LAUSR

PURPOSE This study aimed to describe sac ligation and sequential closure for the management of giant omphalocele (GO) and analyze its outcomes. METHODS The medical records of 13 neonates with GO treated at a tertiary general hospital between July 2012 and April 2020 were reviewed. Sac ligation and progressive external compression were performed on most cases immediately after birth. Staged closure with or without a prosthetic patch was conducted after a period of sac suspension. RESULTS Sac ligation-traction-compression was performed on 12 cases, of which 10 underwent staged closure, one with delayed closure. One patient with coexistent esophageal atresia was deemed ineligible for surgery. Among those who had undergone staged closure, 9 survived; however, one neonate who complicated with bilateral diaphragmatic eventration and severe ventilator-associated pneumonia died from multiple-organ failure. Pentalogy of Cantrell was excluded. One patient in whom primary closure was performed after birth died aged 29 h. Pneumonia was the most common infection among patients (5/13), with three having ventilator-associated pneumonia. The median durations of mechanical ventilation and hospital stay were 22.2 days (range, 1-151) and 44.2 days (range, 2-152), respectively, and 25.6 days and 46.4 days, respectively, among patients with staged closure. Among five infants who required oxygen support for more than 28 days, four had pulmonary hypoplasia. CONCLUSIONS Aside from abdominal wall defects, other major comorbidities and pulmonary hypoplasia influence the prognosis of GO. Sac ligation and staged closure is a effective choice for GO. TYPE OF STUDY Retrospective Study Level of Evidence: Level IV.