with cardiovascular abnormalities responsible for a majority of them (85%). Initial non-operative approach was used in 45 cases, mesh repair in 36, 18 had silo creation or placement, while 120 had primary closure performed. For the remainder of the analysis, we excluded defects categorized as hernias of the cord, and divided the cohort into those with large/giant defects (n=80) and small/medium sized (n=72) ones.  Large defects had a significantly longer LOS, time on ventilator, TPN, time to full feeds, mortality and discharge without repair compared to the smaller ones. We noted no difference in gestational age, birth weight, Apgar scores, or number of anomalies between these groups. (Table)

Conclusions: This is the largest contemporarily managed series of omphaloceles reported. Overall survival was high, and associated anomalies common. There was a high rate of cesarean delivery. Size is an independent risk factor for mortality and morbidity. Better definition of the defect will allow us to select patients for prospective studies on best management techniques.

G age Birth Wt Assoc. Anom Vent Days TPN days Full feeds day LOS days Survival
Large (n 80) 36.6 2951 75% 50.4 36.8 41.7 71.6 75%
Small (n 72) 36.5 2886 63.4% 9.9 16.6 20.1 27.3 7%
p value 0.85 0.64 0.16 0.0001 0.022 0.019 0.0001 0.004