A neonate with giant omphalocele complicated by mechanical intestinal obstruction requiring left hepatic lobe resection: a case report.

BACKGROUND: Giant omphalocele is a rare congenital abdominal wall defect often associated with viscero-abdominal disproportion and frequently requiring prolonged staged repair. Secondary mechanical bowel obstruction during staged reduction is uncommon and may be difficult to distinguish from expected neonatal dysmotility or postoperative ileus. Reports of hepatic resection to facilitate reduction in neonates with giant omphalocele are extremely limited. CASE PRESENTATION: We report a term small-for-gestational-age female neonate with a giant liver-containing omphalocele. Initial management included intensive care support, sac protection, ventilatory support, and staged reduction by sac suspension. On hospital day 15, new bilious or turbid gastric drainage and deterioration of the omphalocele sac prompted urgent reassessment for secondary bowel compromise. Because the infant was clinically unstable, urgent operative exploration was prioritised over additional radiological investigations. Exploratory laparotomy revealed yellow fluid within the sac, dense adhesions involving the small bowel, greater omentum, and liver, and adhesion bands causing small-bowel kinking. Congenital intestinal malrotation was also present, but no frank bowel necrosis was identified. Complete reduction remained unfeasible, and a sterile silo pouch was placed for continued staged management. Despite continued staged treatment, meaningful further reduction could not be achieved because of extremely limited abdominal domain, marked visceral oedema, and the large non-compressible herniated liver. On hospital day 21, second-stage reduction with left hepatic lobe resection was performed as an exceptional rescue strategy after conventional staged reduction had failed. At 6 months' follow-up, the patient was tolerating oral feeding and showed age-appropriate growth and neurodevelopment, with no clinically evident ventral hernia or liver-related complications. CONCLUSION: Neonates with giant omphalocele who undergo staged treatment and develop new bilious or turbid gastric drainage together with deterioration of the omphalocele sac require urgent assessment for mechanical bowel obstruction. Timely reassessment, flexible staged surgery, and multidisciplinary intensive care are critical to successful management. In highly selected cases with persistent severe loss of abdominal domain after failure of conventional staged reduction, hepatic resection may be considered, if at all, only as an exceptional last-resort rescue strategy.