An international survey of gastroschisis and omphalocele (490 cases)

Abstract

In an international survey of 16 pediatric surgery centers on four continents, data concerning 490 cases of gastroschisis and omphalocele has been obtained for study. In 203 cases of gastroschisis, additional “malformations” were found to be infrequent, comparatively mild, rarely multiple, largely limited to the eviscerated gut (atresias and stenoses), and most likely acquired as antenatal complications (volvulus) of the gastroschisis condition. These additional “malformations” are more properly categorized as an integral part of the gastroschisis disorder, just as is the almost universally associated intestinal malrotation. In 287 cases of omphalocele, 41 cases (14%) were classifiable as “syndrome” omphalocele (lower-midline syndrome, upper-midline syndrome or Beckwith-Wiedemann syndrome) and 12 cases were trisomy-associated. In omphalocele cases, additional malformations were frequent, serious and generally multiple (74%). The most frequent malformations in omphalocele cases involved the musculoskeletal/neurologic area (32%), followed by the cardiac area (16%) where the malformations tended to be both multiple and serious. In omphalocele cases, the geographic incidence both of numbers of additional malformations and multiplicity of defects was found to be rather similar, with the major geographic difference being found in the relative incidence of cases of “syndrome” omphalocele.