Exomphalos with intestinal fistulation: case series and systematic review for clinical characterization, management and embryopathogenesis

Systematic review of 28 cases of exomphalos with intestinal fistulation, a rare variant where intestine opens through an intact sac. Most cases present as exomphalos minor with vitellointestinal duct involvement and Meckel diverticulum. Primary surgical excision with fistula closure achieves good outcomes in most patients, with 14% mortality and 25% complication rate.